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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Skin – Immune Disorders

Skin – Immune Disorders
By:Jan Bazner-Chandler
CPNP, CNS, MSN, RN

Key Function of Skin
* Protection – shield from internal injury.
* Immunity – contains cells that ingest bacteria and other substances.
* Thermoregulation – heat regulation through sweating, shivering, and subcutaneous insulation
* Communication / sensation / regeneration

Developmental Variances
* Sweat glands function by the time the child is 3-years-old.
* The visco-elastic property of the dermis becomes completely functional at about 2 years.
* The neonate’s dermis is thin and very hydrated, thus is at greater risk for fluid loss and serves as an ineffective barrier.

Diagnostic Tests
* Cultures
* Scraping
* Skin biopsy
* Skin testing
* Woods lamp
Neonatal skin lesions
* Vascular birth marks: hemangioma
* Port wine stain
* Abnormal pigmentation: Mongolian spots
* Neonatal acne: small red papules and pustules appear on face trunk.
* Milia: white or yellow, 1-2mm papules appearing on cheeks, nose, chin, and forehead
Inflammatory Skin Disorders
* Diaper dermatitis
* Contact dermatitis
* Atopic dermatitis or eczema
Cradle Cap
Treatment
Baby Care
Acne Vulgaris
Management of Acne
Pediculosis
Signs and Symptoms
Nits
Empty nit case
Viable nit
Interventions
Scabies
Assessment
Management
Impetigo
Causative agent
Spread
Interventions
Outcomes
Impetigo / cellulitis
Clinical Manifestations
Poison Oak, Ivy and Sumac
Poison Ivy
Poison Oak
Systemic Response
Burns in Children
Management of Burns
Alert
Flame Burn
Percentage of Areas Affected
Depth of Burns
First Degree Burn
Second Degree Burn
Third Degree or Full-thickness
Wound Management
Children and Their Families
Skin Grafts
Removal of split-thickness
Skin graft with dermatone.
Healed donor site
Compartment Syndrome
Escharotomy / fasciotomy in a severely burned arm.
Burn Wound Covering
Therapy to Prevent Complications Elasticized garment and “air-plane” splints.
Physical therapy to prevent contracture deformity.
Flash burn from gasoline.
Electrical burn caused by biting of electrical cord.
Ball & Bender
Keep Kids Safe
Infants Immune System
Immune Response
Neonatal Sepsis
Sepsis
Major Risk Factors
Minor Risk Factors
Etiology
Diagnostic Tests
Clinical Manifestations
Blood Test
Medical Management
Nursing Interventions
Outcomes
SCIDS
Treatment
Acquired Immunodeficiency Syndrome / AIDS
Killer T-cells
Blood Testing in Infants
Treating Infants in Utero
Modes of Transmission
Interdisciplinary Interventions
Interventions
Community Interventions
Changes in HIV

Skin – Immune Disorders.ppt

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Acne Vulgaris - known as blackheads

Acne Vulgaris
(Otherwise known as zits, pimples and blackheads)
By: Cynthia Salinas, M.D.

Conference Goals
* Review pathogenesis as a way to help us understand why we use certain meds
* Differentiate common types of acne
* Generate a quick differential diagnosis
* Apply a stepwise approach to treatment prior to referral to dermatology

Epidemiology
Onset?
Prevalence?
Causes?
Pathogenesis
HPI
Differential Diagnosis
Types of Acne
Comedonal Acne
Papulopustular Acne
Nodulocystic Acne
Management
Four Major Goals of Treatment
Take home points:
Retinoids
Topical Antibiotics
Other
Comedonal Acne
Papulopustular Acne
Oral antibiotics
Nodulocystic Acne
Education
Completing Therapy
Follow-up on Patient
Conclusions
Sources

Acne Vulgaris.ppt

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Cosmetic Dermatology in Primary Care

Cosmetic Dermatology in Primary Care
by: Jenifer Hammond MD

What is Skin?
* A protective, yet permeable barrier for the human body
* Body’s largest organ
* Most essential source of sensory stimulation

Three layers of skin
* Epidermis
* Dermis
* Subcutaneous fat (hypodermis)

