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Atypical lymphocytic lobular panniculitis.

Cynthia M Magro1, A Neil Crowson, John C Byrd

  • 1Department of Pathology, St. John Medical Center and Regional Medical Laboratories, Tulsa, OK, USA. magro-1@medctr.osu.edu

Journal of Cutaneous Pathology
|March 10, 2004
PubMed
Summary

A new condition, atypical lymphocytic lobular panniculitis, is identified in patients with lymphoma-like skin lesions. This distinct panniculitis presents with specific microscopic, phenotypic, and genotypic features, differing from aggressive subcutaneous T-cell lymphoma.

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Area of Science:

  • Dermatopathology
  • Hematology
  • Oncology

Background:

  • Subcutaneous T-cell lymphoma (SCTCL) is aggressive, but some patients present with indolent, waxing and waning lesions without systemic symptoms.
  • This study investigates a cohort of patients with lymphocytic panniculitis and lymphoid atypia who did not meet full SCTCL criteria.

Purpose of the Study:

  • To characterize a distinct form of lymphocytic panniculitis.
  • To differentiate this condition from aggressive SCTCL and other related disorders.

Main Methods:

  • Prospective enrollment of twelve patients with lymphocytic panniculitis and lymphoid atypia.
  • Comprehensive clinical, histological, phenotypic (flow cytometry), and genotypic (T-cell receptor rearrangement) analyses were performed.

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Main Results:

  • Patients exhibited a chronic, waxing and waning course; some had periodic fevers and cytopenias.
  • Histology revealed atypical lymphocytes (CD4+ or CD8+) expanding fat lobules, with diminished CD5/CD7 expression in most.
  • Clonal T-cell receptor-gamma rearrangements were detected in 10 of 13 biopsies, indicating a neoplastic T-cell process.

Conclusions:

  • A new term, atypical lymphocytic lobular panniculitis, is proposed for this unique clinicopathological entity.
  • This condition is characterized by a specific profile of microscopic, phenotypic, and genotypic findings.
  • Further research is needed to understand the natural history and optimal management of atypical lymphocytic lobular panniculitis.