epidermal nevi, neoplasms and cysts – part 1 dan ladd, do january 28, 2003
TRANSCRIPT
Epidermal Nevi, Neoplasms and Cysts – Part 1
Dan Ladd, DO
January 28, 2003
A brief review….
Keratinizing Epidermal Nevi
• Hyperkeratosis without cellular atypia
• Nevus cells do not occur
• Follow Blaschko’s Lines
• Linear Verrucous Epidermal Nevus
• ILVEN
• Epidermal Nevus Syndrome
Linear Verrucous Epidermal Nevus
Linear Verrucous Epidermal Nevus
• Not pruritic, onset birth or before age 10.
• Verrucous papules, pink, gray or brown.
• Horny excrescences, comedos may be interspersed.
• Bilateral = Icthyosis hystrix
• Extensive = “systematized”
• Extensive + CNS abnormalities = Syndrome
Linear Verrucous Epidermal Nevus
• 62% variable hyperkeratosis, acanthosis and papillomatosis
• Rarely trichoepithelioma, KA, verruciform xanthoma
• Etiology possibly chromosomal mosaicism
• Tx: Phenol, 5-FU, Tretinoin, Shave excision, Cryotherapy, CO2 laser.
LVEN
Blaschko’s lines
• Albert Blaschko
• 1901
• Do not follow nerves, lymphatics or vessels.
• Proposed embryologic origin
LVEN following Blaschko’s lines
ILVEN
ILVEN
• Inflammatory Linear Verrucous Epidermal Nevus.
• Pruritic, usually on female extremity.• Onset usually childhood, can be 40’s, 50’s• Chronic, resistant to topical or IL treatments• Psoriasiform histo – linear psoriasis?• Tx: Deep shave excision, dermabrasion,
Protopic?
Epidermal Nevus Syndrome
ENS – 5 Syndrome types
• Schimmelpenning – sebaceous nevus, cerebral anomalies, coloboma, lipdermoid conjunctiva
• Nevus Comedonicus - cataracts• Pigmented hairy EN – Becker nevus, ipsilateral
breast hypoplasia, scoliosis• Proteus –Hyperplasia of hands and feet,
hemangiomas, lipomas, macrocephaly, hyperostosis, hypertrophy of long bones
• CHILD – Congenital Hemidysplasia, Icthyosiform erythroderma, Limb Defects
Nevoid Hyperkeratosis of the Nipple
Nevoid Hyperkeratosis of the Nipple
• Extremely rare, usu. females, any race• Unilateral NHN Should be distinguished
from breast carcinoma via biopsy, in addition, mammography may be warranted.
• Course varies, unpredictable.• Tx: Keratolytics such as Lactic Acid 12%,
Salicylic acid Gel 6%, Topical retinoids, topical corticosteroids,
Nevus Comedonicus
Nevus Comedonicus
Nevus Comedonicus
• Closely arranged slightly elevated papules, with keratin plugs resembling comedos.
• Inflammatory Cysts, abcesses, fistulas, scars
• Onset usually before age 10, but variable
• Linear array on trunk most common
• Tx: Acne sx, Tretinoin, Isotretinoin to control inflammatory cysts, not comedos.
Clear Cell Acanthoma
Clear Cell Acanthoma
• AKA Degos Acanthoma or Acanthome cellules claires of Degos and Civatte
• Circumscribed reddish moist nodule with crusting, peripheral scale, collarette
• 1-2 cm, shin, calf, thigh, asymptomatic, slow growing
• SCC has been reported• Tx: EDC, Shave biopsy, Excision, Cryo
Seborrheic Keratosis
Seborrheic Keratosis
• Onset 4th-5th decade
• Chest and back most common
• Etiology: Local arrest of maturation of keratinocytes.
• Borst Jadhasson phenomenon may occur, this is normal.
• Sign of Leser Trelat
Sign of Leser Trelat
• Sudden appearance of numerous itchy SK’s
• Validity controversial
• 60% Adenocarcinoma of Stomach
• Lymphoma, Breast CA, Lung SCC.
• For sign to be valid SK’s must parallel the course of the cancer, ie, resolve with removal of cancer.
