Epidermal nevus

Synonym(s)

Organoid nevus; epithelial nevus

Nevus Comedonicus Syndrome
Epidermal nevus. Flesh-colored verrucous nodule on the scalp

Definition

Congenital hamartoma of embryonal ecto-dermal origin, classified on the basis of its main component, which may be keratinoc-ytic, sebaceous, sweat gland, or follicular

Pathogenesis

Probable somatic mutation, which may reflect genetic mosaicism; arises from pluripotential germinative cells of the basal layer of the embryonic epidermis; possible dermal effect on growth

Clinical manifestation

Nevus verrucosus (verucous epidermal nevus): usually present at birth or early childhood; solitary or multiple, linear or S-shaped, verrucous or velvety plaques, never crossing the midline; flexural lesions sometimes macerated and foul-smelling; lesions with sebaceous or apocrine elements may enlarge at puberty

Inflammatory epidermal nevus (ILVN): usually present in the first 5 years of life; pruritic, linear, erythematous, scaly plaques, most commonly on the leg; nevus comedonicus (comedo nevus): confluent clusters of dilated follicular orifices plugged with keratin, giving the appearance of aggregated open comedones; often arranged in a linear, arcuate, or zosteri-form pattern; occasionally paralleling the lines of Voigt or the lines of Blaschko Nevus unius lateris (linear epidermal nevus): solitary linear verrucous plaque, present at birth or in early infancy Nevus sebaceous (sebaceous nevus): usually present at birth; well-circumscribed, pink-to-yellow, smooth or velvety plaques, almost always on the head and neck area; enlarges and thickens at puberty; small risk of malignant degeneration to basal cell carcinoma

Epidermal nevus syndrome: one or more epidermal nevi and involvement of the nervous, ophthalmologic, and/or skeletal systems; mental retardation, seizures, movement disorders; intracranial and/or intraspinal lipomas

Differential diagnosis

Proteus syndrome; CHILD syndrome; wart; Darier disease; lichen striatus; incontinen-tia pigmenti; psoriasis; syndrome of Favre-Racouchot; acne vulgaris; mastocytoma; juvenile xanthogranuloma; xanthoma

Therapy

Nevus verrucosus: surgical excision*; tretinoin; acetretin; inflammatory epidermal nevus: super potent topical corticoster-

Epidermoid cyst 211

oids; cryotherapy; surgical excision; nevus comedonicus: tretinoin; surgical excision; nevus sebaceous: surgical excision*; epidermal nevus syndrome: as above for individual variants

References

Losee JE, Serletti JM, Pennino RP (1999) Epidermal nevus syndrome: a review and case report. Annals of Plastic Surgery 43(2):211-214

Therapy

Cryotherapy; electrodessication and curettage; sun avoidance

References

Majewski S, Jablonska S, Orth G (1997) Epidermodysplasia verruciformis. Immunological and nonimmunological surveillance mechanisms: role in tumor progression. Clinics in Dermatology i5(3):32i-334

0 0

Post a comment