A rare association of aplasia cutis congenita with ipsilateral linear verrucous epidermal naevus

Abstract

Aplasia cutis congenita (ACC) refers to congenital absence of skin occurring alone or in association with other anomalies. Congenital epidermal naevi are benign naevi of epidermal component present at birth or appearing in the first few years of life in characteristic developmental pattern. We are reporting the case of a 9-month-old female baby born of a non-consanguineous marriage reported to the department of dermatology for loss of skin over the right side of the scalp and lesions over the right upper limb since birth. Systemic examination did not reveal any abnormalities. The mother's antenatal history was uneventful. On examination erythematous, thin parchment-like hairless scar with crusting is present over the right frontoparietal region of the scalp. Hyperpigmented verrucous papules with surrounding erythematous skin present over right axilla, dorsum of right ring finger and hypopigmented macules present over extensor aspect of right forearm in blaschkoid distribution. According to modified Frieden's classification, aplasia cutis has been categorised into nine types. Around 86% of cases are seen over the vertex of the scalp. In the third type, ACC is associated with epidermal naevi. This is an unusual association, hence being reported here.