Purpura as a cutaneous association of sickle cell disease

A common chronic feature of sickle cell disease is the presence of painful, punched-out leg ulcers. Other cutaneous findings in patients with homozygo us sickle cell disease have not been described in the literature. We presen t a case of a 50-year-old black woman with sickle cell disease who was admi tted for acute onset of arm and hip pain. After admission she deteriorated clinically, with multiorgan failure and mental status changes. Examination of the skin revealed erythematous papules and plaques with scaly centers an d purpura on the upper trunk. The clinical differential diagnosis was vascu litis versus sepsis. Skin biopsy of two representative lesions was performe d. Hematoxylin- and eosin-stained sections showed a superficial perivascula r mixed inflammatory infiltrate with numerous eosinophils and extravasated erythrocytes, some of which exhibited bizarre morphology of sickled red blo od cells. These findings indicated that the patient's cutaneous lesions, po ssibly multifactorial in origin, could be a component of her sickle cell cr isis. This case is presented as an unusual one in which evaluation of eryth rocyte morphology contributed to patient management and to emphasize the im portance of examining erythrocyte morphology as a part of the histologic ev aluation of stained tissue.