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Journal of the American Academy of Dermatology
Volume 71, n° 6
pages 1117-1126 (décembre 2014)
Doi : 10.1016/j.jaad.2014.07.049
accepted : 25 July 2014
Original Articles

Clinical presentation, immunopathology, and treatment of juvenile-onset mycosis fungoides: A case series of 34 patients
 

Scarlett Boulos, MD a, d, Reena Vaid, MD a, Tariq N. Aladily, MD b, Doina S. Ivan, MD c, Rakhshandra Talpur, MD a, Madeleine Duvic, MD a,
a Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas 
b Department of Hematopathology, University of Texas MD Anderson Cancer Center, Houston, Texas 
c Department of Dermatopathology, University of Texas MD Anderson Cancer Center, Houston, Texas 
d Department of Dermatology, Wright State University Boonshoft School of Medicine, Dayton, Ohio 

Correspondence to: Madeleine Duvic, MD, Department of Dermatology, University of Texas MD Anderson Cancer Center, 1400 Pressler Ave, Unit 1452, Houston, TX 77030.
Abstract
Background

Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, typically presents in middle-aged to elderly individuals.

Objective

We sought to study the demographics, clinicopathologic features, treatment response, and prognosis of patients with biopsy-proven MF diagnosed before 20 years of age.

Methods

Patients were identified from a prospectively collected database for retrospective analysis.

Results

Of 1902 patients with MF, 34 had juvenile-onset MF: 41% were stage IA, 56% were stage IB, and 3% were stage IIB at diagnosis. The male to female ratio was 1.1:1. The median age of symptom onset was 9 years (range 3-19 years), with a delay in diagnosis between 1 month and 14 years. Patients primarily presented with hypopigmented (53%), hyperpigmented (29%), and pink-violaceous (41%) patches/plaques. Immunohistochemistry revealed 39% with CD8+ immunophenotype, 67% of which had hypopigmented lesions. The phototherapy response rate in 21 patients was 81%. All patients who completely responded to narrowband ultraviolet B phototherapy had hypopigmented MF.

Limitations

This is a single cancer center study.

Conclusion

Juvenile-onset MF presents with early-stage disease with an overrepresentation of hypopigmented MF and CD8+ immunophenotype. Narrowband ultraviolet B is an effective treatment option for juveniles, especially for those with the hypopigmented variant.

The full text of this article is available in PDF format.

Key words : cutaneous T-cell lymphoma, immunopathology, juvenile onset, mycosis fungoides, narrowband ultraviolet B radiation, presentation, treatment, vitamin-D deficiency

Abbreviations used : MF, NB, PUVA, TCR, UV



 Supported by the Sherry L. Anderson Research Fund.
 Conflicts of interest: None declared.
 Reprints not available from the authors.



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