Epidermal Nevi, Neoplasms, and Cysts

Epidermal Nevi, Neoplasms, and Cysts

Syringoma
* Small translucent papule
* Commonly on eyelids or upper cheeks
* Axilla, abdomen, forehead, penis, vulva
* Develop slowly and persist indefinitely
* Asymptomatic
* 18% of adults with Down’s syndrome
* Dilated cystic sweat ducts
* Treatment
o Electrodessication
o Laser ablation
o cryotherapy

Variants of Syringoma
* Clear cell syringoma
o Associated with diabetes mellitus
o Identical lesions, histological difference
* Other clinical variants
o Limited to the scalp causing alopecia
o Unilateral linear or nevoid distribution
o Limited to vulva and penis
o Limited to distal extremities

Eruptive syringoma
* Numerous lesions on the neck, chest, axilla, upper arms and periumbilically
* Young persons
* Histologically identical
* Reported in Down’s syndrome
* Clinically may be confused with reticulated papillomatosis of Gougerot-Carteaud

Eccrine hidrocystomas
* Translucent papules 1-3mm
* May have bluish tint
* Usually solitary, however, multiple lesion may be seen
* Occur on the face
* May become more prominent in hot weather
* Treatment – excision
* Topical atropine or scopolamine

Eccrine poroma
* Benign, slow-growing, slightly protruding, sessile, soft, reddish tumor
* Most commonly occur on the sole or the side of the foot. May occur anywhere
* Bleeds with slight trauma
* Frequent cup-shaped shallow depression from which the tumor grows
* Benign – simple excision
* Eccrine poromatosis

Malignant eccrine poroma (porocarcinoma)
* Most arise from longstanding eccrine poromas (50%)
* Clinically similar
* May also manifest as a blue or black nodule, plaque or ulcerated tumor
* M=F, avg 70 yrs
* Legs 30%, feet 20%, face 12%, thighs 8%
* If metastatic, 70% mortality
* Mohs MS TOC

Chondroid Syringoma and Malignant Chondroid Syringoma
* Firm intradermal or subcutaneous nodule
* Most commonly located on the nose or cheeks
* 80 % involving the head and neck
* Symptomatic 5-30mm
* Felt to be of eccrine origin
* Malignant mixed tumor of the skin
* Most occur on extremities. Reported on face, scalp, back, buttocks
* Grow rapidly. Metastasis more the 50%
* Aggressive surgical excision, Adjuvant radiation therapy w/wo chemotherapy

Clear cell hidradenoma (nodular hidradenoma)
* Classified as an eccrine sweat gland tumor
* Single nodular, solid or cystic, occasionally protruding mass
* Flesh colored or reddish
* Anywhere. Most common site is the head
* 20% c/o pain on pressure
* Multiple lesions reported
* Women 2X men
* Extirpation is TOC

Malignant clear cell hidradenoma (hidradenocarcinoma)
* Extremely rare
* Presents as a solitary nodule
* Lower extremity 32.9 %, upper extremity 27.6 %, trunk 11.9 %, head 26.3 %
* Metastasis occurs 60%
* Tx wide local excision, radiation and chemotherapy

Eccrine spiradenoma
* Solitary, 1cm, deep-seated nodule
* Most frequently seen on the ventral surface
* Especially upper half of the body
* Skin-colored, blue or pink with normal overlying skin
* Multiple lesions, linear pattern may be seen
* Paroxysmal pain
* Benign clinical course
* Simple excision
* DDX may include
* A - angiolipoma
* N - neuroma
* G - glomus tumor
* E
* L – leiomyoma

Malignant eccrine spiradenoma
* In long standing lesions malignant degeneration may occur and my be lethal. Malignant Eccrine Spiradenoma

Papillary eccrine adenoma
* Uncommon benign lesion
* Dermal nodules
* Extremities of black patients
* Tendency to recur
* Complete surgical excision

syringoacanthoma
* Extremely rare (21 cases)
* Seborrheic keratosis-like neoplasm
* Significant tissue destruction if left untreated
* Classification remains controversial

Eccrine syringofibroadenoma
* Most presentations are a solitary, hyperkeratotic nodule or plaque involving the extremities
* Characteristic marker of Schopf syndrome
o Hydrocystomas of the eyelids, hypotrichosis, hypodontia, and nail abnormalities

cylindroma
* Dermal eccrine cylindroma, Spiegler’s tumor, turban tumor, and tomato tumor
* benign
* Predominately on scalp and face
* Solitary, firm but rubber-like nodule
* Pinkish to blue
* Few mm to several cm
* Women chiefly affected
* Grow slowly
* Rarely undergo malignant degeneration
* May be mistaken for epidermoid cyst
* excision
* Dominantly inherited form
* Numerous rounded masses of various sizes on the scalp
* Appears soon after puberty
* Resembles bunches of grapes or small tomatoes

Sweat gland carcinoma
* Eccrine carcinoma
o No characteristic clinical appearance
o High incidence of metastatic spread
* Mucinous eccrine carcinoma
o Commonly a round, elevated, reddish, and sometimes ulcerated mass
o Usually head and neck (75%)
o Slow growth and asymptomatic
o 11% incidence of metastasis
o Local excision

Aggressive digital papillary adenocarcinoma
* Aggressive malignancy involving the digit between the nail bed and the distal interphalangeal joint spaces in most cases
* Presents as a solitary nodule
* 50% recurrence rate
* Just under 50% develop metastasis
* All patients should have CXR
* Complete excision TOC
* Amputation may be required

Primary cutaneous adenoid cystic carcinoma
* Rare
* Presents usually on the chest or scalp
* Mohs MS TOC

Microcystic adnexal carcinoma (sclerosing sweat duct carcinoma)
* Generally a very slow-growing plaque or nodule
* Occurs most commonly on the upper lip of women
* Perineural infiltration is common and may be extensive
* TOC Mohs
* No reports of metastases

APOCRINE GLANDS
ceruminoma
* Rare apoeccrine tumor that rarely becomes malignant
* Firm nodular mass in the EAC
* Ulceration and crusting may occur
* Obstruction
* Questionable true entity
* Treatment - excision

Hidradenoma papilliferum
* Benign solitary tumor
* Almost exclusively on the vulva
* Bleeding, ulceration, discharge, itching and pain
* Firm nodule few mm
* excision

Syringadenoma papilliferum (syringocystadenoma papilliferum)
* Most commonly develops in a nevus sebaceous of Jadassohn
* Scalp or face
* Firm rose red papules
* Groups
* Vesicle-like inclusions are seen
* May simulate MC
* Transition to carcinoma is rare
* Excision is advised

Apocrine hidrocystoma/cystadenoma (apocrine retention cyst)
* Benign tumor
* Occurs chiefly on the face. solitary
* Penile shaft- median raphe cyst
* Dome-shaped, smooth-surfaced translucent nodule
* Bluish or brownish
* Simple excision

Apocrine gland carcinoma
* Rare
* Axilla is the most common site
* May be seen in the nipple, vulva and EAC
* May originate from aberrant mammary glands
* Widespread metastases may occur

HAIR FOLLICLE NEVI AND TUMORS
Pilomatricoma (calcifying epithelioma of Malherbe)
* Usually a single tumor
* Most commonly on the face, neck or arms
* Deeply seated firm nodule, covered with normal or pink skin
* Asymptomatic
* Stretching may show “tent sign”
* Derived from hair matrix cells
* Clinical DDX is impossible
* Simple excision
* Familial patterns do occur
* Multiple in Rubinstein-Taybi and Gardner syndrome

pilomatricoma
Malignant pilomatricoma
* Extremely rare
* Do not behave aggressively

Trichofolliculoma
* Benign, highly structured adenoma of the pilosebaceous unit
* Small dome-shaped nodule on the face or scalp
* A small wisp of fine, immature hairs protrude from a central pore
* Simple excisional bx

Trichoepithelioma (epithelioma adenoides cysticum, multiple familial trichoepitheliomas)
* Occur as multiple cystic and solid nodules typically on the face
* Small, rounded, smooth, shiny,slightly translucent and firm.
* Flesh colored or slightly reddish
* Slightly depressed center
* Often grouped and symmetrical
* benign
* Solitary trichoepithelioma
o Nonhereditary
o Mostly on face
* Giant solitary trichoepithelioma
o May reach several cm
o Mostly on thigh and perianal
* Desmoplastic trichoepithelioma
o Difficult to differentiate from morphea-like BCC
o Solitary or multiple on the face

trichoblastoma
* Benign neoplasms of follicular germinative cells
* Asymptomatic
* Scalp and face
* Surgical excision

Trichilemmoma and Cowden’s disease (multiple hamartoma syndrome)
* Benign neoplasm of the hair follicle
* Small solitary papule on the face
* Nose and cheeks
* Multiple
o Marker for Cowden,s syndrome
* Generally limited to the head and neck
* 87% of patients with Cowden’s
* 38% develop malignancies
o Breast 25-36%
o Thyroid 7%
o Colon adenocarcinoma
* Tumor suppressor gene

Trichilemmal carcinoma
* Sun exposed areas
* Face and ears
* Slow growing epidermal papule, indurated plaque or nodule with tendency to ulcerate
* Surgical excision

Trichodiscoma and fibrofolliculoma
* Hundreds of flat or dome-shaped, skin-colored asymptomatic papules
* Face, trunk and extremities
* Autosomal dominant trait
* Controversial entity
* 2-4 mm skin-colored to white papules
* Solitary, more commonly multiple
* Scattered over the face, trunk and extremities

Proliferating trichilemmal cyst
* Large exophytic neoplasms
* Almost exclusively confined to scalp and back of neck
* May ulcerate
* Ass with nevus sebaceous
* Metastasis may occur
* Most respond to surgical excision

Dermoid cyst
* Congenital in origin
* Chiefly along lines of cleavage
* Result from improper embryologic development
* Potential for intracranial communication
* CT or MRI scan is required to rule this out prior to BX over cranial cleavage planes
* Freely mobile and not attached to the skin

Pilonidal cyst
* Midline hairy patch or pit in the sacral region with a sinus orifice in the bottom, or a cyst beneath it
* Usually becomes symptomatic during adolescence
* Opening cyst widely, debriding it, and packing it with silver nitrate crystals
* More advanced surgical intervention may be required
* SCC has been reported to arise from chronic inflammatory pilonidal disease

Pilonidal sinus
Steatocystoma simplex
* Noninheritable counterpart to the more familiar steatocystoma multiplex
* Face limbs or chest
* Simple excision

Steatocystoma multiplex
* Multiple, small, yellowish, cystic nodules 2-6 mm
* Principally on the upper anterior trunk, upper arms, axillae and thighs
* Lesions may be generalizes
* High familial tendency
* Contain a syruplike, yellowish, odorless oily material
* Likely autosomal dominant inheritance
* Tx- excision of individual lesions
* Incision and expression or aspiration

Eruptive vellus hair cysts
* Autosomal dominant inheritance
* Yellowish to reddish brown, small papules of the chest and proximal extremities
* Disseminated lesions reported

