Subcutaneous Panniculitis-like T-cell Lymphoma with a HAVCR2 Mutation Diagnosed after 10 Years of Treatment with Glucocorticoids and Cyclosporine as Lupus Panniculitis

Intern Med. 2023 May 15;62(10):1537-1540. doi: 10.2169/internalmedicine.0428-22. Epub 2022 Sep 28.

Abstract

Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a very rare cutaneous T cell lymphoma that has been reported to be associated with autoimmune disorders but is most commonly associated with systemic lupus erythematosus. We herein report a 26-year-old man thought to have lupus panniculitis (LP) treated for 10 years with corticosteroids and cyclosporine. After several relapses with panniculitis, he was finally diagnosed with SPTCL, which was confirmed to have a HAVCR2 mutation for p.Tyr82Cys. We emphasize that rheumatologists should be aware of the possibility of SPTCL, despite its rare appearance, when making a diagnosis of LP or when encountering clinical manifestations that are not consistent with LP.

Keywords: cyclosporine; immunosuppressant drugs; lupus panniculitis; subcutaneous panniculitis-like T cell lymphoma.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cyclosporine / therapeutic use
  • Diagnosis, Differential
  • Glucocorticoids / therapeutic use
  • Hepatitis A Virus Cellular Receptor 2
  • Humans
  • Lymphoma, T-Cell* / diagnosis
  • Lymphoma, T-Cell* / drug therapy
  • Lymphoma, T-Cell* / genetics
  • Male
  • Mutation
  • Neoplasm Recurrence, Local / diagnosis
  • Panniculitis* / diagnosis
  • Panniculitis* / drug therapy
  • Panniculitis* / genetics
  • Panniculitis, Lupus Erythematosus* / diagnosis
  • Panniculitis, Lupus Erythematosus* / pathology
  • Skin Neoplasms* / diagnosis

Substances

  • Glucocorticoids
  • Cyclosporine
  • HAVCR2 protein, human
  • Hepatitis A Virus Cellular Receptor 2

Supplementary concepts

  • Subcutaneous panniculitis-like T-cell lymphoma