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Epidermal Nevi, Neoplasms and Cysts

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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 ... – PowerPoint PPT presentation

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Title: Epidermal Nevi, Neoplasms and Cysts


1
Epidermal Nevi, Neoplasms and Cysts Part 1
  • JoAnne M. LaRow, D.O.
  • March 23, 2004

2
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

3
Keratinizing Epidermal Nevi
  • Hyperkeratosis without cellular atypia
    characteristic of all
  • Nevus cells do not occur

4
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

5
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

6
Linear Verrucous Epidermal Nevus
7
  • Note shedding of scale within several lesions

8
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.

9
  • Linear erythematous scaly plaque with a
    psoriasform appearance on leg

10
  • Acanthotic epidermis with zones of parakeratosis
    devoid of a granular layer alternating with zones
    of orthohyperkeratosis

11
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?

12
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

13
LVEN
14
Blaschkos lines
  • Albert Blaschko
  • 1901
  • Do not follow nerves, lymphatics or vessels.
  • Proposed embryologic origin

15
LVEN following Blaschkos lines
16
ILVEN
17
Epidermal Nevus Syndrome
18
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

19
Nevoid Hyperkeratosis of the Nipple
20
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

21
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

22
Nevus Comedonicus
23
Nevus Comedonicus
24
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

25
Clear Cell Acanthoma
26
  • A erythematous papule on lower extremitynote
    peripheral scale erosion in superior portion
  • Bpsoriasiform epidermis contains large pale
    keratinocytes

27
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

28
Seborrheic Keratosis
29
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.

30
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)

31
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

32
Inverted Follicular Keratosis
33
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

34
Inverted Follicular Keratosis
  • Irritated SK?
  • 2-10mm papules
  • Flesh colored
  • Firm w/ central scaling
  • Sharply marginated
  • Squamous Eddies
  • Tx shave

35
  • An endophytic proliferation of keratinocytes with
    prominent squamous eddies

36
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

37
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

38
Dermatosis Papulosa Nigra
39
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

40
Stucco Keratosis
41
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.

42
Multiple Minute Digitate Hyperkeratosis
43
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46
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)

47
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

48
Hyperkeratosis Lenticularis Perstans (Flegels
Disease)
49
Hyperkeratosis Lenticularis Perstans (Flegels
Disease)
50
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

51
  • A.multiple symmetric keratotic papules on shins
  • B. spinous layer is maredly thinned, there is a
    lichenoid infiltrate obvious hyperkeratosis

52
HK PK overlying a thinned epidermis, irreg.
acanthosis at periphery, band-like infilt.
53
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

54
Keratotic Plug, Cup-like Invagination
55
  • 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

56
Corps ronds and grains
57
Benign Lichenoid Keratosis
  • Solitary papules
  • Dusky red/violaceous
  • Women, photodist.
  • Forearms, hands, chest
  • Tx LN

58
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

59
Colloid or Civatte bodies in BLK
  • LP-like
  • Parakeratosis
  • Lichenoid Infiltrate
  • DIF IgM _at_ DEJ
  • Plasmas, Eos, Lymphs
  • Histo mimics MF, LP

60
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

61
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

62
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63
  • A. guttate hypopigmentation superimposed on
    hyperpigmentaion resembles raindrops on a dusty
    road
  • B. arsenical keratoses on plantar surface

64
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65
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.

66
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.

67
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

68
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

69
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

70
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

71
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

72
Cutaneous Horn
73
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

74
  • Sharply demarcated, white plaque involving
    ventral surface of tongue floor of mouth

75
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.

76
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

77
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

78

79
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

80
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.

81
White Sponge Nevus
82
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

83
Oral Florid Papillomatosis
84
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

85
Elastotic Nodules of Antihelix
86
Keratoacanthoma
  • 4 types
  • Solitary
  • Multiple
  • Eruptive
  • KA Centrifugum Marginatum

87
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

88
Solitary KA
89
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

90
Multiple KA
91
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

92
Eruptive KA Generalized, esp. shoulders and
arms, but palms and soles are spared
93
Eruptive KA oral lesions, bilateral ectropion
and narrowing of oral aperture
94
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)

95
KA Centrifugum Marginatum
96
KA Centrifugum Marginatum
97
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

98
  • KA-note keratin-filled crater

99
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

100
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)
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