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Hypopigmented mycosis fungoides in childhood and adolescence: a long-term retrospective study.
J Cutan Pathol. 2013 Nov; 40(11):924-34.JC

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.

Authors+Show Affiliations

Department of Pathology, Montefiore Medical Center, Bronx, NY, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

24320808

Citation

Castano, Ekaterina, et al. "Hypopigmented Mycosis Fungoides in Childhood and Adolescence: a Long-term Retrospective Study." Journal of Cutaneous Pathology, vol. 40, no. 11, 2013, pp. 924-34.
Castano E, Glick S, Wolgast L, et al. Hypopigmented mycosis fungoides in childhood and adolescence: a long-term retrospective study. J Cutan Pathol. 2013;40(11):924-34.
Castano, E., Glick, S., Wolgast, L., Naeem, R., Sunkara, J., Elston, D., & Jacobson, M. (2013). Hypopigmented mycosis fungoides in childhood and adolescence: a long-term retrospective study. Journal of Cutaneous Pathology, 40(11), 924-34. https://doi.org/10.1111/cup.12217
Castano E, et al. Hypopigmented Mycosis Fungoides in Childhood and Adolescence: a Long-term Retrospective Study. J Cutan Pathol. 2013;40(11):924-34. PubMed PMID: 24320808.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hypopigmented mycosis fungoides in childhood and adolescence: a long-term retrospective study. AU - Castano,Ekaterina, AU - Glick,Sharon, AU - Wolgast,Lucia, AU - Naeem,Rizwan, AU - Sunkara,Jaya, AU - Elston,Dirk, AU - Jacobson,Mark, PY - 2013/03/27/received PY - 2013/06/20/revised PY - 2013/08/11/accepted PY - 2013/12/11/entrez PY - 2013/12/11/pubmed PY - 2014/8/15/medline KW - T-cell receptor rearrangement KW - cutaneous T cell lymphoma KW - histopathology KW - immunocytochemistry KW - mycosis fungoides SP - 924 EP - 34 JF - Journal of cutaneous pathology JO - J Cutan Pathol VL - 40 IS - 11 N2 - 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. SN - 1600-0560 UR - https://www.unboundmedicine.com/medline/citation/24320808/Hypopigmented_mycosis_fungoides_in_childhood_and_adolescence:_a_long_term_retrospective_study_ L2 - https://doi.org/10.1111/cup.12217 DB - PRIME DP - Unbound Medicine ER -