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Review
March 3, 2021

Mycosis Fungoides in Children and Adolescents: A Systematic Review

Author Affiliations
  • 1Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
JAMA Dermatol. Published online March 3, 2021. doi:10.1001/jamadermatol.2021.0083
Key Points

Question  What are the clinical features of childhood mycosis fungoides (MF)?

Findings  In this systematic review of 571 children and adolescents with MF, the most common subtype was the hypopigmented form, followed by classic MF. Most patients with MF presented with early-stage disease, and the prognosis of MF seems to be more favorable than in the general population, although a significant delay before the establishment of a correct diagnosis of MF in childhood was associated with a poor prognosis.

Meaning  These findings suggest that although the prognosis of childhood MF is not unfavorable, delayed diagnosis may have an adverse effect.

Abstract

Importance  Comprehensive data on childhood mycosis fungoides (MF) is scarce.

Objective  To describe clinical features, immunophenotypes, various treatment options, and prognosis of MF in children and adolescents.

Evidence Review  This systematic review searched MEDLINE via PubMed, Embase, Cochrane, and Scopus databases in October 2019. The search terms included mycosis fungoides, infant, children, and adolescent. No filter for the publication period was used, but studies written in a language other than English were excluded. Reference lists of the relevant articles were also searched manually. Case series and case reports were included if data on childhood MF were extractable. The Asan Medical Center database for cases of childhood MF was also searched. Patients were treated from January 1, 1990, to July 31, 2019, and were younger than 20 years at the time of diagnosis. The methodologic quality of the included studies was assessed with items from the Newcastle-Ottawa scale. Data were analyzed from December 9, 2019, to September 4, 2020.

Findings  A total of 571 unique patients were included. The mean (SD) age at diagnosis was 12.2 (4.2) years; at onset, 8.6 (4.2) years. The female-to-male ratio was 1:1.6 (350 male patients [61.3%]). Among 522 patients with data available at diagnosis, stage 1 disease constituted 478 cases (91.6%), followed by stage 2 (39 [7.5%]) and stage 4 (5 [1.0%]). Among the 567 patients with data available, the most common variant of MF was the hypopigmented form (309 [54.5%]), followed by classic MF (187 [33.0%]). The MF lesions were predominantly the CD4+ and CD8+ immunophenotype in 99 (49.5%) and 79 (39.5%) of 200 patients, respectively. Among the treatments, narrowband UV-B was the most frequently used (150 of 426 [35.2%]). Most patients were alive with the disease (185 of 279 [66.3%]); 83 of 279 (29.8%) were in complete remission; and 11 of 279 (3.9%) had died by the last follow-up. A longer time from onset to diagnosis (hazard ratio [HR], 1.24; 95% CI, 1.06-1.45), granulomatous slack skin (HR, 12.25; 95% CI, 1.99-75.26), granulomatous MF (HR, 14.59; 95% CI, 1.31-162.00), a history of organ transplant (HR, 10.15; 95% CI, 0.98-105.37), and stage 2 disease at the time of diagnosis (HR, 10.22; 95% CI, 2.94-35.50) were associated with worse outcomes.

Conclusions and Relevance  The findings of this review suggest that there is often a significant delay until the establishment of a correct diagnosis of childhood MF, which may be detrimental to the prognosis.

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    1 Comment for this article
    EXPAND ALL
    Loud Thinking:
    Arvind Joshi, MBBS MD FCGP FAMS FICP | Our Own Discussion Group Mumbai PIN 400028 and Ruchi Clinical Diagnostic Center and Ruchi Clinical Laboratory Kharghar PIN 410210 Maharashtra State INDIA.
    I kept wondering to myself if, inspite of biopsies and repeated reviews by reputed Dermatologists, it is possible to mistake Pauci-bacillary Leprosy for Mycosis Fungicides(MF) after my only encounter with this peculiar case:
    A young person was diagnosed as having MF after biopsy reviewed by a highly reputed Dermatologist, and was given topical treatment but no avail. The Parents showed the condition to me. Given that Leprosy was endemic around the region, I thought of it as Pauci-bacillary Leprosy and treated accordingly with standard anti-Leprosy Agents. In several weeks the lesion had faded substantially, however the Parents were uncomfortable with
    the thoughts of Leprosy, hence got a Panel of Dermatologists to review the case. With another biopsy they said that Pauci-bacillary Leprosy cannot be ruled out but they thought that it was MF; and since the person has already completed 3 months on Dapsone, Rifampicin, Levo-flxacin and Minocyclin these drugs may be very well stopped. Thus the anti-Leprosy Agents were stopped and UV irradiation was given. The lesion remained just the same, very faintly visible but he developed deep tanning around the lesion. The concerned Dermatologists said that the tanning will subside in course of time. The tanning did subside but by then th skin lesion became prominent and increased in area. In meanwhile parents, Googled more information about MF. They became apprehensive about possibility of conversion to Lymphoma. So they brought the person again to me. I reinstituted the initial anti-Leprosy Agents. Over several more months the lesion more or less blended with the surrounding skin, leaving only the marks of biopsies visible. Even after a couple of years there is no recurrence.
    CONFLICT OF INTEREST: None Reported
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