Facts of Aging Skin
Clinical Signs of Aging Skin
Clinical Signs of Photoaging Skin
FACTS about PhotoAging
Photodamaged Skin
What is the treatment for Photoaging Skin?
You Are What You Eat!
What is Good Skin Care?
Exfoliate
Microdermabrasion
Hydrate
Protect
Skin Cancer Facts 2007
Topical C
Prevage MD
UVB Protection
Sun Protection Tips
UVA protection “What’s New”
Sunscreen
Retinoids
Rejuvenation Procedures
Peels
What are Chemical Peels?
Types of Peels
Glycolic Acid (AHA)
Jessnar’s Peel
TCA Peel
Facial Fillers
Collagen
Collagen Lip Augmentation
Hyaluronic Acids
Juvederm
Radiesse
Marionette before After 3 months
Nasolabial before After 3 months
Cheeks before After 3 months
Sculptra
Before Sculptra
Sculptra after 3 treatments
Lasers
IPL Photo-Facial
IPL with Levulan Therapy
Laser Hair Removal
“What’s New”
Ablative & Non-Ablative Skin Resurfacing
Mechanism of Light-induced Skin Rejuvenation
Light absorption by tissue water
Inflammatory response
Release of inflammatory mediators into dermal interstitium
Aesthetica Cosmetic & Laser Center
Non-ablative tissue tightening

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Acne

Acne
By: David M. Bracciano, D.O.

Acne Vulgaris
* Chronic inflammatory disease of the pilosebaceous follicles
* Comedones, papules, pustules, cysts, nodules, and often scars
* Face, neck, upper trunk, and upper arms
* Disease of the adolescent
* 90% of all teenagers
* May also begin in twenties
* Usually involution by 25
* Occurs primarily in oily{seborrheic} areas of the skin
* Face occurs; cheeks> nose>forehead>chin
* Ears; comedones in concha, cysts in lobules
* Retroauricular and nuchal cysts

Comedo
* Commonly known as the blackhead
* Basic lesion of acne
* Produced by hyperkeratosis of the lining of the follicles
* Retention of keratin and sebum
* Plugging produced by the comedo dilates the mouth of the follicle
* Papules are formed by inflammation around the comedones

Severity of Acne
* Typical mild acne; comedones predominate
* More severe cases; pustules and papules predominate, heal with scar if deep
* Acne Conglobata; suppurating cystic lesions predominate, and severe scarring results
Types
* Acne comedo; mild case were eruption is composed almost entirely of comedones on an oily skin
* Papular acne; inflammatory papules, most common in young men with coarse, oily skin
* Atrophic acne; residual atrophic pits and scars
Etiology
* Keratin plug in lower infindibulum of hair follicle
* Androgenic stimulation of sebaceous, proliferation of propionbacterium acnes which metabolizes sebum to produce free fatty acids
Pathogenesis
* Disruption of the follicular epithelium permits discharge of the follicular contents into the dermis
* Causes the formation of inflammatory papules, pustules, and nodulocystic lesions
* FFA are chemotactic to components of inflammation
* Effects of tetracycline are obtained by the reduction of FFA
* Antibiotics do not produce involution of the inflammatory lesions present, but inhibit the formation of new lesions
* Topical retinoic acid acts on keratinization, causing horny cells to lose their stickiness
* Androgens enlarge the sebaceous glands
* In women consider hyperandrogenic state

Histology
Treatment
Antibacterials
Tetracyclines
Minocycline
Bacterial Resistance
Oral Contraceptives
Hormonal Therapy
Isotretinoin
Topical Treatment
Benzoyl Peroxide
Topical Retinoids
Topical Antibacterials
Other Topicals
Surgcial Treatment
Intralesional Corticosteroids
Complications of Acne
Acne Conglobata
Acne Fulminans
SAPHO Syndrome
Tropical Acne
Premenstrual Acne
Preadolescent Acne
Neonatal Acne
Infantile Acne
Acne Venenata
Acne Cosmetica
Acne Detergicans
Acne Aestivalis
Excoriated Acne
Acneiform Eruptions
Gram Negative Folliculitis
Acne Keloidalis
Hiradenitis Suppurativa
Perifolliculitis Capitis Abscedens
Acne vs. Rosacea
Ocular Rosacea
Granulomatous Rosacea
Rosacea Etiology
Differential Diagnosis Rosacea
Inflammatory rosacea
Rosacea Treatment
Rosacea Rhinophyma
Pyoderma Faciale
Perioral Dermatitis

