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August 2009

Adult-Onset Erythropoietic Porphyria in the Setting of MDS

Arch Dermatol. 2009;145(8):948-949. doi:10.1001/archdermatol.2009.161

In September 2004, an 81-year-old man with myelodysplastic syndrome (MDS) presented with a 6-week history of painful, pruritic lesions on his ears, scalp, and the dorsal surface of his hands. His urine was red-brown. The use of alcohol and/or new medications and exposure to chemicals and/or radiation were denied. Biopsy specimens revealed subepidermal blistering with few polymorphonuclear cells, abundant solar elastotic material, and homogeneous eosinophilic material surrounding upper dermis small vessels, all fairly nonspecific findings. Urine porphyrin analysis revealed massively elevated porphyrin levels in a pattern consistent with congenital erythropoietic porphyria (CEP). Bacitracin ointment and diphenhydramine treatments were started, but the patient was lost to follow-up.