Pigmented follicular cysts
* Face or neck
* Suggested to be a variant of multiple pilosebaceous cysts

milia
* White keratinous cysts, 1-4 mm
* Chiefly on the face esp under eyes
* May occur in great numbers
* Occur in up to 50 % of newborns
* Primarily develop without a predisposing condition
* Can develop in inflammatory conditions and skin diseases such as epidermolysis bullosa, pemphigus, bullous pemphigoid, PCT, herpes zoster, contact dermatitis, and after prolonged use of NSAIDS
* Variants include MEM (multiple eruptive milia)
* MEP (milia en plaque)
* Tx- incision and expression
* Tretinoin and minocycline for MEP

Pseudocyst of the auricle
* Fluctuant, tense, noninflammatory swelling of the upper ear
* Believed to be ass with trauma
* Tx – drainage
* ILI steroid

Cutaneous columnar cysts
* Four types of cyst that occur in the skin are lined by columnar epithelium
* Branchiogenic cyst
o Small solitary lesions just above the sternal notch
* Thyroglossal duct cysts
o Anterior aspect of the neck
o Malignancies reported 1%
* Cutaneous ciliated cysts
o Usually located on the legs of females
o Perineum vulva and foot regions
* Median raphe cyst
o Developmental defects lying in the ventral midline of the penis, usually on the glans
o Surgical intervention is standard therapy


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Dermatologic Procedures: Pearls and Pitfalls

Dermatologic Procedures: Pearls and Pitfalls
By: Daniel J. Ladd, Jr., D.O.
Dermatology Resident, KCOM

Financial Disclosure
* Lecture sponsored by DERMIK
* Very generous considering content of lecture has little or nothing to do with their products.
* BENZACLIN for ACNE
* PENLAC for ONYCHOMYCOSIS

BENZACLIN BID for ACNE
* SAFE
* EFFECTIVE
* EASY TO USE
* ACNE takes 8W
* Treating ACNE is like brushing TEETH

PENLAC QD FOR ONYCHOMYCOSIS
* SAFE
* EFFECTIVE
* EASY TO USE
* NO DRUG INTERACTION WORRIES
* NO LFT’S
* NO CHF WORRIES

Common Procedures
* Shave Biopsy
* Punch Biopsy
* Excisional Biopsy
* Cryosurgery

Pearl #1

* Pearl: General rule of thumb is to shave a tumor and punch a rash.
* Pitfall: A shave biopsy of a deep melanoma destroys the prognosis/Breslow’s thickness. Result: Now you must assume the worst and put the patient through extensive surgeries and chemotherapy. Moral: Fully excise or refer all suspected melanomas.

Pearl #2
* Pearl: Know where your biopsy is going. Always specify “must be diagnosed by a dermatopathologist”.
* Pitfall: If you do not specify as above it will go to a general pathologist. They may give you less than ideal diagnostic information or even miss the diagnosis. Your patient will not be impressed.

Pearl #3
* Pearl: Communicate with your dermatopathologist; “asymptomatic scaling erythematous annular plaques with central clearing localized to the bilateral shins for 2 weeks, consider tinea vs. granuloma annulare vs. necrobiosis lipoidica” = high yield
* Pitfall: “itchy rash, leg” = low yield

Pearl #4
* Pearl: When the patient asks “what do you think it (the lesion) is?”, the correct answer is “If I knew that I wouldn’t have to do the biopsy”.
* Pitfall: Never attempt to reassure the patient by saying the lesion is “probably going to be nothing at all”, they’ll wonder why you’re putting them through all of this.

Local Anesthesia
* “Doc, will this hurt?”
* “I’m not sure, they’ve only let me try this on animals so far”
* “No, it shouldn’t hurt me a bit”
* “More than a tickle but less than paying taxes”
* Pearl: fears of epinephrine induced necrosis at distal sites (nose, ears, penis, toes, fingertips) are largely unfounded.
* Pitfalls: patients with severe peripheral vascular disease, diabetic angiopathy and Raynaud’s phenomenon may be exceptions to the rule.

Pearl #5
* Local Anesthesia:
* Pearl: INJECT SLOWLY and your patients will love you forever. Decreases pain more than warming or adding bicarbonate.
* Pitfall: ALWAYS make sure they are lying down, especially the patient who “talks tough”.

Pearl #6
* Local Anesthesia
* Pearl: It is OK to give Xylocaine to patients who had allergic reactions to Novocaine at the dentist’s office, Lidocaine is an Amide and Novocaine is an Ester.
* Pitfall: They may not know which medication they reacted to: use Bacteriostatic NS when in doubt.

Pearl #7
* Local Anesthesia
* Pearl: For pediatric patients, let them sit in the lobby with ELA-Max or EMLA covered with Saran Wrap for 30 minutes.
* Pitfall: The above may fail. At this point either refer or insert earplugs and proceed. Remember: very few pediatric rashes will require biopsy for diagnosis.

Pearl #8
* Pearl: Insert needle at a 30 degree angle and slowly retract the needle as you inject the anesthetic. When the tissue blanches you are at the right level.
* Pitfall: If you see a linear trail of blanched skin radiating from the injection site you are probably in a vessel.

Pearl #9
* Regarding Coumadin.
* Pearl: Do not take patients off Coumadin to perform a small dermatologic procedure such as biopsy, excision or Moh’s surgery.
* Pitfalls: Depend on the reason why they are on Coumadin in the first place. Also problematic if you do not have tools for hemostasis.

Hemostasis
* Chemical
* Electrical
* Physical

Chemical Hemostasis
* Drysol
* Aluminum Chloride
* Quick, easy, cheap.
* Q-tip application.
* No odor or discoloration.
* Good for superficial biopsy - shave.
* Monsel’s solution.
* 20% ferric subsulfate.
* Cheap, easy to use.
* Risk of tattooing.
* Superficial only!
* Caustic, may destroy connective tissue if sutured into wound.

High Frequency Electrosurgery
* Monoterminal elecrodessication- low levels of current.
* Risk of Bradycardia or Asystole in patients with Pacemakers or Defibrillators.
* Requires dry field.

Electrocautery
* Heated metal results in tissue dessication, coagulation and necrosis.
* Safe to use in patients with pacemakers.
* Does not require a dry field.

Shave Biopsy
* Sterile #15 blade
* 4x4’s
* Drysol solution
* Sterile Q-tips
* Path specimen container

Shave Biopsy - skin tension
Shave Biopsy - flush with surface
* Endpoint is “pinpoint bleeding”
* Indicates you are at the level of the papillary dermis
* This is where scarring begins and patient satisfaction decreases.
* Pearl: Stay superficial and you can achieve minimal scarring.
* Pink atrophic area has a full year to heal.
* Pitfalls: Skin of upper chest and back scars no matter what. Same with Keloid prone pts.

Punch Biopsy
* Sterile procedure!
* Sterile gloves
* 3 or 4 mm Punch
* 4x4s, Drysol, Q-tips
* Needle driver, forceps
* Suture
* Path specimen bottle
* Twist punch tool until buried to the hub*
* *Caveat: Have a firm grasp of anatomy and skin thickness in the area you are punching before you punch it.
* Finger tendons, facial and neck structures.
* Hemostasis works best in 2 steps.
* First use the Q-tip to buy time to grab needle driver and suture.
* Suture so that closure is low tension - simple palpation reveals.
* Use 6-0 Prolene on the face.
* 4-0 Prolene most other areas.
* Silk for mucosal areas.
* 2 simple interrupted sutures.
* Out 7d face, 10d otw

Excisional Biopsy
* Pearl: If you suspect melanoma excisional biopsy DOWN TO FAT.
* Pitfalls: Punch biopsy, while deep enough is NOT representative of the entire lesion. Shave too shallow, prognosis destroyed.
* Pitfalls: Excision takes more time, reimbursement same, but medicolegally still a bargain because it is the standard of care.
* Using a Sharpie felt tip pen mark a circle around lesion with about 1-2 mm margins around clinically apparent lesion.
* Ellipse should be 3 times longer than circle around lesion.
* Pearl: Try to postion the final suture line within existing wrinkle lines / least tension.
* Whether lesion is malignant or not, your patient will never forget their scar.
* Sterile procedure!
* H2O2 and Betadine
* Pearl: Try not to apply the above too aggressively or to get excess Xylocaine on your ellipse drawing
* Pitfall: ink will rinse away, now you’re lost!

Pearl # 10 : Danger Zones

Pitfall #10: Facial Nerve Damage
* Temporal branch - forehead and eyebrow ptosis, may obstruct vision.
* Zygomatic branch - impaired blinking, eye dries out, clarity of vision is affected.
* Buccal branch - drooping corner of mouth,
* Marginal Mandibular - lower lip function.

BENZACLIN BID for ACNE
* SAFE
* EFFECTIVE
* EASY TO USE
* ACNE takes 8W
* Treating ACNE is like brushing TEETH

PENLAC QD FOR ONYCHOMYCOSIS
* SAFE
* EFFECTIVE
* EASY TO USE
* NO DRUG INTERACTION WORRIES
* NO LFT’S
* NO CHF WORRIES

Dermatologic Procedures: Pearls and Pitfalls.ppt

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Chronic Blistering Dermatoses

Chronic Blistering Dermatoses Part 2
By:David M. Bracciano, D.O.

Pregnancy- Related Dermatoses
* Intrahepatic Cholestasis of Pregnancy
* Polymorphic Eruption of Pregnancy
* Herpes (pemphigoid) gestationis
* purity Urticarial Papules and Plaques of Pregnancy (PUPPP)
* Papular Dermatitis of Pregnancy
* purity Folliculitis of Pregnancy

Intrahepatic Cholestasis of Pregnancy
* Generalized purities and jaundice
* No primary skin lesions, secondary excoriations
* Caused by cholestasis, occurs late in pregnancy, resolves after delivery
* 0.5% of pregnancies
* Tx; oral steroids

Polymorphic Eruption of Pregnancy
* Classification of all purity inflammatory dermatoses of pregnancy:
* Toxemic rash of pregnancy
* Pruigo annularis
* EM gestationis
* PUPPP
* purity Folliculitis of Pregnancy

Polymorphic Eruption of Pregnancy
* Pruritic inflammatory dermatoses of pregnancy occur in 1 of every 120 to 240
* Treatment and prognosis is similar in subtypes

Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)
* First reported in 1979
* Erythematous papules and plaques that begin as 1-2 mm lesions within the abdominal striae
* Spread over the course of a few days to involve the abdomen, buttocks, thighs
* Upper chest, face, and mucous membranes spared

PUPPP
* Lesions coalesce to form urticarial plaques
* Intense pruritis is characteristic
* Primigravidas 75% of the time, usually does not recur with subsequent pregnancies
* Begins late in third trimester and resolves with delivery
* May be associated with increase weight gain
* Histology: perivascular infiltrate in upper and mid dermis, epidermis normal
* Tx: topical or oral steroids

Papular Dermatitis of Pregnancy
* Pruritic generalized eruption of 3-5 mm erythematous papule surmounted by a small, firm, central crust
* May erupt at any time during pregnancy and resolve with delivery
* Marked elevation of urine HCG
* Tx; oral steroids, may recur in subsequent pregnancies