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Dermatology

Dermatology
By: Katrice L. Herndon, MD
Internal Medicine/Pediatrics

Acne Vulgaris

* Acne is a self-limited disorder primarily of teenagers & young adults.
* Acne is a disease of pilosebaceous follicles.
* 4 factors are involved:
* External Factors that contribute to Acne
* Acne vulgaris typically affects those areas of the body that have the greatest number of sebaceous glands:
* In addition to the typical lesions of acne vulgaris, scarring and hyperpigmentation can also occur.
* Hyperpigmentation is most common in patients with dark complexions
* Classification of Acne

Acne Vulgaris What is this?

Acne Rosacea
* Rosacea is an acneiform disorder of middle-aged and older adults.
* Characterized by vascular dilation of the central face, including the nose, cheek, eyelids, and forehead.
* The cause of vascular dilatation in rosacea is unknown.
* The disease is chronic.
* rosacea is a chronic disorder characterized by periods of exacerbation and remission.
* Increased susceptibility to recurrent flushing reactions that may be provoked by a variety of stimuli including hot or spicy foods, drinking alcohol, temperature extremes, and emotional reactions.
* The earliest stage of rosacea is characterized by facial erythema and telangiectasias.
* Patients with rosacea may develop severe sebaceous gland growth that is accompanied by papules, pustules, cysts, and nodules.
Allergic Contact Dermatitis
Psoriasis
Psoriasis Treatment
Vitiligo
Pityriasis Rosea
Cellulitis
Erysipelas
Ecthyma
Treatment
Tinea Vesicolor
Cutaneous Warts
Differential Diagnosis
Secondary Syphilis
Treatment
Herpes Zoster
Treatment
Actinic Keratosis
References

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Healthy Skin Women and Dermatology

Healthy Skin Women and Dermatology
By:Suguru Imaeda, M.D.
Chief of Dermatology, Yale University Health Service

Overview
* Normal structures of the skin
* Changes in the skin over time
* Sun and skin
* Skin cancer
* Maintaining healthy skin
Epidermis
the largest organ
* key role in normal healthy functioning of the body
* Disorders range from those limited to the skin to manifestations in the skin of internal disorders
* plays important role in social and psychosocial functioning of the individual
* undergoes changes with aging and in response to external environmental factors and internal hormonal influences
Gender differences
* Fundamental differences in structure and function of the skin
* Differences impact on presentation of skin disease and its management
* Hormonal influences affect common disorders such as acne, rosacea, lupus erythematosus, psoriasis, lichen planus, anogenital pruritus, hidradenitis suppurativa, and atopic dermatitis
Infancy
Toddler to adolescence
Adolescence
Body piercing
* presents risks for multiple possible complications
* nickel allergy
* secondary infection with staphylococcus or streptococcus
* ear cartilage destruction from pseudomonal infection
* candidal infection of the navel or genitalia
* Keloids
* traumatic tears
Tattoos
* Infection
* Granulomatous reaction
* Photodermatitis
* Difficult to remove
Melasma
* Brown patches on forehead and cheeks
* Very sensitive to sun exposure
* More common in Hispanics, Middle Easterners, and Asians
* Most common cause is oral contraceptive use or pregnancy
Melasma management
* Discontinuation of oral contraceptive
* Avoidance of sun/tanning bed
* Daily application of broad spectrum sunscreen
* 4% hydroquinone or 20% azelaic acid
* ? laser
Intrinsic aging
* Changes of chronologic aging gradually become apparent
* Influenced by genetics, gravity, and hormones
* Clinically, the normal aging process leads to fine wrinkles, dryness, sallow color, thinner skin, laxity and purpura
Aging skin
* Decreased function as environmental barrier, sensory organ and immune organ
* Epidermal and dermal atrophy with loss of appendages
* Decreased sweat production leads to dryness
* Decreased body and scalp hair
* Decreased ovarian estrogen production leads to decreased collagen and increased wrinkling
* Overall thinner, paler, drier, with fine wrinkling and decreased elasticity
Histologically
* dermal thinning
* decreased vascularity
* decreased subcutaneous fat
* reduced cellularity of the dermis
* elastic fiber loss
* dermal thinning
* decreased vascularity
* decreased subcutaneous fat
* reduced cellularity of the dermis
* elastic fiber loss

