16796 Graft-versus-host disease–like erythroderma associated with aggravation of thymoma

Abstract

Thymoma-associated multiorgan autoimmunity (TAMA) is a rare paraneoplastic disease defined as thymoma with liver, intestine or skin manifestations, which resembles graft-versus-host disease (GVHD) histopathologically in the absence of hematopoietic stem cell transplantation. A 76-year-old woman who had been diagnosed with invasive thymoma presented with scaly erythema and reddish papules across the trunk. She had been admitted to internal medicine because of febrile sensation and general fatigue suspecting meningitis. The initial differential diagnosis for her cutaneous lesions included drug eruption due to antibiotics and viral exanthem. Antibiotics were stopped and she was treated with topical steroid, oral antihistamine. But there was no effect on skin lesion, which then spread rapidly to the whole body. And she died of aggravation of thymoma a few days later. The pathologic findings of a biopsy specimen from the abdomen revealed parakeratosis, many apoptotic cells in the epidermal layer, perivascular and interface dermatitis with lymphocytic infiltration. Theses pathologic findings were similar to those seen in GVHD. Interestingly, she never underwent allogenic stem cell or solid-organ transplantation. Given the presence of GVHD-like skin symptoms and aggravation of thymoma, the diagnosis of thymoma associated GVHD-like erythroderma, one of the findings in thymoma-associated multiorgan autoimmunity, was made.