CD20-positive subcutaneous panniculitis-like T-cell lymphoma presenting as polycranial neuropathy: A CARE-compliant case report and literature review

Medicine (Baltimore). 2022 Sep 2;101(35):e30233. doi: 10.1097/MD.0000000000030233.

Abstract

Background: Subcutaneous panniculitis-like T-cell lymphoma(SPTCL) is a very rare cytotoxic T-cell skin lymphoma involving subcutaneous tissue, and mainly affects young females. T-cell phenotype is characterized by CD3+, CD8+, and CD4-. SPTCT with polycranial neuropathy has rarely been described. SPTCL is believed to show an indolent clinical course unless patients develop haemophagocytic syndrome or sudden respiratory failure. Its treatment has not been established yet.

Case presentation: We report a case of intractable SPTCT in a 66-year-old woman with multiple cranial nerve palsies and diabetes. She showed involvement of the bilateral facial nerve, left trigeminal nerve, left auditory nerve, and right oculomotor nerve. The single inconspicuous skin lesion in the trunk presented with an erythematous nodule with a diameter of <5 cm and a slightly pink infiltrated plaque. Electromyography revealed bilateral damage to the facial nerve. Differential immunohistochemical characteristics were observed. Immunohistochemistry demonstrated diffuse CD20 positivity. Cerebral spinal fluid analysis revealed elevated protein levels of 0.92 (0.15-0.45) g/L. Her condition regressed severely over time. She was treated with chemotherapy but died 10 months later, the probable cause of death was lung involvement.

Conclusion: The patient's involvement with the central nervous system may be associated with positivity for CD20. Molecular biomarkers may act as therapeutic targets for SPTCL.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Female
  • Humans
  • Lymphoma, T-Cell* / complications
  • Lymphoma, T-Cell* / diagnosis
  • Lymphoma, T-Cell* / drug therapy
  • Lymphoma, T-Cell, Cutaneous* / drug therapy
  • Panniculitis* / diagnosis
  • Panniculitis* / drug therapy
  • Panniculitis* / etiology
  • Peripheral Nervous System Diseases* / diagnosis
  • Peripheral Nervous System Diseases* / etiology
  • Skin Neoplasms* / pathology

Supplementary concepts

  • Subcutaneous panniculitis-like T-cell lymphoma