Invisible mycosis fungoides: A diagnostic challenge

J Am Acad Dermatol. 2000 Feb;42(2 Pt 2):324-8. doi: 10.1016/s0190-9622(00)90103-8.

Abstract

We describe a 76-year-old woman who presented persistent generalized pruritus as the only cutaneous manifestation of a cutaneous T-cell lymphoma (mycosis fungoides). No cutaneous lesions were observed throughout the patient's course. Skin biopsies obtained from normal-looking pruritic skin revealed a discrete perivascular lymphocytic infiltrate in the upper dermis and focal intraepidermal clusters of atypical lymphoid cells (Pautrier's microabscesses). PCR analysis of TCR-gamma gene disclosed a monoclonal T-cell rearrangement. Sequencing of the PCR monoclonal product identified the J(8)V(2)C(2) TCR gene rearrangement. This observation illustrates the existence of a peculiar and exceedingly rare form of mycosis fungoides characterized only by persistent pruritus unresponsive to several therapeutic approaches. The diagnostic difficulties of this rare variant are stressed.

Publication types

  • Case Reports
  • Duplicate Publication

MeSH terms

  • Aged
  • Female
  • Humans
  • Immunohistochemistry
  • Mycosis Fungoides / complications
  • Mycosis Fungoides / diagnosis*
  • Mycosis Fungoides / metabolism
  • Mycosis Fungoides / pathology
  • Polymerase Chain Reaction
  • Pruritus / etiology
  • Skin / pathology
  • Skin Neoplasms / complications
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / metabolism
  • Skin Neoplasms / pathology