Prurigo Gestationis (Besnier)
* purity, excoriated papules of the proximal limbs and upper trunk
* Onset is 20-34 weeks gestation
* Clears in postpartum period and does not recur
* Tx: topical steroids

Pruritic Folliculitis of Pregnancy
* 2nd or 3rd trimester
* Small follicular pustules scattered widely over the trunk
* May be a type of hormonally induced acne

Impetigo Herpetiformis
* Form of severe pustular psoriasis occurring in pregnancy
* Acute, usually febrile onset of grouped pustules on an erythematous base
* Begins in the groin, axillae, and neck
* Increased WBC, hypocalcemia
* Recurs with pregnancy, fetal death due to placental insufficiency
* Tx; prednisone 1mg/kg

Cicatricial Pemphigoid (Benign Mucosal Pemphigoid)
* Vesicles which quickly rupture, leaving erosions and ulcers with scarring
* Primarily occur on mucous membranes, conjunctiva (66%) and oral mucosa (90%)
* Oral mucosa may be the only affected site for years; desquamative gingivitis of buccal mucosa
Cicatricial Pemphigoid
* Tends to affect middle-aged to elderly women 2:1 female/male
* Ddx; oral lichen planus (biopsy and IF)
* Chronic disease that may lead to slowly progressive shrinkage of the ocular mucous membranes and blindness
* Also occurs in pharynx, esophagus, larynx, nose, penis, vagina, anal mucosa, deafness
* Cutaneous lesions in 25%; tense bullae
* Bullae heal with or without scarring, occur on the face, scalp, neck, and inguinal region and extremities
* Some pts may have antibodies targeted against classic bullous pemphigoid antigens and should be classified as “mucosal predominate bullous pemphigoid”
* Chronic course, pts health not usually affected
* IgA antibodies may explain mucosal scarring tendency
* Little tendency to remission (unlike bullous pemphigoid)
* Subtypes include types that target basement membrane zone antigens (laminin, glycoproteins, )
* Direct IF testing C3 and IgG at the lamina lucida in 80-95%
* Tx: mild cases topical steroids (Temovate/Orabase), intralesional triamcinolone every 2-4 weeks
* Tx: Dapsone, prednisone, Azathioprine or cyclophosphamide

Epidermolysis Bullosa Acquisita
* Antibodies to Type VII collagen
* Skin fragility, healing with scars
* Bullous eruption, scaring, milia
* Need to exclude all other bullous diseases: porphyria cutanea tarda, pemphigoid, pemphigus, dermatitis herpetiformis, and bullous drug eruption

Epidermolysis Bullosa Acquisita
* Tx; unsatisfactory, steroids, dapsone, colchicine, IV Immunoglobulin, Cyclosporin

Dermatitis Herpetiformis
* Chronic, relapsing, severely purity disease
* Grouped symmetrical, polymorphous, erythematous-based lesions
* May be papular, papulovesicular, vesiculobullous, bullous, or urticarial
* Itching and burning are intense
* Spontaneous remissions lasting a week

Dermatitis Herpetiformis
* Eruption usually symmetrical
* Scalp, nuchal area, posterior axillary folds, sacral region, buttocks, knees, forearms
* Pruriginous papules are a common feature
* Vesicles are more common than bullae; however all types of these lesions may be present in one patient
* Course of the disease is generally lifelong, with prolonged remissions being rare

Dermatitis Herpetiformis
* Very few patients with DH ever have diarrhea although DH is associated with Gluten-sensitive-enteropathy (GSE)
* 87% of pts with DH and IgA deposits in the skin are HLA-B8 positive (like GSE)
* Gluten is a protein found in cereals except for rice, oats, and corn
* IgA antibodies are formed in the jejunum, may deposit in the skin
* Associated with; Thyroid disorders, small bowel lymphoma, non-Hodgkins lymphoma
* 70% of pts have abnormalities of the jejunal mucosa
* Gluten-free diet decreases Dapsone dose requirements after 3-4 months
* Ddx: pemphigoid, EM, scabies, contact dermatitis, atopic dermatitis, eczema, insect bites, pruigo nodularis
* IgA in a granular pattern in the dermal papillae in normal skin is specific and pathognomonic for DH
* IgA deposits may be focal, so multiple biopsies may be needed.
* Deposits of the antibody are more often seen in previously involved skin or normal appearing skin adjacent to involved skin
* Equal male:female
* Onset between 20 to 40 years
* Tx: Dapsone 50-300mg daily (hemolytic anemia, methemoglobinemia, check G6PD prior to tx) monitor Hct,WBCs, LFTs
* Tx: Sulfapyridine 0.5g QID to 2-4g/day
* Gluten-free diet will decrease need for meds or allow pt to go off them Celiac Society

Linear IgA Bullous Dermatosis
* Subepidermal blisters, a neutrophillic infiltrate, circulating IGA antibasement membrane zone antibody
* Deposition of IgA antibody at the dermoepidermal junction by direct IF

Linear IgA Bullous Dermatosis Adult Form
* Acquired autoimmune blistering disease
* Clinical pattern similar to dermatitis herpetiformis, or with vesicles and bullae in a bullous pemphigoid-like appearance
* 50% mucous membrane involvement
* Oral and conjunctival lesions may be scarring
* No association with enteropathy or with HLA-B8
* Tends to remit over several years

Linear IgA Bullous Dermatosis Adult Form
* Linear IgA dermatosis can occur as a drug-induced disease:
* Self-limited, less mucosal involvement, usually does not have circulating autoantibody
* IgA is usually deposited in the subbasal lamina area
* Vanco, Lithium, amiodarone, captopril, PCN, lasix, dilantin, and others
* Histo: papillary dermal microabscess with neutrophils, subepidermal bullae may be seen with neutrophils and eosinophils
* Direct IF: homogeneous linear deposition of IgA is present at the BMZ
* Indirect IF: few will have circulating IgA autoantibody with anti-BMZ specificity
* Tx: Dapsone, topical steroids

Linear IgA Bullous Dermatosis Childhood Form
* Chronic Bullous Disease of Childhood: acquired, self-limited bullous disease
* Onset by 2 or 3, remits by age 13
* Bullae develop on erythematous or normal appearing skin
* Trunk, buttocks, genitalia, and thighs
* Perioral and scalp lesions are common, oral lesions not uncommon
* Bullae arranged in a rosette or annular array “cluster of jewels”
* Histo: subepidermal bullae filled with neutrophils, eosinophils may predominate
* Direct IF: linear deposition of IgA at the BMZ
* Indirect IF: positive for circulating IgA antibodies in 50%
* Tx: Sulfapyridine or dapsone, topical steroids

Transient Acantholytic Dermatosis
* Over age 50, fragile vesicles, limited extent, sparse, limited duration
* Rapid crusting, keratotic erosion <1cm
* Usually chest, shoulder
* Direct IF is negative
* Tx: topical steroids, isotretinoin

Nutritional Diseases
* Caused by insufficiency or excess of dietary essentials
* Common in underdeveloped countries, infants and children
* Often pts have features of several disorders if diet is generally restricted
* Alcoholism is the main cause in developed countries
* Postoperative pts, psychiatric pts (anorexia nervosa, bulimia), surgical or inflammatory bowel dysfunction, Crohn’s

Hypovitaminosis A (Phrynoderma)
* Vitamin A: fat soluble found in milk, fish oil, liver, eggs, and as carotenoids in plants
* Common in children in developing world
* Developed countries found in diseases of fat malabsorption; Crohn’s, celiac, cystic fibrosis, cholestatic liver disease
* Vitamin A required for keratinization of mucosal surfaces
* Abnormal keratinization leads to increased mortality from inflammatory disease of the gut and lung ie; diarrhea and pneumonia
* Phrynoderma or “toadskin” resembles keratosis pilaris.
* Keratotic papules over extremities and shoulders arising from pilosebaceous follicles
* Eruption begins on thighs or upper arms. Spreads to shoulders, abdomen, back, and buttocks, face and neck
* Skin displays dryness and scaling

Hypovitaminosis A Ocular Findings
* Major cause of blindness in children in the developing world!
* Earliest finding is delayed adaptation to the dark (nyctalopia)
* Night blindness, xeropthalmia, xerosis corneae, keratomalacia
* Bitot’s Spots; circumscribed areas of xerosis of the conjuctiva lateral to the cornea

Hypovitaminosis A
* Diagnosis: based on eye findings, serum Vitamin A level.
* Tx: 300,000 IU Vitamin A

* Skin findings similar to side effects of Retinoid therapy. Children are at greater risk.
* Loss of hair and coarseness, loss of eyebrows, exfoliation and pigmentation of skin, clubbing, hepatosplenomegaly, anemia, increased LFTs, pseudotumor cerebri with papilledema

Hypervitaminosis A Adults
* Early signs are dryness of the lips and anorexia. Followed by bone and joint pains, follicular hyperkeratosis, branny desquamation of the skin, loss of scalp hair and eyebrows, dystrophy of the nails.
* Fatigue, myalgia, depression, anorexia, liver disease
* Birth defects with excess Vit A in pregnancy

Vitamin D
* Deficiency of Vitamin D causes alopecia, osteomalacia
* Vitamin D overdose can cause hypercalcemia and calcinosis.

Vitamin E Deficiency
* Most common in infants of low birth weight
* Peripheral edema, progressive neuromyopathy, and ophthalmoplegia

Vitamin K Deficiency
* Dietary deficiency of vitamin K, a fat soluble vitamin, does not occur in adults because it is synthesized by bacteria in the large intestine
* Liver disease causes deficiency
* Drugs: coumadin, salicylates, cholestyramine
* Decrease in the vitamin K-dependent clotting factor II, VII, IX, and X.
* Purpura, hemorrhage, and ecchymosis.
* Tx: 5 to 10 mg/day IM Vit K for 2-3 days

Vitamin B1 Deficiency
* Thiamine deficiency results in Beriberi
* Edema, and peripheral neuropathy

Vitamin B2 Deficiency
* Riboflavin deficiency is seen most often in alcoholics.
* Phototherapy for neonatal icterus, boric acid ingestion, hypothyroidism, chlorpromazine
* Oral-ocular-genital Syndrome: angular chelitis, atrophic tongue, photophobia, blepharitis, confluent dermatitis of scrotum
* Tx: 5mg Riboflavin qd

Vitamin B6- Pyridoxine
* Deficiency: occurs in uremia and cirrhosis
* Seborrheic dermatitis, glossitis, chelitis, conjunctivitis, confusion, neuropathy
* Excess: subepidermal vesicular dermatosis, peripheral sensory neuropathy

Vitamin B12 Deficiency Cyanocobalamin
* Absorbed through the distal ileum after binding to gastric intrinsic factor in an acid ph.
* Deficiency caused by: decreased intrinsic factor, achlorhydria, malabsorption syndromes (pancreatic, sprue)
* Because of large body stores in adults, deficiency occurs 3 to 6 years after onset of GI disease!
* Glossitis, hyperpigmentation accentuated in exposed areas resembling Addison’s disease
* Megaloblastic anemia, weakness, paresthesias, ataxia
* Tx: IM B12, neuro defects may not improve

