FAMILIAL PYODERMA-GANGRENOSUM PRESENTING IN INFANCY

Pyoderma gangrenosum (PG) is a rare, poorly understood skin disease th at occurs in all age groups. Less than 0.4% of patients are infants an d represent a diagnostic challenge as early lesions may resemble other skin disorders. Here we report for the first time three siblings affe cted with PG all presenting during infancy. Unlike the older age group , the ulcers spared the legs but involved the buttocks, thighs and per ianal area in all the infants. Conclusion This is the first reported f amily with PG affecting three siblings suggesting autosomal recessive inheritance. The diagnosis may be more difficult in infants due to abs ence of underlying associated disorders and the tendency of the lesion s to appear in areas where infants frequently have other dermatoses. P G characteristically involves the buttocks, thighs and perianal area a nd spares the legs.