Lymphomatoid papulosis types D and E: a multicentre series of the French Cutaneous Lymphomas Study Group

Clin Exp Dermatol. 2021 Dec;46(8):1441-1451. doi: 10.1111/ced.14730. Epub 2021 Jul 28.

Abstract

Background: Lymphomatoid papulosis (LyP) type D (LyP D) and type E (LyP E) have recently been described in small series of cases or isolated case reports.

Aim: To further describe the clinical and histological features of LyP D and E based on a retrospective multicentre study.

Methods: The clinical and histopathological features of 29 patients with an initial diagnosis of LyP D or LyP E were retrospectively assessed using standardized forms.

Results: After exclusion of 5 cases, 24 patients (14 LyP D, 10 LyP E) were enrolled in the study. The median follow-up was 2.5 years (range 1 month to 13 years). LyP D was characterized by multiple recurrent self-regressing small papules that developed central erosion or necrosis, whereas LyP E presented as papulonodular lesions that rapidly evolved into necrotic eschar-like lesions > 10 mm in size. Epidermal changes were more frequent in LyP D, whereas dermal infiltrates were deeper in LyP E. Anaplastic cytology was rare and the DUSP22 rearrangement was never observed. Two patients (8%) had an associated cutaneous lymphoma.

Conclusion: LyP D and E have distinct clinical findings and may be associated with other cutaneous lymphomas.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Age of Onset
  • Female
  • Follow-Up Studies
  • Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
  • Humans
  • Hyperplasia
  • Immunophenotyping
  • Lymphomatoid Papulosis / classification*
  • Lymphomatoid Papulosis / genetics
  • Lymphomatoid Papulosis / pathology*
  • Male
  • Middle Aged
  • Necrosis
  • Neoplasm Recurrence, Local / pathology
  • Retrospective Studies
  • Skin Neoplasms / classification*
  • Skin Neoplasms / genetics
  • Skin Neoplasms / pathology*
  • Skin Ulcer / pathology