Folic Acid Deficiency
* Diffuse hyperpigmentation, glossitis, chelitis, and megaloblastic anemia

Scurvy Vitamin C Deficiency
* Most common vitamin deficiency dxd by dermalologists
* Elderly alcoholics and psychiatric pts

Scurvy “The Four H’s”
* Hemorrhagic signs
* Hyperkeratosis of the hair follicles
* Hypochondriasis
* Hematologic abnormalities
* Perifollicular petechiae and ecchymoses, subungual, subconjunctival, intramuscular, and intraarticular hemorrhage
* “Corkscrew hairs”; hairshafts are curled in follicles capped by keratotic plugs
* Hemorrhagic gingivitis; bleeding gums, epistaxsis, anemia
* Dx: serum ascorbic acid level
* Tx: ascorbic acid 800-1000mg qd x 1 week

Niacin Deficiency Pellagra
* Nicotinic acid, vitamin B3, niacin or its precursor tryptophan is associated with a diet entirely composed of corn, millet or sorghum
* Other vitamin defficiencies or malnutrition coexist
* Most cases are alcoholics in developed countries

Pellegra Causes
* Carcinoid tumors, which divert tryptophan to serotonin
* Intestinal parasites esp; hookworm
* GI diseases ie; Chron’s
* IV alimentation
* Anorexia nervosa
* Meds; Isoniazid, azathioprine, 5-FU, Hydantoins

Pelegra
* Chronic disease affecting GI tract, CNS, skin
* “3 D’s”; diarrhea, dementia, dermatitis
* Dermatitis: photosensative eruption, perineal lesions, thickening and pigmentation over boney prominences, seborrheic dermatitis-like eruption on face

* Photosensitive eruption on face, neck, chest
* (Casal’s necklace), eruption may be vesicular or bullous (wet pellegra)
* After several phototoxic events the skin shows hyperpigmentation, scaling, a copper hue
* Scrotal and perineal erosions, fissures, angular chelitis
* CNS and GI symptoms may occur without skin changes; apathy, muscle weakness, parasthesias, dizziness, psychosis
* Disease is progressive, majority of pts die in 4-5 years if untreated

Pellegra Diagnosis and Treatment
* Diet: Animal protiens, eggs, milk, vegetables
* 100mg nicotinamide qid
* Skin lesions begin to resolve within 24 hours of tx

Biotin Deficiency
* Biotin is universally available and is produced by intestinal bacteria
* Deficiency is rare, can occur in short gut or malabsorption
* Dermatitis is perioral; pathcy, red, eroded lesions on the face and groin
* Candida overgrowth of lesions occurs
* Alopecia including loss of eyebrows and eyelashes
* Neuro: depression, lethargy, parasthesias
* Infants: hypotonia, lethagry seizures, developmental delays
* Inherited form: detecting organic aminoaciduria with 3-hydroxyisovaleric acid
* Tx: 10mg Biotin qd Skin lesions resolve rapidly, but neuro damage may be permenant

Zinc Deficiency
* Inherited or Aquired
* Inherited: Acrodermatitis enteropathica
* Premies at risk due to inadequate body zinc stores
* Weaning from breast from breast milk precipitates clinical zinc deficiency
* Parental nutrition without adequate zinc content may contribute

Zinc
* Acquired: alcoholics, bowel disease, anorexia, AIDS
* Zinc requirements increase with metabolic stress
* Diets containing mainly cereal grains are high in phytate, which binds zinc, Middle East, North Africa

Zinc Dermatitis
* Pustular and bullous, acral and perioral
* Patchy, red, dry, scaling with exudation and crusts. Angular chelitis and stomatitis
* Nail dystrophy, alopecia
* Diarrhea, growth retardation, CNS
* Histo: vacuolation of the keratinocytes of the upper stratum malpighii

Zinc Deficiency Diagnosis and Treatment
* Characteristic skin findings, acral or perioral dermatitis
* Chronic diaper rash with diarrhea in an infant should lead to evaluation for zinc deficiency
* Diagnosis: low serum zinc, alkaline phosphatase
* Tx: zinc sulfate 1-2 mg/kg/day
* Tx: acrodermatitis enteropathica is lifelong

Essential Fatty Acid Defficiency
* Lbw infants, bowel disease, alimentation
* Dermatitis similar to zinc def : xerosis, EFA’s constitute 25% of the fatty acids of the stratum corneum
* Widespread erythema, intertriginous weeping eruption, infection, alopecia
* Decrease in linoleic acid and an increase in palmitoleic and oleic acids
* Ratio of eicosatrienoic acid to arachidonic acid of >0.4 is diagnostic
* Tx: Intralipid 10% IV

Iron Deficiency
* Common in menstration
* Mucocutaneous; glossitis, angular chelitis, pruitus, telogen effluvium
* Plummer-Vinson syndrome: microcytic anemia, dysphagia, glossitis (middle aged women) thin lips, narrow mouth, koilonchia in 50%
* Post-cricoid esophageal web
* Diagnosis: serum iron (Fe+)
* Tx: iron sulfate 325 mg tid

Selenium Deficiency
* IV alimentation, poor soil selenium content, lbw infants
* Children: hypopigmentation of skin and hair (psuedoalbinism), leukonychia
* Cardiomyopathy, muscle pain, elevated muscle enzymes (cpk)
* Tx: 3 ug/kg/day selenium

Protein-energy Malnutrition
* Spectrum of diseases: marasmus, kwashiorkor, and marasmic kwashiorkor
* Endemic in developing world
* Marasmus; def of protein and calories, children < 60% of IBW without edema
* Kwashiorkor; protein def, 60-80% of IBW with edema or hypoproteinemia

Marasmus/ Kwashiorkor
* Cystic fibrosis, dietary restrictions
* Marasmus: skin is dry, wrinkled, loose
* “Monkey facies”; due to lose of buccal fat pad, no edema
* Kwashiorkor; edema, potbelly, hair and areas of skin are hypopigmented, hair is red, gray to white
* Africans call them “Red Children”
* “Flag Sign”; alternating bands of pale and dark hair along a single strand correspond to periods of good and poor nutrition
* “Mosaic skin”; areas of hyper/hypopigmentation resemble peeling paint

Carotenemia and Lycopenemia

* Excessive ingestion of : carots, oranges, squash, spinach, turnips, corn, beans, butter, eggs, pumpkins, sweet potatoes, papaya (seen in Kirksville)
* Yellowish discoloration of skin, palms, soles, central face
* Carotenemia occurs in vegitarians
* Lyconpenemia; red foods, beets, tomatoes, chili beans (flatulence), berries leads to reddish discoloration of skin aka “K.C. Chiefs’ syndrome


Chronic Blistering Dermatoses.ppt

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Abnormalities In Dermal Connective Tissue

Abnormalities In Dermal Connective Tissue
By: Erik Austin, D.O., M.P.H.

Elastosis perforans serpiginosa – Serpiginous arrangement of confluent, keratotic papules on the arms, face/neck, legs

Keratotic papules of EPS
Typical site affected = neck
Elastosis perforans serpiginosa
EPS
* MC in young adults with a M:F ratio of 4:1
* Runs a variable course of 6 mos to 5 years with spontaneous resolution
* Associated with: Down Syndrome, Ehlers-Danlos, osteogenesis imperfecta, Marfan’s, Rothmund-Thomson, acrogeria, systemic sclerosis
* Tx = LN2, Penicillamine
* Annular plaques of EPS
* Atrophic scars often form
* Hyperelastic epidermis that clutches the increased dermal elastic fibers like a claw
* Transepidermal elimination of neutrophils and elastic fibers from the dermis through a channel in the epidermis

Reactive perforating collagenosis (RPC)
Keratotic papules on upper extremity, face or buttocks

Reactive perforating collagenosis
RPC
* Rare, familial, non-pruritic skin disorder
* Lesions begin in 2nd decade
* Involution occurs after 6-8 weeks, with new crops appearing for years
* May be a reaction to trauma
* Acquired form may be assoc. w/systemic dz
* TX = treat underlying disease

Pseudoxanthoma elasticum (PXE)
* Yellow papules, calcified plaques, sagging skin; chicken skin
* Inherited disorder of the skin, eyes, and cardiovascular system
* Has recessive and dominant inheritance
* Exaggerated nasolabial folds is characteristic
* Involvement of the cardiovascular system occurs with a propensity to hemorrhage

Mucosal lesions
* Retinal change = Angioid streaks; in up to 85%
* Mitral valve prolapse, 71% of 14 pts
* Young pt w/hypertension = r/o PXE
* Histo: mid-dermis w/elastic fibers that are swollen and granular - “raveled wool”
* No distinctive therapy
* Limit dietary calcium and phosphorus

Histopathology of PXE
* A. calcium deposits on elastic fibers in advanced PXE
* B. irregularly clumped elastic fibers, Verhoeff van Giesson

Perforating calcific elastosis
* Acquired, localized disorder
* Frequently found in obese, multiparous, middle-aged women
* Yellowish, lax, well circumscribed, reticulated or cobblestones plaques occur in the periumbilical region with keratotic papules
* Shares features with PXE, without systemic features
* Trauma of pregnancy, obesity or surgery promote elastic fiber degeneration
* No effective therapy

Ehlers-Danlos syndromes
* A group of genetically distinct disorders characterized by excessive stretchability and fragility of the skin
* Tendency toward easy scar formation, calcification of the skin to produce, pseudotumors, and hyperextensibility of the joints

Clinical features of Ehlers-Danlos syndrome
* Two types of growths seen with EDS
* Molluscum pseudotumor = a soft fleshy nodule seen in areas of trauma
* Spheroids = hard subcutaneous nodules that become calcified, ?Result of fat necrosis
* Types I, II, III and one subtype each of types of IV, VII and possibly VIII = AD
* One subtype of IV, VI, VII, and X = AR
* Type V = X-linked inheritance
* Treatment is supportive
* Avoidance of trauma

Marfan syndrome
* AD
* Skeletal, cardiovascular, and ocular involvement
* Important abnormalities include: tallness, loose-joints, a dolichocephalic skull, high arched palate, arachnodactyly, pigeon breast, pes planus, poor muscular tone, large deformed ears
* Ascending aortic aneurysm and mitral valve prolapse are commonly seen
* Ectopic lentis and striae
* Gene defect = chromosome 15
* Abnormal elastic tissue in fibrillin 1 and fibrillin 2

Cutis Laxa – loose, hanging skin – usually entire integument is involved
Cutis laxa (generalized elastosis)
* AD = primarily cutaneous, good prognosis
* AR = significant internal involvement, die young
* X-linked recessive = occipital horn syndrome
* Nonfamilial forms have been described
* May be associated with an underlying disease or inflammatory skin process
* Mid-dermal elastosis is an acquired, nonfamilial condition affecting primarily young women, cause unknown
* Tx = disappointing; surgery is unsuccessful

Cutis laxa (generalized elastosis)
* Premature aging, severe pulmonary emphysema, and fragmentation of dermal elastic fibers

