Ann Dermatol.  2018 Apr;30(2):192-201. 10.5021/ad.2018.30.2.192.

Folliculotropic Mycosis Fungoides in 20 Korean Cases: Clinical and Histopathologic Features and Response to Ultraviolet A-1 and/or Photodynamic Therapy

• Jang MS
• Jang JY
• Park JB
• Kang DY
• Lee JW
• Lee TG
• Hwangbo H
• Suh KS

• KMID: 2414681
• DOI: http://doi.org/10.5021/ad.2018.30.2.192

Abstract

BACKGROUND
Folliculotropic mycosis fungoides (FMF) is a variant of mycosis fungoides (MF) that is characterized clinically by variable types of skin eruptions, including plaques, acneiform lesions, and alopecic patches. Histopathologically, FMF is characterized by folliculotropic infiltrates.
OBJECTIVE
This study was conducted to scrutinize the clinical and histopathologic features of FMF in Koreans and the responses to phototherapy.
METHODS
Twenty Koreans diagnosed with MF who had histopathologic evidence of folliculotropism were enrolled.
RESULTS
Eighteen patients had head-and-neck-region infiltration, while five had solitary lesion. In all patients, the atypical lymphocytic infiltrate had a perifollicular distribution. Twelve patients were treated with ultraviolet A (UVA)-1. Eleven of these 12 patients with early-stage FMF experienced >80% improvement (8: complete remission; 3: partial remission). Four patients, including 2 who relapsed after UVA-1, were treated with photodynamic therapy (PDT), reaching complete remission after PDT.
CONCLUSION
As FMF has variable clinical presentations, skin biopsy is required to confirm the diagnosis. And both UVA-1 and methyl aminolevulinate-PDT are clinically effective in treatment of early-stage FMF.

Keyword

Folliculotropic mycosis fungoides; Mycosis fungoides variants; Photodynamic therapy; Phototherapy; Ultraviolet A -1

MeSH Terms

Biopsy
Diagnosis
Humans
Mycosis Fungoides*
Photochemotherapy*
Phototherapy
Skin

Figure

• 

  Fig. 1 (A) Localized erythematous discrete plaques and multiple milia-like lesions on the face. (B) Biopsy specimens show a perifollicular cell infiltrate (H&E, ×40). (C) Small and medium to large pleomorphic lymphocytes with large cell transformation (arrows) are seen (H&E, ×200) (patient #13).

• 

  Fig. 2 (A) Papuloerythroderma with a typical sparing of the abdominal skin folds (‘deck-chair’ sign) and plaques with follicular accentuation. (B) Biopsy specimens show perifollicular infiltrate and coarse collagen bundles in the papillary dermis (H&E, ×40). (C) Folliculotropic lymphocytes and eosinophilic folliculitis are seen (H&E, ×200) (patient #8).

• 

  Fig. 3 (A) Agminated lesion of erythematous discrete papules on the left chest. (B) Close-up view. (C) After 4 times of methyl aminolevulinate-photodynamic therapy treatments, the skin lesions disappeared almost completely. (D) Close-up view. (E) Biopsy specimens show a large dilated follicular unit distended by keratinous material and infiltrated by lymphocytes (H&E, ×40) (patient #16).

• 

  Fig. 4 (A) The facial skin with development of leonine face and scaly patches with erythroderma. (B) Biopsy specimens show a dense band-like infiltrate of lymphocytes in the upper dermis and stuffed dermal papilla with lymphocytes (H&E, ×40). (C) Follicular and perifollicular infiltrates of lymphocytes with follicular mucin are seen (H&E, ×100) (patient #9).

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