Environmental factors on skin
* create extrinsic damage
* major effect is from photoaging with wrinkling, laxity (sagging), lentigenes, dyschromia, coarseness, sebaceous hyperplasia, and telangiectasia
* 90% of visible skin changes of aging
* Visible as early as age 20
Smoking
* shown to decrease both hyaluronic acid and glycosaminoglycan synthesis
* causes decreased capillary blood flow in the skin
* changes accelerate wrinkling
The twenties
* Skin is smooth and coloring is even
* Little need for emollients
* Skin care is simple - variety of products are tolerated
* May be persistent acne associated with hormonal activity manifest by flaring during the week prior to the menstrual period.

The thirties
* thinning of the skin beneath the eyes
* skin is less elastic
* Fine wrinkles begin to appear around the mouth and lateral periorbital region
* Increased fat and sluggish blood flow contribute to puffiness and darkening of the skin beneath the eyes
The forties
* More sallow and less supple
* Skin surface not as smooth
* Liver spots, solar lentigenes, appear on areas of chronic low grade sun exposure - face, dorsal hands, back or dorsal feet
* Thin red spider angiomas appear on the legs
* Weight gain leads to sagging skin
* Cellulite appears on thighs and buttocks
* Deep furrows develop on forehead and lateral periorbital areas (crow’s feet)
* Skin becomes drier - sweat glands grow smaller and become less effective
The fifties and sixties
* Wrinkles are deeper - skin begins to sag and droop
* Skin tone is lighter from decreased circulation
* More solar lentigines form
* Collagen and elastin are thinner
* Collagen is estrogen dependent therefore skin is both thinner and drier
* Dryness occurs from thickening of the stratum corneum
* Moisturizers help keep the skin moist and supple
* Alpha hydroxy acid-containing products help by reducing the thickness of the stratum corneum, promoting thickening of the epidermis and dermis, and promoting synthesis of collagen, elastin, protein and glycosaminoglycan
Pregnancy
* rosy complexion - increase in vascular circulation
* hyperpigmentation on the nipples, vulva, anus and inner thighs from hormonal stimulation
* Freckles (ephelids) and birthmarks may also darken
* Melasma, the mask of pregnancy, from hormonal changes, sun exposure and genetic factors
* Skin tags develop on the neck, chest, inframammary area, inner thighs, and face
* Spider angiomas, purpura and capillary hemangiomas
* Stretch marks, striae distensae
* Varicosities and hemorrhoids
Sun protection
* Signs of extrinsic photoaging not intrinsic genetic aging usually prompts the visit to the dermatologist
* Therefore, it is most important to incorporate into the daily routine a sun protection regimen
Tan
* Coco Chanel declares tanning “in” in 1920’s
* Suntan seen as symbol of health, youth, status
* Skin’s reaction to damage from UV radiation
* Melanocytes produce melanin
Indoor tanning
* Increasingly popular, esp among young women in 20’s
* Advertised as safe, “healthy glow”, little risk of skin cancer
* Controlled tanning protects against sunburn by building up melanin
* Vitamin D helps prevent breast, prostate, colon cancer
Dripping faucet
* Your skin = empty glass
* Dripping water = ultraviolet radiation
* Rate of drip = amount of sun exposure
* Rate of evaporation of water = skin’s ability to repair DNA damage caused by UV radiation
* Your glass is full = you’ve reached your limit of sun exposure
* Water starts spilling over the top = getting skin cancers
Ultraviolet radiation
Sun myths
Basal cell carcinoma and Squamous cell carcinoma
Melanoma
IARC (International Agency for Research on Cancer)
Sunless tanning
Tanning pills
Sun Protection Factor
In vitro CT FL
New proposed labeling guidelines for sunscreens
Sunscreen
UV Index
Anti-aging treatments
* Prevention
* Retinoids
* Lasers
* Chemical peels
* Dermabrasion
* Botox
* Fillers
* $35 billion/year industry
Prevention
* Daily use of sunscreen or moisturizer with sunscreen SPF 15+
* Clothing
* Avoiding unnecessary UV exposure
* Avoiding sun exposure between 10a-4p
Retinoids
* Tretinoin and tazarotene
* Vitamin A derivatives
* Even out skin tone
* Rebuild collagen
* Repair minor sun damage
* Inhibit tumor growth
* Decrease inflammation
Lasers
Chemical peels
Dermabrasion
Botox
Fillers
General skin care
Skin Care Tips
* Sun protection
* Hydration
* Healthy diet – fruits, vegetables, fish
* Gentle skin care products – soaps
* Moisturize
* Don’t smoke

