Columnar epidermal necrosis - A unique manifestation of transfusion-associated cutaneous graft-vs-host disease

Background: in 1978, the first case of columnar epi dermal necrosis was rep orted in a 6-year-old boy. There were scaly, partially vesicular or crusty, erythematous lesions mainly involving the extremities that histopathologic ally showed peculiar features of focal, total epidermal necrosis accompanie d by a lichenoid tissue reaction. He developed the skin eruption after rece iving a blood transfusion from his mother when he showed debility induced b y vaccination with an alternated live measles virus vaccine. The lesions ra pidly regressed after sun exposure. To our knowledge, there has been no rep ort of a similar case despite such unique features. Observation: We encountered a similar case of columnar epidermal necrosis i n a 15-year-old Japanese girl with chronic graft-vs-host disease; the lesio ns occurred 3 months after the transfusion of peripheral blood stem cells f rom her HLA antigen-matched brother. However, there was no exacerbation of liver dysfunction, diarrhea, or bone marrow aplasia. The peculiar cutaneous lesions responded well to topical phototherapy. Conclusion: These 2 patients shared a similarity in their lesions and circu mstances under which the blood transfusion was performed to a debilitated p atient from a close family member. We believe that focal epidermal necrosis observed in patients with this condition represents a variant of blood tra nsfusion-associated lichenoid graft-vs-host disease that occurs uniquely in a skin-targeted fashion.