Epidermal Nevi, Neoplasms and Cysts – Part 1 Epidermal Nevi, Neoplasms and Cysts – Part 1

Epidermal Nevi, Neoplasms and Cysts – Part 1

JoAnne M. LaRow, D.O.

March 23, 2004

Keratinizing Epidermal Nevi

• Aka hard nevus of Unna

• Soft epidermal nevus

• Nevus verrucosus (verrucous nevus)

• Nevus unius lateris

• Linear epidermal nevus

• Systematized nevi

• Ichthyosis hystrix

Keratinizing Epidermal Nevi

• Hyperkeratosis without cellular atypia characteristic of all

• Nevus cells do not occur

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”(linear hyperkeratotic

papules & plaques-often showing a parallel arrangement);unilateral or bilateral (often symmetrical)

• Extensive + CNS abnormalities = Syndrome


• Histology: hyperkeratosis, acanthosis, & papillomatosis (60% of the time)

• 16% show epidermolytic hyperkeratosis

• Rare malignancies have been reported:trichoepithelioma, keratoacanthoma, & verruciform xanthoma

• Note shedding of scale within several lesions


• 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.

• Linear erythematous scaly plaque with a psoriasform appearance on leg

• Acanthotic epidermis with zones of parakeratosis devoid of a granular layer alternating with zones of orthohyperkeratosis

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?

ILVEN

• Believed to be a type of epidermal nevus• Familial patterns reported & sporadic mosaic

forms exist• May be associated with CHILD syndrome

(congential hemidysplasia with ichthyosiform erythroderma & limb defects)

• Differs from LEN by presence of erythema & pruritus clinically & histologically by inflammation & parakeratosis

Blaschko’s lines

• Albert Blaschko

• 1901

• Do not follow nerves, lymphatics or vessels.

• Proposed embryologic origin

LVEN following Blaschko’s lines

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• Isolated finding, unassociated with other conditions• Unilateral NHN Should be distinguished from breast

carcinoma via biopsy, in addition, mammography may be warranted.

• Bx results identical to seb. K.• Course varies, unpredictable.• Tx: Keratolytics such as Lactic Acid 12%, Salicylic

acid Gel 6%, topical corticosteroids; oral retinoids are ineffective

Nevus Comedonicus

• Closely arranged slightly elevated papules, with keratin plugs resembling comedos.

• Hamartomas of pilosebaceous unit-resulting in dilated, keratin-filled pores

• Rarely pruritic; usually asymptomatic • Onset usually before age 10, but variable• Most common site is face, then trunk• Tx: difficult;localized lesions:excise; manual extraction,

dermabrasion, keratolytics(helpful); ammonium lactate applied every 2 weeks has been helpful to remove keratin plugs

Nevus Comedonicus

Clear Cell Acanthoma

• AKA Degos Acanthoma or Acanthome cellules claires of Degos and Civatte

• Usually solitary lesion on leg• Blanchable, erythematous, discrete papule or

plaque; may have attached ‘wafer-like’ scale at periphery

• 1-2 cm, shin, calf, thigh, asymptomatic, slow growing

• SCC has been reported• Tx: EDC, Shave biopsy, Excision, Cryo

Clear Cell Acanthoma

• A: erythematous papule on lower extremity;note peripheral scale & erosion in superior portion

• B:psoriasiform epidermis contains large pale keratinocytes

Seborrheic Keratosis• Onset 4th-5th decade• Chest and back most common• Only on hair-bearing areas• Etiology: Local arrest of maturation of keratinocytes.• At least 6 different types of histologic types:

acanthotic, hyperkeratotic, reticulated, clonal, irritated, & melanoacanthoma

• Borst Jadhasson phenomenon may occur, this is normal.

• Sign of Leser Trelat

Seborrheic Keratosis

Sign of Leser Trelat

• Sudden appearance of numerous itchy SK’s

• Pathogenesis? Neoplasm may secrete growth factor leading to epithelial hyperplasia

• 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

• Descrete groups or ‘clones’ of basaloid, squamatized, or pale keratinocytes in epidermis appear different than their neighbors

• This can be benign or malignant

• Mainly seen with irritated seborrheic keratosis, Bowen’s dx, & rarely with hidroacanthoma simplex ( a form of eccrine poroma limited to epidermis)

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


• Bengin lesion of middle-aged & older adults

• Typically solitary; most commonly on face & neck

• Asymptomatic, firm, white-tan to pink papule

• Histologically, endophytic growth pattern with squamous eddies & inflammation


• Irritated SK?