Blepharochalasis
* Lax eyelid skin due to swelling of lids
* Uncommon
* AD
* Lack of elastic fibers, and abundant IgA deposits have been demonstrated
* Ascher Syndrome = progressive enlargement of the upper lip and blepharochalasis / treatment is surgical

Anetoderma (macular atrophy)
* A group of disorders characterized by looseness of the skin due to loss of elastic tissue
Anetoderma – macular atrophy and atrophic plaques – buttonhole sign. Typical location: trunk, arms, shoulders, thighs
* Anetoderma: decreased elastic fibers in the papillary and reticular dermis
Striae rubra, striae alba: depressed lines or bands

Striae distensae
* Can occur secondary to pregnancy or after sudden weight gain or muscle mass
* Associated with Cushing’s syndrome and
* Prolonged application of topical steroids
* Overtime striae become less noticeable
* Tx = topical tretinoin; vascular lasers

Linear focal elastosis (elastotic striae)
* Asymptomatic, palpable, striaelike yellow line of the middle and lower back
* Distinguished from striae in that there is no depression

Acrodermatitis chronica atrophicans
* Acquired diffuse thinning of the skin
* Reddish appearance on extensor surfaces
* Progresses to smooth , soft, atrophic skin
* Results from infection with Borrelia

Osteogenesis imperfecta
* Affects: bones, joints, eyes, ears, and skin
* types I-IV, I and IV = AD
* II and III = AD/AR
* 50% are type I
* type II is lethal within 1st week of life
* Brittle bones, fractures occur early in life, sometimes in utero
* Loose-jointedness and dislocations
* Blue sclera
* Deafness
* Thin skin; atrophic scars
* EPS has associated
* Defect is abnormal collagen synthesis, resulting in type I collagen of abnormal structure
* Major causes of death = respiratory failure and head trauma
* Type I and IV have a normal life span
* TX = Pamidronate

Homocystinuria
Inborn error in the metabolism if methionine
* Homocystine in the urine and CT abnormalities
* cystathionine synthetase deficient
* Genu valgum, kyphoscoliosis, pigeon breast, frequent fractures
* Facial skin has a characteristic flush
* Other skin is blotchy red
* Hair is fine, sparse and blonde
* Teeth are irregularly aligned
* Downward dislocations of lens
* TX = hydroxocobalamin and cyanocobalamin – variable results

ERRORS IN METABOLISM
SYSTEMIC AMYLOIDOSIS primary systemic amyloidosis
* Involves mesenchymal tissue, the tongue, heart, gastrointestinal, and skin
* Cutaneous manifestations in 40%
* Amyloid fibril proteins are composed of AL
* Derived from immunoglobulin light chains
* 90% will have fragment in urine and serum
* Waxy, firm, flat-topped or spherical papules
* Coalesce to form nodules and plaques
* Eyes, nose, mouth, and mucocutaneous junctions are commonly involved
* Purpuric lesions and ecchymosis (15%)
* Results from amyloid infiltration of vessels
* Glossitis with macroglossia (20%)
* May cause dysphagia
* Bullous disease is rare and scarring
* Subepidermal: DDx PCT and EBA
* Systemic findings: peripheral neuropathies, arthropathy, GI bleeding, cardiac disease
* Prognosis is poor, median survival 13 mos, 5 mos in myeloma associated cases
* Treatment is difficult = melphalen, prednisone, hematopoietic stem cell transplantation
* Macroglossia with dental impression of the tongue
* Periorbital ecchymosis, “raccoon sign”
Secondary systemic amyloidosis
* Amyloid involvement of adrenals, liver spleen, and kidney as a result of some chronic disease (TB, leprosy, etc.)
* Skin is not involved
* Amyloid fibrils are designated AA, protein component is unrelated to immunoglobulin
* Treat the underlying condition

CUTANEOUS AMYLOIDOSIS primary cutaneous amyloidosis
* Divided into macular and lichen amyloid
* Asian , Hispanic, and Middle Eastern
* Amyloid deposition contains keratin
* Histologic picture is similar for both
* Differ only in size of amyloid deposits
* Absence of amyloid deposits around blood vessels excludes systemic involvement
* Macular Amyloidosis: pruritic, brown macules with a rippled pattern

Lichen amyloidosis
* Pruritic, keratotic, hyperigmented plaques on the legs
* Tx = high potency corticosteroids, oral retinoids, cyclophosphamide, dermabrasion and occlusion
Extremities, trunk, genitals and face with localized nodules

* Lesions contain numerous plasma cells, amyloid is immunoglobulin-derived AL
* TX = physical removal or destruction

Secondary cutaneous amyloidosis
* Following PUVA therapy and in benign and malignant cutaneous neoplasms, deposits of amyloid may be found
* Most frequently associated neoplasms are NMSC and SKs
* In all cases, this is keratin-derived amyloid

Familial syndromes associated with amyloidosis (heredofamilial amyloidosis)
* Muckle-Wells syndrome
* MEN IIA
* Most present with neurologic disease and are now designated familial amyloidotic polyneuropathy
* Four types identified FAP I through IV
* AD inherited

PORPHYRIAS
* Porphyrinogens are the building blocks of hemoproteins
* Produced primarily in the liver, bone marrow and erythrocytes
* Each form is associated with a deficiency in the metabolic pathway of heme synthesis
* Absorption of UV radiation in the Soret band (400-410 nm) by the increased porphyrins leads to photosensitivity
* Activated porphyrins form reactive oxygen species that causes tissue damage

Current grouping of the porphyrias is based on the primary site of increased porphyrin production
* Erythropoietic forms
o Congenital erythropoietic porphyria (CEP)
o Erythropoietic protoporphyria (EPP)
o Erythropoietic coproporphyria ECP
* Hepatic forms
o Acute intermittent porphyria (AIP)
o ALA dehydrogenase deficiency
o Hereditary coproporphyria (HCP)
o Variegate porphyria (VP)
o Porphyria cutanea tarda

Porphyria cutanea tarda
* Most common porphyria
* Photosensitivity leads to bullae, which leads to ulcers, scarring, milia and dyspigmentation
* Hypertrichosis, fragility and skin thickening
* Alcoholism is common; Hep C in 94%
* Associated with DM, LE, HIV, and

estrogen therapy
* Multiple erosions with hemorrhagic crusts, as well as an intact blister on the lateral fourth finger

PCT in chronic renal failure
* Deficiency = uroporphyrinogen decarboxylase
* Most common = sporadic nonfamilial form, (80%), abnormal enzyme activity
* Presents in midlife
* Familial type = AD; deficiency in liver and RBCs
* Nonfamilial = acquired toxic; associated with exposure to hepatotoxins
* Diagnosis = suspected on clinical grounds
* Coral red fluorescence of urine
* 24 hour urine
* Uroporphyrins to coproporphyrins 3:1 to 5:1
* DIF shows IgG and C3 at the DEJ, and in the vessel walls in a linear pattern

Histologic features of PCT
* Subepidermal blister with minimal dermal inflammatory infiltrate. Festooning of dermal papillae.

treatment
* Remove environmental exposures
* Sunscreens
* Phlebotomy / uroporphyrinogen decarboxylase is inhibited by iron
o 500 ml at 2 week intervals, hemoglobin 10 g/dL
o Several months, 6-10 phlebotomies
* Antimalarials / full doses may produce severe hepatotoxic reaction
* Remission may last for years
* Iron chelation
* May respond to transplant in renal failure
* May improve with treatment if assoc. with Hep C

pseudoporphyria
* Skin and Histo similar to PCT
* Normal urine and serum porphyrins
* No hypertrichosis, dyspigmentation or cutaneous sclerosis
* Commonly caused by NSAIDs, naproxen, sunbed use, hemodialysis

treatment
* Sun protection
* Discontinue inciting medication
o May resolve over several months

Hepatoerythropoietic porphyria
* Very rare form / AR
* Deficiency of uroporphyrinogen decarboxylase, 10% of normal in both the liver and erythrocytes
* Dark urine at birth
* Vesicles, scarring, hypertrichosis, pigmentation, red fluorescence of teeth
* Abnormal urinary porphyrins as in PCT
* Elevated erythrocyte protoporphyrins
* Increased coproporphyrins

Hepatoerythropoietic porphyria
Acute intermittent porphyria
* Second most common form
* Characterized by periodic attacks of abdominal colic, gastrointestinal disturbances, paralyses, and psychiatric disorders
* No skin lesions are seen
* AD / deficiency in porphobilinogen deaminase
* Only 10 % develop disease, all are at risk for primary liver cancer
* Severe abdominal colic +/- NVDC
* Elevated urinary porphobilinogen
* Increased dALA in plasma and urine
* No specific treatment
* Avoid precipitating factors
* Glucose loading
* Hematin infusions
* Pain management
* Oral contraceptives may prevent attacks in women with premenstrual symptoms

Hereditary coproporphyria HCP
* Rare, AD
* Deficiency of coproporphyrinogen oxidase
* One third are photosensitive
* Prone to GI attacks
* Fecal coproporphyrin is always increased
* Urinary coproporphyrin, ALA, and PBG are only increased during attacks

Variegate porphyria VP
* AD
* Decreased activity of protoporphyrinogen oxidase
* Majority of relatives have silent VP
* Characterized by skin lesions of PCT and the GI and neurologic disease of AIP
* Suspect VP when finding indicate both PCT and AIP, esp. with history of South African ancestry
* Fecal coproporphyrins and protoporphyrins are always elevated
* During attacks, urine porphobilinogen and ALA are elevated
* Urinary coproporphyrins are increased over uroporphyrins
* A finding in the plasma of “X porphyrin,” fluorescence at 626 nm is characteristic and distinguishes this form from others
* Symptomatic treatment as for PCT and AIP

Erythropoietic protoporphyria EEP
* AD and AR forms
* Ferochelatase activity is 10 to 25% of normal in affected persons
* Typically presents in childhood, 2-5 years
* Burning of the skin upon sun exposure
* Elevated protoporphyrin IX absorbs both the Soret band and also at 500-600 nm
* Severe liver disease in 10%
* Excessive porphyrins are deposited in liver
* Diagnosis on clinical grounds
* Urine porphyrin levels are normal
* Erythrocyte protoporphyrin is elevated
* Erythrocyte, plasma, and fecal protoporphyrin can be assayed to confirm the diagnosis
* Skin biopsy confirms diagnosis
* Tx = sun protection
* Beta carotene, phototherapy, cysteine
* Transfusions for anemia

Erythropoietic protoporphyria
* Subtle scarring
Erythropoietic protoporphyria
* Erythema and hemorrhagic crusts
Congenital erythropoietic porphyria, CEP
* Gunther’s disease
* AR; defect of uroporphyrinogen III synthase
* Presents after birth with red urine
* Severe photosensitivity
* Blistering, scarring, ectropion and corneal damage
* Mutilating scars, hypertrichosis, profuse eyebrows, long eyelashes, “monkey face”
* Growth retardation, hemolytic anemia, thrombocytopenia, porphyrin gallstones, osteopenia
* Suspect in an infant with dark urine and photosensitivity

