APLASIA CUTIS CONGENITA – A CASE SERIES OF VARIED PRESENTATIONS

Abstract

Aplasia cutis congenita (ACC) belongs to a heterogeneous group of disorder, characterised by localised absence of skin and in some cases subcutaneous tissues, involving multiple possible body locations. The most common site is the scalp (70%), however any skin site can be affected including the trunk and the extremities . Herein we report a series of 4 cases of aplasia cutis congenita with varied cutaneous manifestations ranging from absence of epidermis to deeper defects extending upto the dermis and subcutaneous tissue. Our rst case presented to us with congenital absence of skin with ulceration and eschar formation of extremities, buttocks and bullae formation over hands and lower back. Clinicopathological diagnosis was made to be aplasia cutis congenita associated with epidermolysis bullosa (Bart’s syndrome). The second case presented with a single, well dened, rhomboid shaped deep ulceration with overlying eschar formation over the vertex extending upto the right parietal region of scalp, diagnosed to be a case of Type 2 Aplasia cutis congenita - Adams-Oliver syndrome. Our third case was a supercial variant of aplasia cutis congenita with linear , erythematous, blanchable atrophied parchment like scar on the back, without any underlying bony deformity. The last was a sporadic case of bitemporal aplasia cutis congenita with thinning of skin over bilateral temporal areas with overlying telangiectasia.