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

Area of Science:

• Dermatology
• Oncology
• Pediatrics

Background:

• Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma.
• Hypopigmented mycosis fungoides (HMF) is a variant that typically affects younger patients.
• Understanding HMF in pediatric populations is crucial for early diagnosis and management.

Purpose of the Study:

• To describe the clinical presentation, histopathologic features, and long-term outcomes of pediatric HMF patients.
• To analyze treatment responses and disease progression in children diagnosed with HMF.
• To identify key differences in HMF histology in pediatric versus adult cases.

Main Methods:

• Retrospective review of 69 pediatric MF cases diagnosed between 1992 and 2010.
• Analysis of clinical data, histopathology, and treatment outcomes for 50 HMF patients.
• Long-term follow-up data for 35 pediatric HMF patients.

Main Results:

• 50 of 69 pediatric MF patients had HMF, with a mean age of 13.6 years.
• Most pediatric HMF patients were African American or Hispanic, presenting with multiple hypopigmented patches.
• Biopsies showed epidermotropism of CD8+ T-lymphocytes; fibroplasia and lichenoid infiltrate were variable. Most patients responded to treatment, but recurrence rates were high, with one progression to plaque/tumor stage.

Conclusions:

• Pediatric HMF presents differently from classic MF, often lacking significant fibroplasia or lichenoid infiltrate in early stages.
• HMF in children typically follows a waxing-and-waning course with frequent relapses after therapy cessation.
• Effective management of pediatric HMF requires long-term monitoring and repetitive treatments due to high recurrence rates.