A 57-year-old man was referred to our department with scaly erythema over the face, trunk, and extremities. He had a history of metastatic thymoma and myasthenia gravis (MG). He underwent thymectomy 11 years prior and chemotherapy 9 years prior. He had been admitted to the department of neurology due to muscle weakness, fatigability, and dysgeusia. He presented with extensive erythema and scaling over his entire body, and was in a state of erythroderma. In addition, he had leg edema and white patches on the tongue. Skin biopsy from the thigh demonstrated hyperkeratosis with parakeratosis, epidermal liquefaction and spongiosis, apoptosis of keratotic cells in the epidermis, and lymphocytic infiltration into the epidermis and dermis. Based on the graft-versus-host disease (GVHD)-like skin symptoms, accompanied by thymoma and MG, we diagnosed the condition as thymoma-associated multi-organ autoimmunity (TAMA) complicated by MG. Topical corticosteroid treatment moderately improved the erythema. A 3 day course of intravenous methylprednisolone improved the dysgeusia. However, pneumonia occurred frequently due to Pseudomonas aeruginosa infections and we detected low immunoglobulin G (IgG) levels. He was promptly treated with antibiotics and supplemental intravenous immunoglobulin (5 g/day, three days). His general condition recovered temporarily, but he developed disseminated Mycobacterium abscessus infection and passed away 10 months after the first visit. TAMA was reported to affect the skin, liver, and colon, and it is clinicopathologically similar to GVHD. GVHDlike erythroderma is a cutaneous feature of TAMA. MG is a frequent autoimmune complication of TAMA, and TAMA can be accompanied by reduced serum IgG levels and Good syndrome. Patients with TAMA that exhibit GVHD-like erythroderma generally have an unfavorable clinical course related to severe infections. Skin Research, 20 : 343-348, 2021
View full abstract