Hypopigmented mycosis fungoides in childhood and adolescence: A long-term retrospective study

Ekaterina Castano, Sharon Glick, Lucia Wolgast, Rizwan Naeem, Jaya Sunkara, Dirk Elston, Mark Jacobson

Research output: Contribution to journalArticle

41 Scopus citations

Abstract

Patients with hypopigmented mycosis fungoides (HMF) present at a younger age than those with classic MF. Our goal was to describe the clinical presentation, histopathologic features and long-term outcome in patients who developed HMF before the age of 21. It was observed that among 69 pediatric patients diagnosed with MF between 1992 and 2010, 50 had HMF. Thirty-five patients had clinical follow-up. There were 37 males and 32 females with a mean age of 13.6 years. Most patients were African American or Hispanic and presented with multiple hypopigmented patches. All biopsies showed epidermotropism of T-lymphocytes, whereas fibroplasia and lichenoid infiltrate were variable. All specimens tested were CD8+. Treatment modalities included topical steroids, narrow band ultraviolet B and psoralen and ultraviolet A. HMF patients were followed for <1-12 years. Most children responded to treatment, but recurrence rates were high. One patient progressed to plaque/tumor stage. Others did not progress; however, many were lost to follow-up. We present a large cohort of children with HMF and report on the features of disease and progression. A major difference in histology of HMF was lack of fibroplasia and lichenoid infiltrate, probably because of presentation in the early patch stage. Most patients have a waxing-and-waning course and relapse after discontinuation of therapy, requiring repetitive treatment.

Original languageEnglish (US)
Pages (from-to)924-934
Number of pages11
JournalJournal of cutaneous pathology
Volume40
Issue number11
DOIs
StatePublished - Nov 1 2013

Keywords

  • T-cell receptor rearrangement
  • cutaneous T cell lymphoma
  • histopathology
  • immunocytochemistry
  • mycosis fungoides

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology

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