• 2-10mm papules

• Flesh colored

• Firm w/ central scaling

• Sharply marginated

• Squamous Eddies

• Tx: shave

• An endophytic proliferation of keratinocytes with prominent squamous eddies

Dermatosis Papulosa Nigra

• Multiple hyperpigmented sessile to filiform papules

• Most common in individuals of African descent with darkly pigmented skin

• Found almost exclusively on face ( malar & forehead areas)

• Histologic features similar to acanthotic SKs


• Familial predisposition• Variant of SK? Delayed presentation of nevoid

condition like patterned lentiginoses? Variant of acrochordon?

• Irregular Acanthosis and heavy deposits of pigment at the basal layer.

• Tx: Light electrodessication with curettage, cryo( may produce hypopigmentation), snip excision or curettage without electrodesiccation

Stucco Keratosis

• AKA Keratoelastoidosis verrucosa or keratosis alba

• Males >40 years old.• “Stuck on” appearance• Lower legs near Achilles tendon• Easily scratched off• Histo: Hyperkeratotic SK• Tx: Lac Hydrin 12%, Emollients or topical

retinoids

Stucco Keratosis

Multiple Minute Digitate Hyperkeratosis

• AKA Spiny keratoderma• 3 types-AD, sporadic type, and postinflammtory• All characterized by multiple minute keratotic

papules unassociated with follicular orifices• A spiked projection occurs at top of papule• No associated abnormalities• 6 families described• Post-inflammatory variant usually result of

irradiation therapy.

Multiple Minute Digitate Hyperkeratosis

Hyperkeratosis Lenticularis Perstans (Flegel’s Disease)

• Very rare, possibly AD disorder• Multiple keratotic papules with disc-like appearance is

symmetric distribution• Usually expressed mid-to-late adulthood-cases as young

as 13 yrs reported• Individual papules are small-1-5mm; larger

lesions>5mm often have collarette of scale& bleed when scale removed

• Most common on dorsum of feet, legs, palms & soles too• Flegel’s dx has been associated with endocrine disorders

(DM & hyperthyroidism)

Flegel’s Disease

• Etiology unknown• Absent or altered keratinocyte membrane-

coating granules (Odland bodies) on microscopic exam

• Lipid-by-products within Odland bodies felt to influence stratum corneum desquamation, & if absnt or abnormal hyperkeratosis may occur

Hyperkeratosis Lenticularis Perstans (Flegel’s Disease)

Flegel’s Disease

• Pathology: discrete compact hyperkeratotic mound, contrasting with normal basket-weave cornified layer of normal epidermis

• Focal parakeratosis & hypogranulosis

• Thin atropic stratum spinosum often sharply indented or depressed at lateral margin

• Band-like infiltrate or lymphocytes present in papillary dermis along with dilated blood vessels

• A.multiple symmetric keratotic papules on shins

• B. spinous layer is maredly thinned, & there is a lichenoid infiltrate & obvious hyperkeratosis

HK & PK overlying a thinned epidermis, irreg. acanthosis at

periphery, band-like infilt.

Warty Dyskeratoma

• Solitary skin-colored to red-brown papule or nodule with a central pore containing a keratotic plug

• Usually located on head: face, neck, scalp.

• Relatively uncommon, without a genetic predisposition

• No malignant degeneration has been reported

• Histology is characteristic…

Keratotic Plug, Cup-like Invagination

• A portion of cup-shaped lesion is seen. The central keratotic plug is seen on left.

• Lower portion of cup is occupied by numerous villi with acantholytic epithelium

Corps ronds and grains

Benign Lichenoid Keratosis

• Solitary papules

• Dusky red/violaceous

• Women, photodist.

