Aplasia Cutis Congenita with rare association and unusual Presentation.

Abstract

Aplasia cutis congenita ( ACC) is a congenital absence of skin most commonly affecting scalp. In very rare instances it can involve extremities and trunk. It is usually diagnosed clinically at birth. Most cases are sporadic with a few reports of familial occurrence. Here, we present two cases of which one had scalp involvement correlating to antenatal methimazole intake and the other one had unusual presentation with involvement of deep tissues up to the periosteum of right lower limb. Both cases were managed conservatively and successfully discharged home. But unfortunately were lost to follow-up. ACC is a rare condition with varying degree of severity depending upon the extent and depth of the involvement of the skin and underlying tissues. Mild cases do not need specific intervention while severe cases need to be managed by a multidisciplinary team of pediatrician, dermatologist and plastic surgeon. Methimazole is a known teratogen associated with this condition hence should be avoided in pregnancy.