Congenital erythropoietic porphyria
* Erythrodontia
* Severe mutilation
* Fluorescence of circulating red blood cells, CEP with UVA
* Vs. transient fluorescence in EPP
* High amounts of uroporphyrin I and coproporphyrin I are found in the urine, stool and red cells
* Treatment – strict avoidance of sunlight and sometimes splenectomy for the hemolytic anemia
* Oral activated charcoal
* Repeated transfusions to maintain hematocrit level at 33% - turns off demand for heme
* Bone marrow transplantation
Transient erythroporphyria of infancy (purpuric phototherapy-induced eruption)

* Report of seven infants exposed to 380 to 700 nm blue lights, for the treatment of indirect hyperbilirubinemia, who developed marked purpura on the exposed skin
* All infants had received transfusions
* Elevated plasma coproporphyrins and protoporphyrins were found in 4
* Pathogenesis is unknown

Abnormalities In Dermal Connective Tissue.ppt

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Cardiac Assist Devices

Cardiac Assist Devices
By: Wayne E. Ellis, Ph.D., CRNA

Types
Pacemakers
AICDs
VADs

History
* First pacemaker implanted in 1958
* First ICD implanted in 1980
* Greater than 500,000 patients in the US population have pacemakers
* 115,000 implanted each year

Pacemakers Today
* Single or dual chamber
* Multiple programmable features
* Adaptive rate pacing
* Programmable lead configuration

Internal Cardiac Defibrillators (ICD)
* Transvenous leads
* Multiprogrammable
* Incorporate all capabilities of contemporary pacemakers
* Storage capacity

Temporary Pacing Indications
* Routes = Transvenous, transcutaneous, esophageal
* Unstable bradydysrhythmias
* Atrioventricular heart block
* Unstable tachydysrhythmias
* *Endpoint reached after resolution of the problem or permanent pacemaker implantation

Permanent Pacing Indications
* Chronic AVHB
* Chronic Bifascicular and Trifascicular Block
* AVHB after Acute MI
* Sinus Node Dysfunction
* Hypersensitive Carotid Sinus and Neurally Mediated Syndromes
* Miscellaneous Pacing Indications

Chronic AVHB
* Especially if symptomatic

Pacemaker most commonly indicated for:
* Type 2 2º
o Block occurs within or below the Bundle of His
* 3º Heart Block
o No communication between atria and ventricles

Chronic Bifascicular and Trifascicular Block
* Differentiation between uni, bi, and trifascicular block
* Syncope common in patients with bifascicular block
* Intermittent 3º heart block common

AVHB after Acute MI
* Incidence of high grade AVHB higher
* Indications for pacemaker related to intraventricular conduction defects rather than symptoms
* Prognosis related to extent of heart damage

Sinus Node Dysfunction
* Sinus bradycardia, sinus pause or arrest, or sinoatrial block, chronotropic incompetence
* Often associated with paroxysmal SVTs (bradycardia-tachycardia syndrome)
* May result from drug therapy
* Symptomatic?
* Often the primary indication for a pacemaker

Hypersensitive Carotid Sinus Syndrome
• Syncope or presyncope due to an exaggerated response to carotid sinus stimulation
• Defined as asystole greater than 3 sec due to sinus arrest or AVHB, an abrupt reduction of BP, or both

Neurally Mediated Syncope
* 10-40% of patients with syncope
* Triggering of a neural reflex
* Use of pacemakers is controversial since often bradycardia occurs after hypotension

Miscellaneous
* Hypertrophic Obstructive Cardiomyopathy
* Dilated cardiomyopathy
* Cardiac transplantation
* Termination and prevention of tachydysrhythmias
* Pacing in children and adolescents

Indications for ICDs
* Cardiac arrest due to VT/VF not due to a transient or reversible cause
* Spontaneous sustained VT
* Syncope with hemodynamically significant sustained VT or VF
* NSVT with CAD, previous MI, LV dysfunction and inducible VF or VT not suppressed by a class 1 antidysrhythmic

Device Selection
* Temporary pacing (invasive vs. noninvasive)
* Permanent pacemaker
* ICD

Pacemaker Characteristics
• Adaptive-rate pacemakers
•Single-pass lead Systems
• Programmable lead configuration
• Automatic Mode-Switching
• Unipolar vs. Bipolar electrode configuration

ICD selection
* Antibradycardia pacing
* Antitachycardia pacing
* Synchronized or nonsynchronized shocks for dysrhythmias
* Many of the other options incorporated into pacemakers

Approaches to Insertion
Mechanics
Unipolar Pacemaker
Bipolar Pacemaker
Indications
1. Sick sinus syndrome (Tachy-brady syndrome)
2. Symptomatic bradycardia
3. Atrial fibrillation
4. Hypersensitive carotid sinus syndrome
* Second-degree heart block/Mobitz II


Complete heart block
* Sinus arrest/block
* Tachyarrhythmias
Supraventricular, ventricular
To overdrive the arrhythmia
Atrial Fibrillation
1. Asynchronous/Fixed Rate
2. Synchronous/Demand
3. Single/Dual Chamber
4. Programmable/nonprogrammable
Synchronous/Demand
Examples of Demand Pacemakers
DDI
VVI/VVT
AAI/AAT
Disadvantage: Pacemaker may be fooled by interference and may not fire

Dual Chamber: A-V Sequential
Facilitates a normal sequence between atrial and ventricular contraction
Provides atrial kick + ventricular pacing
Atrial contraction assures more complete ventricular filling than the ventricular demand pacing unit
A-V Sequential
Disadvantage: More difficult to place
More expensive
Contraindication: Atrial fibrillation, SVT
Developed due to inadequacy of “pure atrial pacing”
Single Chamber
Atrial
Ventricular
“Pure Atrial Pacing”
Problems with Atrial Pacing
Electrode difficult to secure in atrium
Tends to float

Ventricular
Programmability
Table of Pacer Codes
Types of Pulse Generators
Examples
Other Information
Undersensing: Failure to sense ... much more in 105 slides

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Arrhythmia

ARRHYTHMIA
Edited by Yingmin Chen

* Definition of Arrhythmia:
The Origin, Rate, Rhythm, Conduct velocity and sequence of heart activation are abnormally.

Anatomy of the conducting system
Pathogenesis and Inducement of Arrhythmia
* Some physical condition
* Pathological heart disease
* Other system disease
* Electrolyte disturbance and acid-base imbalance
* Physical and chemical factors or toxicosis


Mechanism of Arrhythmia
* Abnormal heart pulse formation
* Sinus pulse
* Ectopic pulse
* Triggered activity
* Abnormal heart pulse conduction
* Reentry
* Conduct block

Classification of Arrhythmia
* Abnormal heart pulse formation
* Sinus arrhythmia
* Atrial arrhythmia
* Atrioventricular junctional arrhythmia
* Ventricular arrhythmia
* Abnormal heart pulse conduction
* Sinus-atrial block
* Intra-atrial block
* Atrio-ventricular block
* Intra-ventricular block
* Abnormal heart pulse formation and conduction

Diagnosis of Arrhythmia
* Medical history
* Physical examination
* Laboratory test

Therapy Principal
* Pathogenesis therapy
* Stop the arrhythmia immediately if the hemodynamic was unstable
* Individual therapy

Anti-arrhythmia Agents
* Anti-tachycardia agents
* Anti-bradycardia agents
Anti-tachycardia agents
* Modified Vaugham Williams classification
* I class: Natrium channel blocker
* II class: ß-receptor blocker
* III class: Potassium channel blocker
* IV class: Calcium channel blocker
* Others: Adenosine, Digital

Anti-bradycardia agents
* ß-adrenic receptor activator
* M-cholinergic receptor blocker
* Non-specific activator

Clinical usage
Anti-tachycardia agents:
* Ia class: Less use in clinic
* Guinidine
* Procainamide
* Disopyramide: Side effect: like M-cholinergic receptor blocker

Anti-tachycardia agents:
* Ib class: Perfect to ventricular tachyarrhythmia
1. Lidocaine
2. Mexiletine
Anti-tachycardia agents:
* Ic class: Can be used in ventricular and/or supra-ventricular tachycardia and extrasystole.

1. Moricizine
2. Propafenone

Anti-tachycardia agents:
* II class: ß-receptor blocker
* Propranolol: Non-selective
* Metoprolol: Selective ß1-receptor blocker, Perfect to hypertension and coronary artery disease patients associated with tachyarrhythmia.
* III class: Potassium channel blocker, extend-spectrum anti-arrhythmia agent.
* Amioarone: Perfect to coronary artery disease and heart failure patients
* Sotalol: Has ß-blocker effect
* Bretylium
* IV class: be used in supraventricular tachycardia
* Verapamil
* Diltiazem
* Others:
Adenosine: be used in supraventricular tachycardia

Anti-bradycardia agents
* Isoprenaline
* Epinephrine
* Atropine
* Aminophylline
Proarrhythmia effect of antiarrhythmia agents
* Ia, Ic class: Prolong QT interval, will cause VT or VF in coronary artery disease and heart failure patients
* III class: Like Ia, Ic class agents
* II, IV class: Bradycardia

Non-drug therapy
* Cardioversion: For tachycardia especially hemodynamic unstable patient
* Radiofrequency catheter ablation (RFCA): For those tachycardia patients (SVT, VT, AF, AFL)
* Artificial cardiac pacing: For bradycardia, heart failure and malignant ventricular arrhythmia patients.

Sinus Arrhythmia

Sinus tachycardia
* Sinus rate > 100 beats/min (100-180)
* Causes:
* Some physical condition: exercise, anxiety, exciting, alcohol, coffee
* Some disease: fever, hyperthyroidism, anemia, myocarditis
* Some drugs: Atropine, Isoprenaline
* Needn’t therapy
Sinus Bradycardia
* Sinus rate < 60 beats/min
* Normal variant in many normal and older people
* Causes: Trained athletes, during sleep, drugs (ß-blocker) , Hypothyriodism, CAD or SSS
* Symptoms:
* Most patients have no symptoms.
* Severe bradycardia may cause dizziness, fatigue, palpitation, even syncope.
* Needn’t specific therapy, If the patient has severe symptoms, planted an pacemaker may be needed.
Sinus Arrest or Sinus Standstill
* Sinus arrest or standstill is recognized by a pause in the sinus rhythm.
* Causes: myocardial ischemia, hypoxia, hyperkalemia, higher intracranial pressure, sinus node degeneration and some drugs (digitalis, ß-blocks).
* Symptoms: dizziness, amaurosis, syncope
* Therapy is same to SSS
Sinoatrial exit block (SAB)
* SAB: Sinus pulse was blocked so it couldn’t active the atrium.
* Causes: CAD, Myopathy, Myocarditis, digitalis toxicity, et al.
* Symptoms: dizziness, fatigue, syncope
* Therapy is same to SSS

Sinoatrial exit block (SAB)
* Divided into three types: Type I, II, III
* Only type II SAB can be recognized by EKG.

