Primary Cutaneous Anaplastic Large Cell Lymphoma Arising in a Patient with Rhupus Syndrome and Sjogren's Syndrome

Zirui Gao; Qianxi Xu; Xue Chen; Dandan Mao; Jianzhong Zhang; Jiang Jin

doi:10.2147/CCID.S366789

Primary Cutaneous Anaplastic Large Cell Lymphoma Arising in a Patient with Rhupus Syndrome and Sjogren's Syndrome

Clin Cosmet Investig Dermatol. 2022 May 30:15:975-979. doi: 10.2147/CCID.S366789. eCollection 2022.

Authors

Zirui Gao¹, Qianxi Xu¹, Xue Chen¹, Dandan Mao¹, Jianzhong Zhang¹, Jiang Jin¹

Affiliation

¹ Department of Dermatology, Peking University People's Hospital, Beijing, People's Republic of China.

Abstract

Rhupus syndrome, as an overlap syndrome of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), is relatively rare because of their substantially different immunopathological mechanisms. Herein, we report the first case of primary cutaneous anaplastic large cell lymphoma (PC-ALCL) in a patient with rhupus syndrome and Sjogren's syndrome and review the relevant literature. A 52-year-old Chinese woman with a history of rhupus syndrome and Sjogren's syndrome was treated with methotrexate, who developed gradually increasing nodules on the waist. Histopathological studies showed that the dermis and subcutaneous tissue were infiltrated with medium-to-large, atypical lymphocytes with the oval nucleus. The tumor cells showed CD3-, CD4-, CD8-, CD30+, LCA+, and EBV-encoded RNA (EBER) in situ hybridization (ISH) was positive. Therefore, the patient was diagnosed with PC-ALCL. Both immune disorders and EBV infection may be related to the onset of PL-ALCL, and further studies are needed to clarify the pathogenesis.

Keywords: Sjogren’s syndrome; lymphoproliferative disease; methotrexate; rheumatoid arthritis; systemic lupus erythematosus.

Publication types

Case Reports

Grants and funding

No funding or sponsorship was received for the conduct of this study or the preparation of this article.