Gunther′s Disease

Abstract

A three year old female child of Gunther’s disease with blistering over exposed parts of body, mutilation of nose and fingers, scanning alopecia and erythrodontia, is reported. Her urine was strongly positive for uropoprhyrinogen. The blistering could not be prevented by strict avoidance of sun, sunscreen with high SPF, chloroquine, high doses of activated charcoal and betacarotene, though there was significant symptomatic improvement by repeated packed erythrocyte transfusion.