Borst-Jadhasson Phenomenon
• Clonal variant
• “Nested”
• Diagnosis is still SK
• R/O porocarcinoma via neg. CEA stain.
• R/O Bowen’s via lack of atypical cells
Inverted Follicular Keratosis
Inverted Follicular Keratosis
• Irritated SK?
• 2-10mm papules
• Flesh colored
• Firm w/ central scaling
• Sharply marginated
• Squamous Eddies
• Tx: shave
Dermatosis Papulosa Nigra
Dermatosis Papulosa Nigra
• 35% of African Americans. Asians also.
• Favors malar, periorbital, then spreads down to neck and upper chest.
• Variant of SK?
• Irregular Acanthosis and heavy deposits of pigment at the basal layer.
• Tx: Light electrodessication with curettage.
Stucco Keratosis
Stucco Keratosis
• AKA Keratoelastoidosis verrucosa
• Males >40 years old.
• “Stuck on” appearance
• Lower legs near Achilles tendon
• Easily scratched off
• Histo: Hyperkeratotic SK
• Tx: Lac Hydrin 12%, Emollients
Multiple Minute Digitate Hyperkeratosis
Multiple Minute Digitate Hyperkeratosis
• AKA Spiny keratoderma
• 3 types- AD, Sporadic and Post-inflamm.
• No associated abnormalities
• 6 families described
• Post-inflammatory variant usually result of irradiation therapy.
Hyperkeratosis Lenticularis Perstans (Flegel’s Disease)
Hyperkeratosis Lenticularis Perstans (Flegel’s Disease)
Hyperkeratosis Lenticularis Perstans (Flegel’s Disease)
• Rough yellow-brown plaques, 2-5mm
• Small psoriasiform discs
• Insteps, dorsum of feet, legs, M 30-40 yo.
• Histology characteristic
• Defect in membrane coating granules (Odland bodies)
• Lac-Hydrin, Corticosteroids, 5-FU
HK & PK overlying a thinned epidermis, irreg. acanthosis at
periphery, band-like infilt.
Warty Dyskeratoma
• Brown reddish papule with soft yellowish central keratotic plug.
• Face, neck, scalp.
• Histology is characteristic…
Keratotic Plug, Cup-like Invagination
IE lacunae containing acantholytic cells and pseudovilli
Corps ronds and grains
Benign Lichenoid Keratosis
• Solitary papules
• Dusky red/violaceous
• Women, photodist.
• Forearms, hands, chest
• Tx: LN
Colloid or Civatte bodies in BLK
• LP-like
• Parakeratosis
• Lichenoid Infiltrate
• DIF + IgM @ DEJ
• Plasmas, Eos, Lymphs
• Histo mimics MF, LP
Arsenical Keratoses
• Keratotic pointed 2-4mm warty lesions
• Palms, soles, may be hyperpigmented
• When picked off leaves a cup shaped dell
• “Taking drops” (Fowlers solution)
• “Pills” (Asiatic pills)
• Check for a history of AD, Asthma, PV.
• Histo: same as AK, Tx: same as AK
Arsenical Keratosis
• Arsenic is an ubiquitous elemental metal
• Pesticides, rodentcides, herbicides
• Dessicants, feed additives
• Pressure treated lumber – shipbuilders, carpenters
• American cigarette tobacco in 1960’s
• Chinese proprietary medicines
Actinic Keratosis
• Multiple, discreet, flat or elevated, verrucous or keratotic, red, pigmented or skin colored usually with adherent scale but sometimes smooth
• Photodistributed, 3-10mm
• Hypertrophic AK may become cutaneous horn, and SCC may be present at the base.
Actinic Keratosis
• 0.25% to 20% risk of nonmelanoma CA
• P53 mutation present in SCC and AK usu.
• Be most suspicious of AK’s on lip, temple and hand as higher risk metastasis if SCC.
• Risk of SCC metastasis is related to thickness, so palpate “AK’s” before deciding whether to destroy vs biopsy.
