Atypical clinicopathologic presentation of primary cutaneous diffuse large B-cell lymphoma, leg type

J Am Acad Dermatol. 2015 Jun;72(6):1016-20. doi: 10.1016/j.jaad.2015.02.1134. Epub 2015 Mar 29.

Abstract

Background: Primary cutaneous diffuse large B-cell lymphoma, leg type (cDLBCL-LT) is a well-defined entity of cutaneous B-cell lymphoma affecting predominantly elderly patients, mostly women. The typical clinical presentation is characterized by solitary or multiple, rapidly growing plaques or tumors on 1 leg (rarely both legs).

Objective: We sought to describe a new clinical variant of cDLBCL-LT that deviates from the conventional one.

Methods: Clinical, histopathologic, phenotypical, and molecular features of 3 cases of cDLBCL-LT presenting with patches or thin plaques were reviewed (all were women, aged 60, 62, and 87 years; lesions were located on the leg in all patients).

Results: These patients presented with patches or thin plaques that represented the first manifestation of cDLBCL-LT. All 3 patients reported a history of long-standing lesions (present for 6, 9, and 18 months, respectively). Histology revealed moderately dense, perivascular infiltrates of small lymphocytes admixed with variable numbers of large cells that were CD20(+), Bcl-2(+), and MUM-1(+).

Limitations: There were only a small number of cases.

Conclusions: We reported an unusual clinical presentation of cDLBCL-LT that deviates from the conventional one and that represents a formidable diagnostic challenge. Biopsy specimens of unusual patches/thin plaques or annular lesions should be obtained from the legs of adult patients if the lesions do not respond to conventional treatment.

Keywords: annular erythema; clinical presentation; cutaneous B-cell lymphoma; leg type; primary cutaneous diffuse large B-cell lymphoma.

MeSH terms

  • Aged, 80 and over
  • Biopsy, Needle
  • Chemoradiotherapy / methods
  • Combined Modality Therapy
  • Female
  • Follow-Up Studies
  • Hematopoietic Stem Cell Transplantation / methods
  • Humans
  • Immunohistochemistry
  • Leg
  • Lymphoma, Large B-Cell, Diffuse / diagnosis
  • Lymphoma, Large B-Cell, Diffuse / mortality
  • Lymphoma, Large B-Cell, Diffuse / pathology*
  • Lymphoma, Large B-Cell, Diffuse / therapy*
  • Lymphoma, T-Cell, Cutaneous / diagnosis
  • Lymphoma, T-Cell, Cutaneous / mortality
  • Lymphoma, T-Cell, Cutaneous / pathology*
  • Lymphoma, T-Cell, Cutaneous / therapy*
  • Middle Aged
  • Risk Assessment
  • Sampling Studies
  • Skin Neoplasms / mortality
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / therapy*
  • Survival Rate
  • Tomography, X-Ray Computed / methods
  • Treatment Outcome