Klemke C D, Dippel E, Assaf C, Hummel M, Stein H, Goerdt S, Orfanos C E
Department of Dermatology, University Medical Center Benjamin Franklin, The Free University of Berlin, Hindenburgdamm 30, D-12200 Berlin, Germany.
Br J Dermatol. 1999 Jul;141(1):137-40. doi: 10.1046/j.1365-2133.1999.02935.x.
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.
我们描述了一名患有毛囊蕈样肉芽肿（MF）的患者，这是一种罕见的亲毛囊性皮肤T细胞淋巴瘤（CTCL）变异型。CTCL中的毛囊受累在临床上通常表现为黏液性秃发，组织学上与毛囊黏液变性相关。毛囊性MF在临床表现、组织学以及可能的预后方面与MF相关的黏液性秃发/毛囊黏液变性有所不同。我们的患者表现出毛囊性MF的特征性表现，即浸润性斑块，可见大量增大的、类似粉刺样的毛囊漏斗；组织学表现为非典型淋巴细胞仅亲毛囊性浸润，有时形成毛囊内Pautrier微脓肿，且无亲表皮性和毛囊黏液变性。尽管接受了光化学疗法、口服维甲酸和干扰素α治疗，但患者的毛囊性MF迅速发展为具有大肿瘤性病变的进行性CTCL，但对电子束治疗有反应。我们患者的病程证实了毛囊性MF可能比经典MF预后更差的观点。然而，电子束照射诱导毛囊性MF缓解，通过体外光化学疗法和干扰素α联合治疗得以维持。
