A Case of Aplasia Cutis Congenita, Type VII

Abstract

Aplasia cutis congenita (ACC) is a rare congenital defect in which localized or widespread areas of the skin are absent at birth. In the majority of cases, it is limited to the scalp especially on the vertex although other areas of the body may also be involved. Other congenital malformations can be associated with ACC. We present herein the case of a new born male with unilateral absence of skin on the extensor surface of the right lower leg. There was no associated malformation or skin disease such as blistering or nail abnormailty. According to the classification outlined by Frieden, the condition was diagnosed as type VII aplasia cutis congenita. The treatment of this large ulcer was conservative, wet dressing and prophylactic topical antibiotics. On follow up after 2 years showed that the patient was nearly cured of the ulcer and had only minimal scar formation.

Keywords: Aplasia cutis congenita; Leg; Unilateral.

Fig. 1. Large ulcer about 20 cm length along the right leg without nail dystrophy. On the day of birth. Arrow indicates biopsy site.

Fig. 2. A skin biopsy specimen taken from the lesion (A) showed absence of epidermis and inflammatory infiltrate and proliferation of blood vessels in the dermis, when compared with proximal normal skin (B) (H&E, ×100).

Fig. 3. Clinical features 15 days after birth. New hyperproliferative granulation tissue has appeared and blood clots were observed.