Healthy Skin Women and Dermatology.ppt

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Dermatology Review

Dermatology Review
By:Jennifer Best, MD

Acanthosis Nigricans
* Velvety discoloration of skin in flexural creases
* Most commonly seen in insulin resistant states (e.g. DM, PCOS, niacin use), endocrinopathy, malignancy

Xanthelasma
* Soft, polygonal papules and plaques consisting of cholesterol, usually located on upper lids
* When seen in children and young adults, associated with familial hypercholesterolemia
Necrobiosis lipoidica
* Well-demarcated plaque, yellow-orange to tan-pink with thinning and telangiectasia
* Non-painful
* Usually located on shins/feet
* Associated with long-standing, juvenile-onset DM
Molluscum contagiosum
* Centrally umbilicated papules seen in children and sexually active adults
* Viral cause
* More aggressive and common on face in HIV
Angular cheilitis
* Fissuring of corners of mouth
* Associated with thrush, atopic dermatitis, nutritional deficiencies and denture use
Prurigo nodularis
* Pickers’ nodules
* Nodular lesions due to chronic excoriation of the skin
Keratosis pilaris
* Benign sandpaper like bumps (“goosebumps”) on extensor surfaces
* Associated with atopy or a normal variant
Morphea
* Plaques are initially purplish and become ivory in color
* Localized scleroderma
Nikolsky’s sign
* POSITIVE when epidermis is dislodged from the dermis by lateral shearing pressure and blister extends
* Seen in toxic epidermal necrolysis, scalded skin syndrome and pemphigus vulgaris
Seborrheic keratosis
* What is it?
* Does it have malignant potential?
* Warty brown growths seen on aging skin – looks “stuck on”
* No malignant potential, purely cosmetic
Seborrheic dermatitis
* Always think of HIV in seborrheic dermatitis that is extensive or refractory to treatment
Rhinophyma
* Bulbous erythematous enlargement of the nose
* Seen in advanced rosacea
Hidradenitis suppurativa
* Sebaceous cysts seen in follicular areas (e.g. groin, axillae, scalp)
* More common in African Americans
Hereditary Hemorrhagic Telangiectasia
* Other names?
* Dermatologic manifestations?
* Clinical associations?
* Otherwise known as Osler-Weber-Rendu Syndrome
* Autosomal dominant
* Red macular/papular telangiectasias and AVMs on or around mucous membranes/GI tract
* Associated with bleeding tendency
Acrochordon
* Common name?
* Skin tag
Rosacea
* 1. Papules
* 2. Pustules
* 3. Telangiectasias
* Located over cheeks
* May involve nasolabial folds
* Exacerbated by alcohol, hot beverages, spicy foods, sun exposure
Nail pitting
* Associated with?
* Psoriasis
Condyloma lata
* What is it?
* What organism is responsible?
* Flat flesh-colored warts seen in anogenital region
* Representative of secondary syphilis
Condyloma acuminata
* What is it?
* What organism is responsible?
* Human papilloma virus (HPV)
* Genital warts
Whitlow
* Herpes simplex virus infection on finger
* Often seen in health care workers
Tinea versicolor
* Macules with fine scaling on trunk, upper arms, neck, abdomen, axillae with varying pigmentation
* Asymptomatic
* Caused by Malassezia furfur (looks like “spaghetti and meatballs” on KOH prep)
Ascending skin lesions
* Differential diagnosis?
* Mycobacterium marinum
* Sporothrix schenkii
* Nocardia
* Francisella tularensis
Hypopigmented anesthetic macules
* Leading diagnosis?
* Leprosy (Hansen’s Disease)
Yellow-orange skin discoloration
* What should you think of?
* Jaundice
* Increased beta carotene ingestion
* Hypothyroidism (without thyroid hormone, problems metabolizing beta carotene)
* Usually seen best on palms
Argyria
* What is it?
* Silver-gray discoloration of the skin due to intradermal deposition of silver
* Permanent!
Hot tub folliculitis
* What causes it?
* Pseudomonas aeruginosa
Heliotrope lids
* Purplish discoloration around eyes seen in dermatomyositis
* Also associated with…
* Purplish discoloration around eyes seen in dermatomyositis
* Also associated with…Gottron papules
* Recall association of dermatomyositis with malignancy in older patients
Common drug causes of slate-gray discoloration
Common cause of bullous cellulitis in liver disease
Erysipelas
* Responsible organism?
* Group A beta-hemolytic strep
* Raised erythematous plaques, may involve face or extremities
Bullous pemphigoid
* Most common blistering disorder
* Most common in elderly
* Nikolsky negative
* Commonly related to medications (sulfa, furosemide, penicillin
Erythema marginatum
