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

Intern Med. 2023 Nov 1;62(21):3231-3235. doi: 10.2169/internalmedicine.1419-22. Epub 2023 Mar 15.

Abstract

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.

Keywords: HAVCR2; TIM-3; adult-onset Still's disease (AOSD); subcutaneous panniculitis-like T-cell lymphoma (SPTCL).

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Erythema
  • Female
  • Humans
  • Inflammasomes
  • Lymphoma, T-Cell* / diagnosis
  • Lymphoma, T-Cell* / pathology
  • NLR Family, Pyrin Domain-Containing 3 Protein
  • Panniculitis* / diagnosis
  • Panniculitis* / genetics
  • Panniculitis* / pathology
  • Still's Disease, Adult-Onset* / diagnosis

Substances

  • NLR Family, Pyrin Domain-Containing 3 Protein
  • Inflammasomes

Supplementary concepts

  • Subcutaneous panniculitis-like T-cell lymphoma