Epidermal Nevi, Neoplasms and Cysts

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Epidermal Nevi, Neoplasms and Cysts Part 1

• JoAnne M. LaRow, D.O.
• March 23, 2004

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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

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Keratinizing Epidermal Nevi

• Hyperkeratosis without cellular atypia
  characteristic of all
• Nevus cells do not occur

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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(linear hyperkeratotic
  papules plaques-often showing a parallel
  arrangement)unilateral or bilateral (often
  symmetrical)
• Extensive CNS abnormalities Syndrome

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Linear Verrucous Epidermal Nevus

• Histology hyperkeratosis, acanthosis,
  papillomatosis (60 of the time)
• 16 show epidermolytic hyperkeratosis
• Rare malignancies have been reportedtrichoepithel
  ioma, keratoacanthoma, verruciform xanthoma

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Linear Verrucous Epidermal Nevus
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• Note shedding of scale within several lesions

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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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• Linear erythematous scaly plaque with a
  psoriasform appearance on leg

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

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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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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

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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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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
• 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

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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 difficultlocalized lesionsexcise manual
  extraction, dermabrasion, keratolytics(helpful)
  ammonium lactate applied every 2 weeks has been
  helpful to remove keratin plugs

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Nevus Comedonicus
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Nevus Comedonicus
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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

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Clear Cell Acanthoma
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• A erythematous papule on lower extremitynote
  peripheral scale erosion in superior portion
• Bpsoriasiform epidermis contains large pale
  keratinocytes

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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

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Seborrheic Keratosis
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Sign of Leser Trelat

• Sudden appearance of numerous itchy SKs
• 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 SKs must parallel the
  course of the cancer, ie, resolve with removal of
  cancer.

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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,
  Bowens dx, rarely with hidroacanthoma simplex
  ( a form of eccrine poroma limited to epidermis)

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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

• 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

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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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• An endophytic proliferation of keratinocytes with
  prominent squamous eddies

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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

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Dermatosis Papulosa Nigra

• 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

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Dermatosis Papulosa Nigra
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Stucco Keratosis

• AKA Keratoelastoidosis verrucosa or keratosis
  alba
• Males gt40 years old.
• Stuck on appearance
• Lower legs near Achilles tendon
• Easily scratched off
• Histo Hyperkeratotic SK
• Tx Lac Hydrin 12, Emollients or topical
  retinoids

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Stucco Keratosis
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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.

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Multiple Minute Digitate Hyperkeratosis
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Hyperkeratosis Lenticularis Perstans (Flegels
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
  lesionsgt5mm often have collarette of scale bleed
  when scale removed
• Most common on dorsum of feet, legs, palms
  soles too
• Flegels dx has been associated with endocrine
  disorders (DM hyperthyroidism)

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Flegels 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

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

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• A.multiple symmetric keratotic papules on shins
• B. spinous layer is maredly thinned, there is a
  lichenoid infiltrate obvious hyperkeratosis

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

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Keratotic Plug, Cup-like Invagination
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• 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

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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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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

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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

• 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

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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 1960s(mostly from
  use of arsenic-containing insecticides)
• Chinese proprietary medicines

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• A. guttate hypopigmentation superimposed on
  hyperpigmentaion resembles raindrops on a dusty
  road
• B. arsenical keratoses on plantar surface

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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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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

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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 differsburning 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

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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 aks useful on hairless
  scalp
• Chemical peels, CO2 laser

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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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Cutaneous Horn
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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

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

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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.

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Leukoplakia

• Vulvar often mistaken for LSA 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

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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

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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

• 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.

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White Sponge Nevus
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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

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Oral Florid Papillomatosis
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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

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Elastotic Nodules of Antihelix
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Keratoacanthoma

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

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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

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Solitary KA
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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

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Multiple KA
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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

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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

• 16 cases (uncommon variant)
• Peripheral expansion with central healing leaving
  atrophy
• Dorsum hands, pre-tibial
• No tendency for spontaneous resolution (unlike
  giant solitary KAs no tendency for spontaneous
  involution)

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KA Centrifugum Marginatum
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KA Centrifugum Marginatum
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KA-Etiology

• ? Variant of regressing SCC
• Conditions known to promote progression of AKs
  malignant degeneration of premalignant lesions
  also promote expression development of KAs
  (sun exposure, tar therapy, immunosuppressed
  states)
• Inflammatory cells in KAs mostly are CD4 T
  lymphoctes activated by interleukin 2 adhesion
  molecules-like inflammatory cells in SCCs

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• KA-note keratin-filled crater

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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 EDC of an ordinary lesion
  lt 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

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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 KAs
• Intralesional IFN-alpha-1 report of regression of
  5 of 6 large (gt2cm) KAs (Grob et al)