Aplasia cutis congenita is a rare skin condition characterized by the absence of localized or widespread areas of skin at birth. We are reporting a variant aplasia cutis congenita, which involved over 90% of the body surface area, which occurred in a baby born to a mother with pemphigus vulgaris who was on oral prednisolone and azathioprine. A case of extensive aplasia cutis congenita was seen and oral intake of azathioprine by the mother during pregnancy was suspected as an etiologic factor.

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... e parents of the patient did not consent for biopsy or autopsy so the histopathological picture was not available and hence involvement of other systems could not be ascertained. Due, to financial constraints some of the investigations which might have helped in assigning the patient to a particular category of aplasia cutis such as karyotyping and CT scan brain could not be carried out.