Sick Sinus Syndrome (SSS)
* SSS: The function of sinus node was degenerated. SSS encompasses both disordered SA node automaticity and SA conduction.
* Causes: CAD, SAN degeneration, myopathy, connective tissue disease, metabolic disease, tumor, trauma and congenital disease.
* With marked sinus bradycardia, sinus arrest, sinus exit block or junctional escape rhythms
* Bradycardia-tachycardia syndrome

Sick Sinus Syndrome (SSS)
* EKG Recognition:
* Sinus bradycardia, ≤40 bpm;
* Sinus arrest > 3s
* Type II SAB
* Nonsinus tachyarrhythmia ( SVT, AF or Af).
* SNRT > 1530ms, SNRTc > 525ms
* Instinct heart rate < 80bmp

Sick Sinus Syndrome (SSS)
* Therapy:
* Treat the etiology
* Treat with drugs: anti-bradycardia agents, the effect of drug therapy is not good.
* Artificial cardiac pacing.

Atrial arrhythmia
Premature contractions
* The term “premature contractions” are used to describe non sinus beats.
* Common arrhythmia
* The morbidity rate is 3-5%
Atrial premature contractions (APCs)
* APCs arising from somewhere in either the left or the right atrium.
* Causes: rheumatic heart disease, CAD, hypertension, hyperthyroidism, hypokalemia
* Symptoms: many patients have no symptom, some have palpitation, chest incomfortable.
* Therapy: Needn’t therapy in the patients without heart disease. Can be treated with ß-blocker, propafenone, moricizine or verapamil.

Atrial tachycardia
* Classify by automatic atrial tachycardia (AAT); intra-atrial reentrant atrial tachycardia (IART); chaotic atrial tachycardia (CAT).
* Etiology: atrial enlargement, MI; chronic obstructive pulmonary disease; drinking; metabolic disturbance; digitalis toxicity; electrolytic disturbance.........

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Cardiac Arrhythmias

Cardiac Arrhythmias
By:Elise Georgi Morris, M.D.

Objectives
* Identify common arrhythmias encountered by the family physician
* Discuss arrhythmia etiologies
* Discuss initial primary care work-up and treatment
* Practice questions

Normal Sinus Rhythm
Implies normal sequence of conduction, originating in the sinus node and proceeding to the ventricles via the AV node and His-Purkinje system.
EKG Characteristics: Regular narrow-complex rhythm

Sinus Bradycardia
* HR< 60 bpm; every QRS narrow, preceded by p wave
* Can be normal in well-conditioned athletes
* HR can be<30 bpm in children, young adults during sleep, with up to 2 sec pauses

Sinus bradycardia--etiologies
* Normal aging
* 15-25% Acute MI, esp. affecting inferior wall
* Hypothyroidism, infiltrative diseases
(sarcoid, amyloid)
* Hypothermia, hypokalemia
* SLE, collagen vasc diseases
* Situational: micturation, coughing
* Drugs: beta-blockers, digitalis, calcium channel blockers, amiodarone, cimetidine, lithium

Sinus bradycardia--treatment
* No treatment if asymptomatic
* Sxs include chest pain (from coronary hypoperfusion), syncope, dizziness
* Office: Evaluate medicine regimen—stop all drugs that may cause
* Bradycardia associated with MI will often resolve as MI is resolving; will not be the sole sxs of MI
* ER: Atropine if hemodynamic compromise, syncope, chest pain
* Pacing

Sinus tachycardia
* HR > 100 bpm, regular
* Often difficult to distinguish p and t waves

Sinus tachycardia--etiologies
* Fever
* Hyperthyroidism
* Effective volume depletion
* Anxiety
* Pheochromocytoma
* Sepsis
* Anemia
* Exposure to stimulants (nicotine, caffeine) or illicit drugs
* Hypotension and shock
* Pulmonary embolism
* Acute coronary ischemia and myocardial infarction
* Heart failure
* Chronic pulmonary disease
* Hypoxia

Sinus Tachycardia--treatment
* Office: evaluate/treat potential etiology :check TSH, CBC, optimize CHF or COPD regimen, evaluate recent OTC drugs
* Verify it is sinus rhythm
* If no etiology is found and is bothersome to patients, can treat with beta-blocker

Sinus Arrhythmia
* Variations in the cycle lengths between p waves/ QRS complexes
* Will often sound irregular on exam
* Normal p waves, PR interval, normal, narrow QRS

Sinus arrhythmia
* Usually respiratory--Increase in heart rate during inspiration
* Exaggerated in children, young adults and athletes—decreases with age
* Usually asymptomatic, no treatment or referral
* Can be non-respiratory, often in normal or diseased heart, seen in digitalis toxicity
* Referral may be necessary if not clearly respiratory, history of heart disease

Sick Sinus Syndrome
* All result in bradycardia
* Sinus bradycardia (rate of ~43 bpm) with a sinus pause
* Often result of tachy-brady syndrome: where a burst of atrial tachycardia (such as afib) is then followed by a long, symptomatic sinus pause/arrest, with no breakthrough junctional rhythm.

Sick Sinus Syndrome--etiology
* Often due to sinus node fibrosis, SNode arterial atherosclerosis, inflammation (Rheumatic fever, amyloid, sarcoid)
* Occurs in congenital and acquired heart disease and after surgery
* Hypothyroidism, hypothermia
* Drugs: digitalis, lithium, cimetidine, methyldopa, reserpine, clonidine, amiodarone
* Most patients are elderly, may or may not have symptoms

Sick sinus syndrome--treatment
* Address and treat cardiac conditions
* Review med list, TSH
* Pacemaker for most is required

Paroxysmal Supraventricular Tachycardia
* Refers to supraventricular tachycardia other than afib, aflutter and MAT
* Occurs in 35 per 100,000 person-years
* Usually due to reentry—AVNRT or AVRT

PSVT
* Initial eval: Is the patient stable?
* Determine quickly if sinus rhythm
* If not sinus and unstable, cardioversion
* Unstable sinus tachycardia---IV beta-blocker, and treat cause
* Sxs of instability would include: chest pain, decreased consciousness, short of breath, shock, hypotension—unstable sxs require shock
* If stable, determine whether regular rhythm (sinus or PSVT) vs irregular (afib/flutter, MAT)? p waves (MAT vs. AF)?
* If regular, determine whether p waves are present, if can’t see---administer adenosine (6mg, can give 2 doses) or CSM or other vagal maneuvers)

* CSM or adenosine commonly terminate the arrhythmia, esp, AVRT or AVNRT
* Can also use CCB or beta blockers to terminate, if available
* Counsel to avoid triggers, caffeine, Etoh, pseudoephedrine, stress
* No p waves —junctional tachycardia, AVRT or AVNRT, Afib
* AVRT and AVNRT: can have retrograde p waves and short RP interval
* Abnormal p waves morphology: MAT

Atrial Fibrillation
* Irregular rhythm
* Absence of definite p waves
* Narrow QRS
* Can be accompanied by rapid ventricular response

Atrial Fibrillation—causes and associations
* Hypertension
* Hyperthyroidism and subclinical hyperthyroidism
* CHF (10-30%), CAD
* Uncommon presentation of ACS
* Mitral and tricuspid valve disease
* Hypertrophic cardiomyopathy
* COPD
* OSA
* ETOH
* Caffeine
* Digitalis
* Familial
* Congenital (ASD)

Atrial fibrillation--assessment
* H & P—assess heart rate, sxs of SOB, chest pain, edema (signs of failure)
* If unstable, need to cardiovert
* Echocardiogram to evaluate valvular and overall function
* Check TSH
* Assess for RVR
* Assess onset of sxs—in the last 24-48 hours? Sudden onset? Or no sxs?

Atrial fibrillation--management
* Rhythm vs Rate control—if onset is within last 24-48 hours, may be able to arrange cardioversion—use heparin around procedure
* Need TEE if valvular disease (high risk of thrombus)
* If unable to definitely conclude onset in last 24-48 hours: need 4-6 weeks of anticoagulation prior to cardioversion, and warfarin for 4-12 weeks after

Atrial Fibrillation
* Cardioversion: synchronized (w/QRS) delivery of current to heart; depolarizes tissue in a reentrant circuit; afib involves more cardiac tissue, but cardiovert
* Defibrillation: non-synchronized delivery of current

Atrial fibrillation--management
* Rate control with chronic anticoagulation is recommended for first line approach for majority of patients; overall Afib is a stable rhythm
* Beta-blockers (atenolol and metoprolol) or calcium channel blockers (verapamil or diltiazem) recommended. Digoxin not recommended for rate control
* Anticoagulation: LMWH and then warfarin; can use aspirin for anticoagulation if CI to warfarin, not as effective

Atrial fibrillation--management
* Goal INR of 2.5 (2.0-3.0)
* Rhythm control---second line approach, if unable to control rate or pt with persistent sxs
* Can also consider radiofrequency ablation at pulm veins
* P wave from another atrial focus
* Occurs earlier in cycle
* Different morphology of p wave
* Benign, common cause of perceived irregular rhythm
* Can cause sxs: “skipping” beats, palpitations
* No treatment, reassurance
* With sxs, may advise to stop smoking, decrease caffeine and ETOH
* Can use beta-blockers to reduce frequency

1st Degree AV Block
* PR interval >200ms
* If accompanied by wide QRS, refer to cardiology, high risk of progression to 2nd and 3rd deg block
* Otherwise, benign if asymptomatic

2nd Degree AV Block Mobitz type I (Wenckebach)

* Progressive PR longation, with eventual non-conduction of a p wave
* May be in 2:1 or 3:1
Wenckebach, Mobitz type I
* Usually asymptomatic, but with accompanying bradycardia can cause angina, syncope esp in elderly—will need pacing if sxs
* Also can be caused by drugs that slow conduction (BB, CCB, dig)
* 2-10% long distance runners
* Correct if reversible cause, avoid meds that block conduction

2nd degree block Type II (Mobitz 2)
* Normal PR intervals with sudden failure of a p wave to conduct
* Usually below AV node and accompanied by BBB or fascicular block
* Often causes pre/syncope; exercise worsens sxs
* Generally need pacing, possibly urgently if symptomatic

3rd Degree AV Block

* Complete AV disassociation, HR is a ventricular rate
* Will often cause dizziness, syncope, angina, heart failure
* Can degenerate to Vtach and Vfib
* Will need pacing, urgent referral
* Extremely common throughout the population, both with and without heart disease
* Usually asymptomatic, except rarely dizziness or fatigue in patients that have frequent PVCs and significant LV dysfunction
* No treatment is necessary, risk outweighs benefit
* Reassurance
* Optimize cardiac and pulmonary disease management

Non-sustained Ventricular tachycardia
* Defined as 3 or more consecutive ventricular beats
* Rate of >120 bpm, lasting less than 30 seconds
* May be discovered on Holter, or other exercise testing

Non-sustained ventricular tachycardia
* Need to exclude heart disease with Echo and stress testing
* If normal, there is no increased risk of death
* May need anti-arrhythmia treatment if sxs
* In presence of heart disease, increased risk of sudden death
* Need referral for EPS and/or prolonged Holter monitoring

Ventricular fibrillation
* Defibrillation

Practice Questions—Case studies

References

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Bradycardia-tachycardia syndrome

Bradycardia-tachycardia syndrome
By: Presented by Ri

Sick sinus syndrome

* Multiple manifestations on EKG
* Sinus bradycardia
* Sinus arrest
* Sinoatrial block
* Bradycardia –tachycardia syndrom

Bradycardia-tachycardia syndrome

* Alternating patterns of bradycardia and tachycardia
* Often there is a long pause (asystole) between heartbeats, especially after an episode of tachycardia
* Tachycardia: PSVT, atrial fibrillation, atrial flutter

causes

* Most cases are idiopathic
* Intrinsic causes
* Extrinsic causes
* Cardiac surgery, especially to the atria, is a common cause of sick sinus syndrome in children.