* Pink rings on the trunk and inner surfaces of the arms and legs which come and go for as long as several months - barely raised and non-pruritic. Face is generally spared.
* Associated with rheumatic fever (but only less than 5% of patients)
* Considered a major Jones criterion when it does occur (4 others?)
Erythema nodosum
* Painful, erythematous round plaques, commonly pretibial
* Associated with infection (including strep, fungal, TB), IBD, sarcoidosis, drugs (OCPs, sulfa)
Drug cause of skin necrosis
* Warfarin (1:10,000)
* High doses
* Overweight women
* Breasts, buttocks, thighs, abdomen
Bacillary angiomatosis
* Associated with Bartonella infections (quintana, henselae)
* Cutaneous vascular tumors seen almost exclusively in HIV
Lupus pernio
* Dermatologic manifestation of sarcoidosis
* Indurated violaceous lesions, usually on face
Erythema multiforme
* Strong association with HSV, mycoplasma
* Drug causes (sulfa, phenytoin, PCN, allopurinol)
* Target lesions – can be localized to hands/face or generalized
* On a continuum with Stevens-Johnson
Dermatitis herpetiformis
* Grouped erythematous papules and plaques – seen on extensor areas (elbows, knees, trunk, buttocks, sacrum) as well as scalp, face and hairline
* Associated with celiac sprue
Herpes zoster
* Reactivation of VZV along dermatomal distribution
* Increasing incidence with age and immunosuppression
* Pain precedes rash
Erythema migrans
* “Bulls-eye” lesions seen in 50-70% of patients with Lyme disease (Borrelia burgdorferi).
Serology?
Infectious disease most associated with cryoglobulinemia?
Infectious disease most strongly associated with cryoglobulinemia?
* Hepatitis C virus
* Can cause vasculitic lesions (palpable purpura) commonly on lower extremities
Kaposi’s Sarcoma
* Associated with?
* HIV
* HHV-8
Keratoderma Blenorrhagicum
* Red-brown papules/pustules/vesicles with erosion and crusting on dorsilateral and plantar foot and palms
* Associated with reactive arthritis
Livedo reticularis
* Associated with?
* Vasculitis
* Vascular spasm
* Atheroemboli
* Normal variant (finer pattern)
* Most common on extremities/abdomen
Pyoderma gangrenosum
* Painful, hemorrhagic pustule or macule that breaks down into an ulcer with irregular raised borders and purulent base
* Usually on lower extremities
* Most strongly associated with IBD, but also associated with inflammatory arthritis, hematologic disorders and other GI conditions
Increased skin elasticity
* Ehlers-Danlos
Lichen planus
* Associated with?
* Hepatitis C virus
* Purple, planar, pruritic, polygonal papules on volar wrists, ankles, genitals, mucous membranes and nails
* Wickham’s striae are white lines inside lesions
Peutz-Jeghers
* Cutaneous hyperpigmented macules of lips, perioral and perinasal areas
* Associated with polyps and hamartomas of the GI tract (small bowel, colon and stomach)
* Associated with increased cancer risk
Koebner phenomenon
* What is it?
* With what dermatologic condition is it associated?
* Development of lesions at the site of skin trauma
* Seen in psoriasis (1/3 of patients), but also eczema, vitiligo, lichen planus or sclerosus
Pathergy
* What is it?
* With which disease is it associated?
* Development of pustule at the site of skin breach (i.e. blood draws, injections)
* Seen in Behcet’s Disease
Dermatographism
* With which condition is it associated?
* Development of wheal and flare at site of minor friction
* Seen in urticaria
Eruptive xanthomas
* Yellowish papules located on extensor surfaces (knees, buttocks, elbows)
* Seen in familial hypertriglyceridemia
Urticaria pigmentosa
* Name the disease…
* Flat top papules and brown plaques that for wheal and flare when scratched
* Seen in mastocytosis
Osler’s nodes
* Painful, purplish lesion usually on hands - immune complex deposition
* Seen in endocarditis
Janeway lesions
* Flat , bluish-red, non-painful lesions on palms and soles
* Seen in endocarditis
Cat-scratch disease
* Causative organism?
* Bartonella
* Following cat scratch, development of acute, tender regional lymphadenopathy
“Slapped-cheek” rash
Eosinophilic folliculitis
Koilonychia
Corkscrew hairs and perifollicular hemorrhage
Tophus
Peau d’orange
Actinic keratosis
Porphyria cutanea tarda
Nailfold telangiectasia
Beau’s lines
Clubbing
Oral Hairy Leukoplakia
Pityriasis rosea
Spider angioma
Café au lait spots
Caput medusae
Migratory necrolytic erythema
Acrodermatitis enteropathica
Ecthyma gangrenosum
Sweet’s syndrome

