Follicular mycosis fungoides.
We describe a patient with follicular mycosis fungoides (MF), a rare folliculotropic variant of cutaneous T-cell lymphoma (CTCL). Follicular involvement in CTCL usually presents clinically as alopecia mucinosa associated histologically with follicular mucinosis. Follicular MF differs from alopecia mucinosa/follicular mucinosis associated with MF with regard to its clinical presentation, histology and, presumably, prognosis. Our patient presented with the characteristic findings of follicular MF, i.e. infiltrated plaques showing numerous enlarged, comedo-like follicular infundibula; histology was dominated by exclusive folliculotropism of atypical lymphocytes sometimes forming follicular Pautrier's microabscesses, and by lack of epidermotropism and follicular mucinosis. Despite photochemotherapy and treatment with oral retinoids and interferon alpha, the patient's follicular MF rapidly developed into a progressive CTCL with large tumorous lesions, but responded to electron beam therapy. The course of our patient's disease confirms the notion that follicular MF may be associated with a worse prognosis than classical MF. However, electron beam irradiation induced remission of follicular MF that was maintained by a combination therapy consisting of extracorporeal photopheresis and interferon alfa.
Klemke
C D
CD
Department of Dermatology, University Medical Center Benjamin Franklin, The Free University of Berlin, Hindenburgdamm 30, D-12200 Berlin, Germany.
Dippel
E
E
Assaf
C
C
Hummel
M
M
Stein
H
H
Goerdt
S
S
Orfanos
C E
CE
D000368:Aged; D003131:Combined Modality Therapy; D018450:Disease Progression; D006201:Hair Diseases / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy; D018859:Hair Follicle / Q000473:pathology*; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use; D016410:Lymphoma, T-Cell, Cutaneous / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D008297:Male; D009182:Mycosis Fungoides / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy; D017893:Photopheresis; D012878:Skin Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy
D016898:Interferon-alpha