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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