• Forearms, hands, chest

• Tx: LN

Lichenoid Keratosis• Aka: lichen planus-like-keratosis, solitary lichen

planus, solitary lichenoid keratosis

• Solitary, usually asymptomatic, lesion

• Most commonly on upper chest or forearms

• Represents an inflammatory stage of solar lentigo, actinic keratosis, or seborrheic keratosis

• Histologically, appears almost identical to lichen planus

Colloid or Civatte bodies in BLK

• LP-like

• Parakeratosis

• Lichenoid Infiltrate

• DIF + IgM @ DEJ

• Plasmas, Eos, Lymphs

• Histo mimics MF, LP

Arsenical Keratoses• Precancerous papules seen most often on palms & soles• Present as symmetric, punctate, yellow, corn-like

papules 2-10 mm • Common areas are thenar & hypothenar eminences,

distal plams, lateral fingers & dorsal interphalangeal joints

• Weight-bearing plantar surfaces on feet• Persistent lesions may coalesce into kertotic plaques• SCC may arise, often producing pain, bleeding,

fissuring, or ulceration

Arsenical Keratosis

• Arsenic is ubiquitous elemental metal• Exists in nature as metalloids, alloys, and chemical

compounds• Deposited into water, soil, & vegetation• Pesticides, rodentcides, herbicides• Dessicants, feed additives• Pressure treated lumber – shipbuilders, carpenters• American cigarette tobacco in 1960’s(mostly from use

of arsenic-containing insecticides)• Chinese proprietary medicines

• A. guttate hypopigmentation superimposed on hyperpigmentaion resembles ‘raindrops on a dusty road’

• B. arsenical keratoses on plantar surface

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

Actinic Keratoses

• Tx: varied• Cryo with liquid nitrogen most effective & practical with

limited number of sites• Repetitive superficial freezes (3 cycles) very effective &

minimizes scarring• Healing usually occurs within 1 week on face & up to 4

weeks on arms & legs• 5-fluorouracil topically for extensive, broad or numerous

lesions• Fluoroplex crm or solution, 1%, or Efudex, 2% recommended

for face; 5% Efudex crm for trunk, scalp, hands, arms, neck

Actinic Keratosis

• Rub 5-FU gently BID for 3-4 weeks on head & neck, 4-6 weeks for other areas, or until there is a severe inflammatory rxn

• 1% solution is effective on lips• Use extreme care around eyes & mouth• Individual sensitivity differs:burning rxn will

occur within several days• Stop tx when peak response occurs-characterized

by color change from bright to dusky red, by re-epithelialization, & crust formation

Actinic Keratosis

• Another tx cycle for –FU is 4 times daily for 7-21 days

• Shorter cycle may result in better compliance?• Topical application of a 20% solution of

aminolevulinic acid to lesions followed by exposure to a red light source (580-740 nm) (photodyna,ic therapy)

• Dermabrasion for severe ak’s useful on hairless scalp

• Chemical peels, CO2 laser

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

Cutaneous Horn

Leukoplakia

• Whitish thickening of mucosal epithelium• Glistening, opalescent, may be reticulated or

pigmented• Attempts to remove it cause bleeding• Common sites: floor of mouth, lateral & ventral

surfaces of tongue, soft palate• May arise on genitalia, anus• Seen mainly in males over age of 40• Non-homogeneous lesions & those of tongue & floor

of mouth have higher rate of malignant transformation

• Sharply demarcated, white plaque involving ventral surface of tongue & floor of mouth

Leukoplakia• Bx reveals orthokeratosis or parakeratosis with

minimal inflammation or varying degrees of dysplasia • Benign form usually occurs from chronic irritation;

very little chance of conversion to dysplasia• Premalignant features seen in only 10-20%• Dysplasia is clinically impossible to predict• Changes may be more or less hyperemia &

tenderness-with bleeding tendency• Most commonly has a chronic course in which

malignant transformation follows 1-20 year lag time.

Leukoplakia

• Vulvar often mistaken for LS&A; mainly in obese women after menopause

• Penile more often Erythroplasia of Queyrat• Risks: UV, Biter, Smoker, esp. pipe• Oral Hairy Leukoplakia (white, corrugated

plaques occurring primarily on sides of tongue)in pt with AIDS - virally induced lesion

Leukoplakia Treatments

• If dysplastic complete removal is the goal.• Cryo, CO2 Laser, surgical excision.• In actinic cheilitis + leukoplakia of tongue

exposed surface of lip may be removed & replaced by sliding forward mocosa from inner aspect of lip

• Cryotherapy is effective or Isotretinoin 1 to 2mg/kg/day for 3 months or 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

• Spongy overgrowth of mucosa• Most common on buccal, but can be vaginal or rectal• No extramucosal lesions; no tx• Progression stops at puberty-usually• AD mut of K4, K13• Tetracycline is helpful• EM show aggregated tonofilaments.• Histo: Acanthosis, vacuolated prickle cells and

acidophilic condensations in cytoplasm.

