The epidermal nevus syndrome (ENS) is an uncommon neurocutaneous disorder in which epidermal nevi are found in association with congenital abnormalities of the brain, eye, and/or skeleton. The association of epidermal nevi and neurologic abnormalities was comprehensively described by Schimmelpenning in 1957. Pavone et al. (1991) identified a homogeneous variant of ENS with hemimegalencephaly, g...

Epidermal nevi are hamartomas that are characterized by hyperplasia of epidermis and adnexal structures. These nevi may be classified into a number of distinct variants, which are based on clinical morphology, extent of involvement, and the predominant epidermal structure in the lesion. Variants include verrucous epidermal nevus, nevus sebaceous, nevus comedonicus, eccrine nevus, apocrine nevus...

Apocrine nevus is a rare benign tumor of which only 9 cases have been reported in the literature. The apocrine nevus is seen mostly in the areas where the apocrine glands are normally distributed such as axillae, head and neck. Our case was a 16-year old girl who referred with a verrucous erythematous plaque over right parietal side of the scalp. The patient had the plaque since birth with part...

Becker's nevus, also called Becker's melanosis or pigmented hairy epidermal nevus, is a benign cutaneous hamartoma characterized by hyperpigmented macule usually with hypertrichosis. It is rarely associated with neurofibromatosis. We report a 2 1⁄2 yrs old male child with coexistent congenital Becker’s nevus and type 1 neurofibromatosis.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a unilateral skin disorder which usually manifests in infancy or childhood. Female predominance, frequent leg involvement, pruritus, resistance to treatment, and a psoriasiform histologic pattern are other common characteristics of this disease. Atypical presentations of ILVEN have been described and include late onset in life, wide...

Becker's nevus is a relatively common acquired focal epidermal melanotic hypermelanosis usually found in the region of the shoulder girdle of young men. Various skeletal malformations and cutaneous dermatoses have been reported in Becker's nevus. Osteoma cutis is a rare disorder characterized by compact bone formation in the dermis or subcutaneous tissue. Secondary ossification on nevi has been...