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Selected Skin Diseases and Treatment

Selected Skin Diseases and Treatment
Tailored for the Athletic Trainer
By:Dr. Garth Russo

Dermatology: Common Pathology
* Infectious
o Bacterial
o Viral
o Fungal
o Parasitic
* Immunologic
o Inflammatory
o Allergic
o Acne
Bacterial Infections
* Folliculitis
* Cellulitis
* Impetigo
* Boil/Furuncle/Abscess/Carbuncle

Folliculitis: Common
Folliculitis: Special Circumstances
Hot Tub Folliculitis
Pseudofolliculitis barbae
Acne Keliodalis
Cellulitis
Impetigo
Boil, Furuncle, Abscess, Carbuncle
Abscess
Carbuncle
Furuncle
Hidradenitis Suppurativa
Viral Infections
Herpes Simplex
Herpes Virus Behavior
Oral Herpes
Genital Herpes
Cutaneous Herpes: Herpes Gladiatorum
Varicella Virus
* Chicken Pox
* Shingles
Shingles
Variola: Smallpox
Warts
* Common wart
* Plantar Wart
* Genital Wart
Common Wart: Verucca Vulgaris
Plantar Wart
Genital Warts
Molluscum Contagiosum
Viral Exanthems
Fungal Infections
Fungal Infections:Treatment
Tinea Corporis: Ringworm
Tinea Versicolor
Tinea Pedis: Athlete’s Foot
Tinea Cruris
Tinea Capitis
Tinea Unguium
Parasites: Scabies
Scabies: Treatment
Immunologic Processes
* Inflammatory Conditions
* Allergic Responses
* Acne
Inflammatory Conditions
* Inflammatory Conditions
o Eczema
o Psoriasis
o Contact Dermatitis
o Pityriasis Rosea
+ This class
Allergic Reactions
Urticaria: Hives
Acne Vulgaris
Cystic Acne
Papulopustular Acne
Other Acne
* Steriod Acne
* Neonatal Acne
* Occupational Acne
* Acne Mechanica
* Acne Cosmetica
* Excoriated Acne
Mechanical/Occupational Acne