Actinic Keratosis
• Risk factors other than UV:
• Tanning beds
• X-rays
• Polycyclic aromatic hydrocarbons
• Arsenic exposure
• Thermal injuries, Scars, HPV
• Organ transplants, BCC/SCC ratio flips
Cutaneous Horn
Cutaneous Horn
• Face, scalp, hands, penis, eyelid
• Horny excresences, skin colored
• Diagnosis at the base varies, often benign
• 55% SK, VV, Angioma, Tricholemmoma
• 25% AK
• 20% SCC or BCC
• More malignancy in elderly, fair skin
Leukoplakia
• Whitish thickening of mucosal epithelium
• Glistening, opalescent, may be reticulated or pigmented
• Attempts to remove it cause bleeding
• Lips, gums, cheeks and edges of tongue
• May arise on genitalia, anus
• Males over age of 40
Leukoplakia
• Degree of dysplasia varies
• Premalignant features seen in only 10-20%
• Dysplasia is clinically impossible to predict
• MC Chronic course in which malignant transformation follows 1-20 year lag time.
Leukoplakia
• Vulvar often mistaken for LS&A
• Penile more often Erythroplasia of Queyrat
• Risks: UV, Biter, Smoker, esp. pipe
• Oral Hairy Leukoplakia is associated with AIDS and is virally induced, white corrugated plaques MC seen on tongue.
Leukoplakia Treatments
• If dysplastic complete removal is the goal.
• Cryo, CO2 Laser
• Removal with advancement flap of inner mucosa moved forward
• Isotretinoin 1 to 2mg/kg/day for 3 months.
• 5-FU
Leukoplakia with Tylosis and Esophageal Carcinoma
• Extremely rare, AD• PPK age 5-15• Howell-Evans Synd.• 38x risk esoph ca• TOC gene 17q25• H-E Synd 17q23• Variable oral
leukokeratosis and follicular keratosis
White Sponge Nevus
White Sponge Nevus
• Spongy overgrowth of mucosa
• MC Buccal, but can be vaginal or rectal
• AD Mut of K4, K13
• Tetracycline is helpful
• EM show aggregated tonofilaments.
• Histo: Acanthosis, Vacuolated prickle cells and acidophilic condensations in cytoplasm.
Oral Florid Papillomatosis
Oral Florid Papillomatosis
• Distinctive “Cauliflower” white mass
• Covering tongue & adjacent mucosa
• Slow growing, fungating, no Lymphadenop.
• Well differentiated SCC- mets rare, late
• Progressive, may become SCC
• AKA Verrucous Carcinoma
• TX: Surgical Excision
Elastotic Nodules of Antihelix
Elastotic Nodules of Antihelix
• Bilateral semitranslucent nodules
• Exclusively upper antihelix location
• “Orange Peel” surface appearance
• Histo: HK, basal cell proliferation, collagen replaced by amorphous elastotic material.
• Frequently mistaken for BCC.
• Sun damage suspected as etiology.
Keratoacanthoma
• 4 types
• Solitary
• Multiple
• Eruptive
• KA Centrifugum Marginatum
Solitary KA
Multiple KA
• Ferguson-Smith type of multiple self-healing Keratoacanthomas
• MC-face, 3-10 lesions, usu. young men
• Ferguson-Smith type of self-healing squamous epithelioma
• Familial. Key is pruritis leading to mistaken diagnosis of prurigo nodularis
Multiple KA
Eruptive KA
• Immunosuppression is key
• SLE, Leukemia, Leprosy, Kidney transplant, photochemotherapy, thermal burn, radiation therapy have all been associated.
• Lesions may be in linear array
• Pruritis sometimes associated.
Eruptive KA – Generalized, esp. shoulders and arms, but palms and soles are spared
Eruptive KA – oral lesions, bilateral ectropion and narrowing of oral aperture
KA Centrifugum Marginatum
KA Centrifugum Marginatum
KA Centrifugum Marginatum
• 16 cases
• Regressing SCC variant
• Peripheral expansion with central healing leaving atrophy
• Dorsum hands, pretibial
• No tendency for spontaneous resolution
KA- Treatment
• Can spontaneously involute, but impossible to tell how long it will take.
• IL Bleomycin 1mg/mL, dil. w/ Xylocaine