Subcutaneous Panniculitis-like T-cell Lymphoma Lacking Subcutaneous Tumor Mimicking Adult-onset Still's Disease

• Published in: Internal Medicine Vol. 62; no. 21; pp. 3231 - 3235
• Main Authors: Tada, Maria, Kachi, Shion, Onozawa, Masahiro, Fujieda, Yuichiro, Yoshida, Shota, Oki, Yotaro, Kamada, Kazuro, Nagai, Jun, Okada, Satomi, Kikuchi, Ryo, Hisada, Ryo, Hasegawa, Yuta, Ohigashi, Hiroyuki, Goto, Hideki, Hashimoto, Daigo, Nakazato, Shinichi, Matsuno, Yoshihiro, Teshima, Takanori, Atsumi, Tatsuya
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01-11-2023; Japan Science and Technology Agency
• Subjects: adult-onset Still's disease (AOSD); Case Report; Case reports; Chemotherapy; Cyclosporins; Erythema; Genetic screening; HAVCR2; Hemophagocytic syndrome; Inflammasomes; Internal medicine; Juvenile rheumatoid arthritis; Lymphocytes T; Phenotypes; Remission; subcutaneous panniculitis-like T-cell lymphoma (SPTCL); T-cell lymphoma; TIM-3; Adult-onset Still's disease (AOSD); TIM-3; Subcutaneous panniculitis-like T-cell lymphoma (SPTCL); HAVCR2
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.1419-22

We herein report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) resembling adult-onset Still's disease (AOSD). A 40-year-old woman presented with a fever, erythema, and painful subcutaneous nodules on the trunk. Laboratory data and a bone marrow analysis showed hemophagocytic syndrome. Although AOSD was suspected, based on a histopathological evaluation of the erythema, she was diagnosed with SPTCL. She was refractory to combination chemotherapy but achieved durable remission with cyclosporine monotherapy. Genetic testing revealed a homozygous HAVCR2 c.245A>G variant (rs184868814) that had caused NLRP3 inflammasome activation. SPTCL and AOSD share a pathogenesis in terms of NLRP3 inflammasome activation, so the clinical phenotype of SPTCL reasonably mimics AOSD.