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Dermatology

Folliculitis Decalvans
* An inflammatory reaction of the hair follicles
* Leads to cicatricial alopecia
* Small pustules surround the follicles
* Erythema, scaling, and smooth shiny depressed scars are apparent
* Pseudopelade
* When the pustules have healed and scarring remains –pseudopelade occurs
* Note intact follicles and single hairs growing
* May occur on axillae and groin as well
* Etiology is unknown
* Scarring alopecia in a middle-aged man, associated with a hyperkeratotic scale-crust with follicular hyperkeratosis and erythema

* TREATMENT:
* Cephalosporins, dicloxacillin, and azithromycin and rifampin may be added to therapy for better long-term control
* Oral zinc or vitamin C supplementation may enhance response
* Chronic inflammation reactions may be helped with topical steroids and by intralesional triamcinolone
* Thick, asbestos-like (amiantaceous), shiny scales attached to the lower part of the hair shaft, rather like tiles overlapping on a roof
* Crusting may be localized or, less commonly generalized over the entire scalp
* There are no structural changes in the hair, but in some patches where the crusting is thick, there may be purulent exudate under the crust and temporary alopecia may occur

Tinea Amiantacea
* Etiology is likely secondary to an infection occurring in seborrheic dermatitis or inverse psoriasis
* Treatment should be shampoo daily or every other day with selenium sulfide susupension, or a tar shampoo , for a few weeks
* Prior application of Baker’s P&S liquid is helpful to remove scale and crust
* Derma-Smoothe and FS shampoo are also effective
Keratosis Follicularis Contagiosa
* Also known as epidemic acne, epidemic follicular eruption, epidemic follicular keratosis, and Brooke’s disease
* Unknown etiology
* Occurs in children
Keratosis Follicularis Contagiosa
* Eruption is widespread and symmetrical, affecting chiefly the back of the neck, the shoulders, and the extensor surfaces of the extremities
* Onset is acute, may affect large numbers of patients in a localized geographic area , and spontaneously involutes over a 3-to-6-week period
* There is a horny thickening of these areas, especially pronounced about the follicles, where small black corneous may be discerned
* Etiology has been hypothesized to be infectious- but not proven

Folliculitis Nares Perforans
Perforating Folliculitis
Kyrle’s Disease
Reactive Perforating Collagenosis
Trichrome stain
Perforating Disease of Hemodialysis
Traumatic Anserine Folliculosis
Disseminate and Recurrent Infundibulofolliculitis
Lichen Spinulosus
Histology:
Treatment:
Hyperhidrosis
Gustatory Hyperhidrosis
Other Localized Forms of Hyperhidrosis
Generalized Hyperhidrosis
Treatment:
Anhidrosis= absence of sweating
Bromidrosis= fetid sweat
Chromhidrosis
Fox-Fordyce Disease
Apocrine gland sweating does not occur in areas of involvement
Treatment is difficult-No form of therapy is uniformly effective
Granulosis Rubra Nasi
Hidradenitis
Neutrophilic Eccrine Hidradenitis
Recurrent Palmoplantar Hidradenitis
Sagittal view of nail unit
Lichen Planus of Nails
Treatment is unsatisfactory-
Psoriatic Nails
Darier’s Disease
Onychomadesis
Beau’s Lines
Half and Half Nails
Muehrcke’s Lines
Mees’ Lines
Terry’s Nails
Onychorrhexis (Brittle Nails)
Onychoschizia
Pitted Nails (Stippled Nails)
Racquet Nails (Nail en Raquette)
Chevron Nail (Herringbone Nail)
Hapalonychia
Platonychia
Nail-Patella Syndrome
Other bone features
Median Nail Dystrophy
Pterygium Unguis
Onychogryphosis
Anonychia
Onychoatrophy
Onychomadesis
Beau’s Lines
Half and Half Nails
Mees’ Lines
Terry’s Nails
Onychorrhexis (Brittle Nails)
Onychoschizia
Pitted Nails (Stippled Nails) .......
Leukonychia or White Nails
Nail-Patella Syndrome
Median Nail Dystrophy
Pterygium Unguis
Pterygium Inversum Unguis
Hangnail
Pincer Nails
Onychophagia
Onychotillomania

Dermatology.ppt

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