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Folliculotropic Mycosis Fungoides

Educational Objective
To identify the key insights or developments described in this article
1 Credit CME

A man in his 60s presented with a 20-year history of progressive pruritic skin lesions on his face and trunk. The lesions first appeared on his back without identifiable cause and spread, reaching the abdomen and eventually the head and face. Skin examination revealed multiple edematous red plaques distributed over the patient’s forehead, eyelids, and chin that were accompanied by alopecia of the right eyebrow (Figure, A). These plaques were ill-defined, ranging from 3 cm to 6 cm in diameter. Simultaneously, we could identify generalized follicular red papules on his abdomen, back, and both sides of the chest (Figure, B). The papules were 2 mm in size and partially fused into red patches. Punch biopsies were performed from the chin and back, the results revealing dense and deep infiltration of lymphocytes in and around hair follicles. Immunohistochemical staining showed that the infiltrating cells expressed CD3 and CD4, while CD8 and CD56 were negative. His baseline laboratory investigations yielded normal results. His workup results were negative for extracutaneous involvement. The diagnosis of skin-limited folliculotropic mycosis fungoides (FMF) was established. The skin lesions achieved partial remission following treatment with interferon α (IFNa) injection and UV-B phototherapy.

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Article Information

Corresponding Author: Yu Zhang, MD, PhD, Department of Dermatology, Tianjin Academy of Traditional Chinese Medicine Affiliated Hospital, 354 Beima Rd, Hongqiao District, Tianjin, China (niuniuzy7375@aliyun.com).

Published Online: February 2, 2022. doi:10.1001/jamadermatol.2021.5181

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this article.

References
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Willemze  R , Cerroni  L , Kempf  W ,  et al.  The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas.   Blood. 2019;134(13):1112.PubMedGoogle Scholar
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Mitteldorf  C , Stadler  R , Sander  CA , Kempf  W .  Folliculotropic mycosis fungoides.   J Dtsch Dermatol Ges. 2018;16(5):543-557.PubMedGoogle ScholarCrossref
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van Santen  S , Roach  RE , van Doorn  R ,  et al.  Clinical staging and prognostic factors in folliculotropic mycosis fungoides.   JAMA Dermatol. 2016;152(9):992-1000.PubMedGoogle ScholarCrossref
4.
van Santen  S , van Doorn  R , Neelis  KJ ,  et al.  Recommendations for treatment in folliculotropic mycosis fungoides: report of the Dutch Cutaneous Lymphoma Group.   Br J Dermatol. 2017;177(1):223-228.PubMedGoogle ScholarCrossref
AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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