Hemophagocytic syndrome presenting with a facial erythema in a patient with systemic lupus erythematosus

Hemophagocytic syndrome is a systemic reactive histiocytic proliferative disorder affecting the reticuloendothelial system. Skin lesions develop in 12.5% to 55.6% of the patients. We report a 35-year-old woman with systemic lupus erythematosus and Graves' disease complicated with parvovirus B19-associated hemophagocytic syndrome. She presented with slightly infiltrated erythematous macules and plaques with ulcers on the scalp, face, and back. A skin biopsy specimen taken from an area of facial erythema demonstrated typical phagocytic cells in the skin. Hemophagocytic syndrome-associated specific cutaneous lesions should be included in the differential diagnosis of facial and nonfacial erythema in patients with systemic lupus erythematosus.