Congenital cerebriform melanocytic naevus with cutis verticis gyrata

Introduction

Naevus refers to a variety of hamartomatous or non-neoplastic proliferation of abnormal mixtures of tissue′s normal components.[1] Congenital melanocytic naevus of> 20 cm is a rare occurrence.[2] It is one of the causes of cutis verticis gyrata which presents as hypertrophy with parallel folds of skin. The common sites of occurrence are the parietal, occipital and the temporal regions of the scalp.[3]

Case Report

A 25- year-old man presented to us with decreased hair over back of scalp and increased growth of hair on upper back since birth. He gave a history of oozing and crusting at the age of two years for which he took treatment and the lesions healed. Various ayurvedic preparations and topical applications were used with no relief. There was no history of epilepsy or mental retardation.

Examination revealed 32 x 10 cm naevus from back of scalp to upper back, scarring alopecia in upper part, parallel folds of skin in middle part and hypertrophy and hypertrichosis in the lower part. [Figure - 1]. A 2. 5 cm hairy pigmented solitary plaque is seen over the right arm.

Bio-psy from the rugose area showed dermis with groups and nests of round to oval naevus cells, some with melanin pigment in their cytoplasm, few cells in the lower portion were spindle shaped [Figure - 2].

Discussion

Congenital melanocyfic naevus is seen in 1-2 % of newborns.[4] The naevus cells are derived from epidermal melanocytes. [5] According to the size there are three variants. 1. < 1. 5 cm-small, 2. 1. 5-20 cm - intermediate, 3. > 20 cm - giant variety′ A rare form of giant variety is cerebriform melanocytic naevus. A size of > 10 cms is seen in 1 out of 20,000 newborns.[6] The naevus may present as a convoluted mass over the scalp which may not be pigmented. They become larger prone f o r malignancy. Most common malignancy seen is malignant melanoma, in 1. 8-42% cases.[7] Cutis verticis gyrata is hypertrophy of scalp skin with parallel or gyrate folds. Various causes of cutis verticis gyrata are:- Hereditary, traumatic, endocrinal, inflammatory, tumours and in association with other conditions. The commonest cause of cutis verticis gyrata amongst the tumours is cerebriform melanocytic naevus.[1] The main modalities of treatment are tissue expanders, dermabrasjon excision and skin grafting.[8] We present this case for its extensive nature and the rare occurrence of the two conditions in the same patient.