White Sponge Nevus

Oral Florid Papillomatosis

• Distinctive “Cauliflower” white mass• Covering tongue & adjacent mucosa• Slow growing, fungating, no lymphadenop.• Expect eventual epidermoid carcinoma in most pts• Well differentiated SCC- mets rare, late• Progressive, may become SCC• AKA Verrucous Carcinoma• TX: Surgical Excision; however often recurs &

spreads; recombinant-alpha 2a interferon + CO2 laser has been used

Oral Florid Papillomatosis

Elastotic Nodules of Antihelix

• Bilateral semi-translucent 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.• Tx: removal via shave excision & fulguration of

base

Elastotic Nodules of Antihelix

Keratoacanthoma

• 4 types

• Solitary

• Multiple

• Eruptive

• KA Centrifugum Marginatum

Solitary KA

• Type of KA rapidly growing papule enlarging from 1mm to as large as 25 mm in 3-8 weeks

• Fully developed is dome-shaped, skin-colored nodule with a smooth crater filled with central keratin plug

• Smooth shiny lesion is sharply demarcated from its surroundings

• Telangiectases may run through it

Solitary KA

Multiple KA

• Ferguson-Smith type of multiple self-healing keratoacanthomas

• Histologically & clinically identical to solitary type • Most common on face, 3-10 lesions localized to 1 site

usu. young men• Familial type-Ferguson-Smith type of self-healing

squamous epithelioma • Key is pruritis leading to erroneous dx of pruritus

nodularis

Multiple KA

Eruptive KA• Eruption of dome-shaped, skin-colored papules from 2-7

mm in diameter• Eruption is generalized but spares palms & soles• Oral mucous membranes can be involved • 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, plus bilateral ectropin &

narrowing of oral aperture

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

• 16 cases (uncommon variant)

• Peripheral expansion with central healing leaving atrophy

• Dorsum hands, pre-tibial

• No tendency for spontaneous resolution (unlike giant solitary KA’s no tendency for spontaneous involution)

KA Centrifugum Marginatum

KA-Etiology

• ? Variant of regressing SCC

• Conditions known to promote progression of AK’s & malignant degeneration of premalignant lesions also promote expression & development of KA’s (sun exposure, tar therapy, & immunosuppressed states)

• Inflammatory cells in KA’s mostly are CD4+ T lymphoctes activated by interleukin 2 adhesion molecules-like inflammatory cells in SCC’s

• KA-note keratin-filled crater

KA- Treatment• Can spontaneously involute, but impossible to tell how long it will

take.• Grade I SCC cannot be excluded even with a bx-biopsy excision or

ED&C of an ordinary lesion < 2cm can & should be done –safest course

• 5-FU solution straight from ampule & intralesional (0.5- 1 ml of 25 mg/ml methotrexate)

• IM methotrexate (25 mg/week)• IL Bleomycin (1mg/mL, dil. w/ Xylocaine)-clearing occurred

within 20 days of tx• Recommend excision if involution is not complete after 3 weeks of

topical tx• Mohs’ sx for facial lesions

KA-tx

• Podophyllum in compound tincture of benzoin useful in giant keratoacanthomas

• Oral retinoids are therapeutic & helpful in large or recalcitrant lesions

• Eruptive forms tend to be very resistant to tx-good results achieved with oral & topical retinoids & cyclophosphamide

• Radiation tx on giant KA’s• Intralesional IFN-alpha-1 report of regression of 5

of 6 large (>2cm) KA’s (Grob et al)

Epidermal Nevi, Neoplasms and Cysts – Part 1 Epidermal Nevi, Neoplasms and Cysts – Part 1

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Transcript of Epidermal Nevi, Neoplasms and Cysts – Part 1 Epidermal Nevi, Neoplasms and Cysts – Part 1