Epidermal Nevi, Neoplasms and Cysts Part 1

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Epidermal Nevi, Neoplasms and Cysts Part 1

• Dan Ladd, DO
• January 28, 2003

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A brief review.
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Keratinizing Epidermal Nevi

• Hyperkeratosis without cellular atypia
• Nevus cells do not occur
• Follow Blaschkos Lines
• Linear Verrucous Epidermal Nevus
• ILVEN
• Epidermal Nevus Syndrome

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Linear Verrucous Epidermal Nevus
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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

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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.

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LVEN
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Blaschkos lines

• Albert Blaschko
• 1901
• Do not follow nerves, lymphatics or vessels.
• Proposed embryologic origin

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LVEN following Blaschkos lines
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ILVEN
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ILVEN

• Inflammatory Linear Verrucous Epidermal Nevus.
• Pruritic, usually on female extremity.
• Onset usually childhood, can be 40s, 50s
• Chronic, resistant to topical or IL treatments
• Psoriasiform histo linear psoriasis?
• Tx Deep shave excision, dermabrasion, Protopic?

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Epidermal Nevus Syndrome
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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

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Nevoid Hyperkeratosis of the Nipple
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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,

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Nevus Comedonicus
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Nevus Comedonicus
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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.

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Clear Cell Acanthoma
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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

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Seborrheic Keratosis
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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

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Sign of Leser Trelat

• Sudden appearance of numerous itchy SKs
• Validity controversial
• 60 Adenocarcinoma of Stomach
• Lymphoma, Breast CA, Lung SCC.
• For sign to be valid SKs must parallel the
  course of the cancer, ie, resolve with removal of
  cancer.

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Borst-Jadhasson Phenomenon

• Clonal variant
• Nested
• Diagnosis is still SK
• R/O porocarcinoma via neg. CEA stain.
• R/O Bowens via lack of atypical cells

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Inverted Follicular Keratosis
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Inverted Follicular Keratosis

• Irritated SK?
• 2-10mm papules
• Flesh colored
• Firm w/ central scaling
• Sharply marginated
• Squamous Eddies
• Tx shave

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Dermatosis Papulosa Nigra
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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.

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Stucco Keratosis
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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

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Multiple Minute Digitate Hyperkeratosis
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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.

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Hyperkeratosis Lenticularis Perstans (Flegels
Disease)
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Hyperkeratosis Lenticularis Perstans (Flegels
Disease)
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Hyperkeratosis Lenticularis Perstans (Flegels
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

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HK PK overlying a thinned epidermis, irreg.
acanthosis at periphery, band-like infilt.
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Warty Dyskeratoma

• Brown reddish papule with soft yellowish central
  keratotic plug.
• Face, neck, scalp.
• Histology is characteristic

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Keratotic Plug, Cup-like Invagination
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IE lacunae containing acantholytic cells and
pseudovilli
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Corps ronds and grains
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Benign Lichenoid Keratosis

• Solitary papules
• Dusky red/violaceous
• Women, photodist.
• Forearms, hands, chest
• Tx LN

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Colloid or Civatte bodies in BLK

• LP-like
• Parakeratosis
• Lichenoid Infiltrate
• DIF IgM _at_ DEJ
• Plasmas, Eos, Lymphs
• Histo mimics MF, LP

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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

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Arsenical Keratosis

• Arsenic is an ubiquitous elemental metal
• Pesticides, rodentcides, herbicides
• Dessicants, feed additives
• Pressure treated lumber shipbuilders,
  carpenters
• American cigarette tobacco in 1960s
• Chinese proprietary medicines

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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.

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Actinic Keratosis

• 0.25 to 20 risk of nonmelanoma CA
• P53 mutation present in SCC and AK usu.
• Be most suspicious of AKs on lip, temple and
  hand as higher risk metastasis if SCC.
• Risk of SCC metastasis is related to thickness,
  so palpate AKs before deciding whether to
  destroy vs biopsy.

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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

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Cutaneous Horn
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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

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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

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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.

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Leukoplakia

• Vulvar often mistaken for LSA
• 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.

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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

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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

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White Sponge Nevus
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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.

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Oral Florid Papillomatosis
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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

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Elastotic Nodules of Antihelix
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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.

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Keratoacanthoma

• 4 types
• Solitary
• Multiple
• Eruptive
• KA Centrifugum Marginatum

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Solitary KA
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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

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Multiple KA
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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.

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Eruptive KA Generalized, esp. shoulders and
arms, but palms and soles are spared
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Eruptive KA oral lesions, bilateral ectropion
and narrowing of oral aperture
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KA Centrifugum Marginatum
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KA Centrifugum Marginatum
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KA Centrifugum Marginatum

• 16 cases
• Regressing SCC variant
• Peripheral expansion with central healing leaving
  atrophy
• Dorsum hands, pretibial
• No tendency for spontaneous resolution

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KA- Treatment

• Can spontaneously involute, but impossible to
  tell how long it will take.
• IL Bleomycin 1mg/mL, dil. w/ Xylocaine