Cutis laxa: a case report and an update.

ABSTRACT

Very few cases of Cutis Laxa are reported worldwide. This is the first case report of its kind from Bangladesh. A 10 year-old girl looking very aged was admitted in the paediatric ward of Dhaka Medical College with most striking skin feature. There was loosely hanging skin over the face which was inelastic, hyper extensible and which recoils slowly after stretching. She had downward slanting palpebral fissures, periorbital oedema, a broad flat nose with everted nostril, sagging cheeks, and large and soft ears. Skin biopsy showed fragmentation of the elastic tissue with collagen tissue in the dermis, which was suggestive of Cutis Laxa. Her chest x-ray showed hypertranslucent lung fields, prominence of pulmonary conus area and some ring like opacity near the lower zone with cardiomegaly, which is suggestive of corpulmonale with bronchiectasis. Her echocardiography showed dilated right atrium, ventricle and pulmonary artery which were suggestive of corpulmonale. All these confirmed the diagnosis.