epidermal nevi, neoplasms and cysts – part 1 dan ladd, do january 28, 2003

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