Clinical manifestations

* Many people with sick sinus syndrome have no symptoms
* Symptoms are related to the decresed cardiac output that occurs with the bradyarrythmias or tachyarrythmias
* Fainting , Fatigue , Shortness of breath, or dyspnea, Chest pains , Confusion , Palpitations
* Bradycardia-tachycardia syndrome: peripheral thromboembolism and stroke

Treatment

* If the disorder is asymptomatic (without symptoms), no treatment is necessary
* Bradycardia-tachycardia syndrome
* Associated tachycardia may be treated with medications after the person is protected from symptomatic bradycardia by a pacemaker.
* Warfarin has been shown to decrease the number of strokes and embolic events

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Arrhythmias and EKGs

Arrhythmias and EKGs

Outline
* Sinus Arrhythmia and Sick Sinus Syndrome
* Multifocal Atrial Tachycardia
* Bigeminal Rhythms
* Preexcitation and AVRT

Mechanisms of Arrhythmogenesis
Sinus Arrhythmia
EKG Characteristics: Presence of sinus P waves
Variation of the PP interval which cannot be q attributed to either SA nodal block or PACs

When the variations in PP interval occur in phase with respiration, this is considered to be a normal variant. When they are unrelated to respiration, they may be caused by the same etiologies leading to sinus bradycardia.

Sick Sinus Syndrome
* Characterized by a collection of symptoms and ECG findings due to chronic dysfunction of the sinoatrial (SA) node:
o Chronic and severe sinus bradycardia
o Sinus pauses
o Sinus arrhythmia
o Complete sinus arrest
o Progressive development of atrial arrhythmias (a-flutter, a-fib, atrial tachycardia)
* Patients are usually elderly and present with lightheadedness and/or syncope, but it can also manifest as angina, dyspnea, and palpitations.

* About 50% of people with SSS also display some degree of dysfunction of the AV node
Sinus bradycardia (rate of ~43 bpm) with a sinus pause

Etiologies of Sick Sinus Syndrome
Familial SSS (due to mutations in SCN5A)
Infiltrative diseases
Pericarditis
Lyme disease
Hypothyroidism
Rheumatic fever
Sinus node firbosis
Atherosclerosis of the SA artery
Congenital heart disease
Excessive vagal tone
Drugs
Tachycardia-Bradycardia Syndrome
* Common variant of sick sinus syndrome severe bradycardia alternates with paroxysmal tachycardias, most often atrial fibrillation.
* There is usually a prolonged pause in the cardiac rhythm following cessation of the tachyarrhythmia.
Tachycardia-Bradycardia Syndrome
Abrupt termination of atrial flutter with variable AV block, followed by sinus arrest with a junctional escape beat.

Multifocal Atrial Tachycardia
Bigeminal Rhythms
* Arrhythmias in which each normal sinus beat is followed by a premature contraction (PAC, PJC, or PVC).
* Results in a couplet rhythm which can be detected by pulse or auscultation.
* Generally benign
Atrial Bigeminy
Ventricular Bigeminy
Preexcitation
ECG Characteristics of WPW:
1. Short PR interval
2. QRS prolongation
3. Delta wave
Preexcitation is a condition characterized by an accessory pathway of conduction, which allows the heart to depolarize in an atypical sequence.
The most common form of preexcitation is called Wolfe-Parkinson-White (WPW) syndrome, in which a direct atrioventricular connection allows the ventricles to begin depolarization while the standard action potential is still traveling through the AV node.

AV Reentrant Tachycardia (AVRT)
In patients with WPW, a reentrant rhythm can be generated where the AV node serves as one arm of the reentrant circuit, and the accessory pathway as the other.

Types of AVRT
* Orthodromic AVRT (More common) – Narrow complex tachycardia in which the wave of depolarization travels down the AV node and retrograde up the accessory pathway.
* Antidromic AVRT (Less common) – Wide complex tachycardia in which the wave of depolarization travels down the accessory pathway and retrograde up the AV node.

Mechanism of orthodromic AVRT
Mechanism of antidromic AVRT
What is this arrhythmia?
Antidromic AVRT
Classification Scheme for Arrhythmias

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Joints of the Foot

Joints of the Foot

There are 26 bones in the foot; all but five are involved in at least two joints.
* Hind foot
* Midfoot
* Forefoot
* foot biomechanics1

Subtalar joint: where the talus rests on and articulates with the calcaneus. This is a synovial joint with a weak capsule supported by medial, lateral, posterior & interosseous talocalcaneal ligaments.

* The interosseous talocalcaneal ligament (very strong) lies in the tarsal sinus (separates the anterior & posterior talocalcaneal joints).
* Anatomical subtalar joint- functionally a single synovial joint between the slightly concave articular surface of the talus and the convex posterior articular surface of the calcaneus.

Important Intertarsal joints:
1. Subtalar (talocalcaneal) joint
2. Transverse tarsal joint (calcaneocuboid & talonavicular)

* The main movement at these joints are foot eversion & inversion, eversion is augmented by extension of the toes (especially the lateral toes), inversion is augmented by toe flexion especially the 1st &2nd toes.

Transverse tarsal joints – a compound joint
1. Talonavicular part of the talocalcanealnavicular joint

2. Calcaneocuboid joint

* These 2 separate joints are aligned transversely. At this joint the forefoot & midfoot rotate as a unit on the hind foot around an AP axis. This augments inversion/eversion of the foot.
* Anatomical amputations of the foot are made through this joint.


1. Intertarsal joints:
These bones are so tightly opposed by ligaments that little movement occurs between them

2. Tarsometatarsal joints:
Plane type synovial joints involved in gliding/sliding type movements

3. Metatarsophalangeal joints
Flexion/extension in the foot occurs at the metatarsalphalangeal joints & the interphalangeal joints

4. Interphalangeal joints
Each has plantar, medial & lateral collateral ligaments, dorsal extensor aponeuroses act as dorsal ligaments.

All the joints proximal to the metatarsalphalangeal joints are united by dorsal & plantar ligaments.

All the bones of the metatarsals and interphalangeal joints are united by lateral & medial collateral ligaments.

Major ligaments of the Plantar foot
Plantar calcaneonavicular (Spring) ligament

* Fills a wedge shaped gap between the talar shelf & inferior margin of the posterior articular surface of the navicular. This ligament supports the head of the talus and plays an important role in the transfer of weight from the talus & maintaining the longitudinal arch.

Long Plantar Ligament
* Traverses from the plantar surface of the calcaneus to the groove on the cuboid. Some fibers extend to the base of the metatarsals (forming a tunnel for the tendon of the fibularis longus. This ligament is important in maintaining the longitudinal arch.

Plantar calcaneocuboid (short plantar) ligament:
* Located deep to the long plantar ligament, it runs from the anterior part of the inferior surface calcaneus to the inferior surface of the cuboid. It is located on a plane between the plantar calcaneonavicular (spring) ligament and the long plantar ligament. It is also involved in maintenance of the longitudinal arch.

Arches of the Foot
* The ligamentous bony arrangement of the foot allows considerable flexibility/deformation with weight bearing contact. The arches distribute the weight of the foot (pedal platform) acting both as shock absorbers & spring boards during ambulation of all types.
* Weight distribution is between the calcaneus and sesamoid bones at the 1st metatarsal and head of the 2nd metatarsal; weight is shared laterally with the heads of metatarsals 3-5. Elastic arches between weight bearing points compress with loading and recoil with unloading.
* Lateral Longitudinal arch
* Medial Longitudinal arch
* Transverse Arch

All three work as a unit in weight bearing

* Medial Longitudinal Arch higher and more prominent than the lateral arch.
* Consists of the calcaneus, talus, navicular, 3 cuneiforms and 3 metatarsals
* Talar head is the keystone of the medial longitudinal arch
* The medial arch is supported by the Tibialis anterior ligament as it attaches to the 1st metatarsal and medial cuneiform. Also the tib posterior & FHL.
* The tendon of the fibularis longus passes from lateral to medial and also supports the medial longitudinal arch.
* Lateral Longitudinal Arch is much flatter and consists of the calcaneus, cuboid & lateral metatarsals.
* The medial arch is involved in weight bearing while the lateral arch is involved in balance
* Transverse arch: cuboid, cuneiforms and bases of the metatarsals. This forms the medial & lateral parts of the longitudinal arches which serve as pillars fro the transverse arch.
* The tendon of the fibularis longus & tibialis posterior crossing the sole of the foot obliquely help maintain the curve of the transverse arch.
* The Arches of the foot are maintained by both passive & dynamic supports.

Passive factors
1. Shape of the united bones (especially the transverse arch).

2. 4 layers of fibrous tissue
o 1. Plantar aponeurosis
o 2. Long plantar ligament
o 3. Short plantar ligament
o 4. Spring ligament

Dynamic Support
1. Active (reflexive) bracing action of the intrinsic muscles of the foot support the longitudinal arches.
2. Active & tonic contraction of muscles & tendons extending into the foot:
Longitudinal arch
flexor hallicus longus
flexor digitorum longus
Transverse arch
fibularis longus
tibialis posterior &anterior
3. Plantar ligaments & aponeurosis bear the greatest stress and are most important in maintaining the arches.

Pes Planis (flat feet)
Prior to 3 years of age it is normal to have flat feet due to a fat pad. After the age of 3 this fat pad disappears.

Pes planis can be classified as:

* Flexible: the arch is normal when unloaded however with loading the arch is lost. this is the most common type. It is due to inadequate passive arch support (weak, loose ligaments).
* Rigid type, the arch is absent regardless of loading, this may be due to a congenital deformity.

* Acquired “fallen arches” is due to a tibialis posterior dysfunction due to trauma, denervation and/or degeneration. The plantar calcaneal ligament fails allowing the head of the talus to rotate inferomedially creating a prominence on the medial aspect of the mid-hind foot junction. This is often referred to as over pronation.

Talipes equinovarus (Club foot)
* Club Foot
* A congenital deformity males/females 2/1 Foot inverted, ankle plantarflexed and forefoot abducted. The abnormality is related to short, tight muscles, tendons and ligaments.

* Orthopedic Subtalar joint: anatomical subtalar joint + talocalcaneal part of the talocalcaneonavicular joint (these straddle the interosseous talocalcaneal ligament
* The main movement at this subtalar joint is inversion/eversion.

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