Folliculotropic mycosis fungoides - An aggressive variant of cutaneous T-cell lymphoma
Folliculotropic mycosis fungoides - An aggressive variant of cutaneous T-cell lymphoma
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DOI：
10.1001/archderm.144.6.738
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发表时间：
2008-06-01
影响因子：
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通讯作者：
Guitart, Joan
中科院分区：
文献类型：
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作者：
Gerami, Pedram;Rosen, Steve;Guitart, Joan
目的：研究促毛囊性蕈样肉芽肿 (FMF) 患者的临床特征、治疗反应和结果，并将我们对 43 名患者的单中心经验与荷兰皮肤淋巴瘤小组的研究结果进行比较。背景：西北大学多学科皮肤淋巴瘤小组的单中心经验。患者：43 名 FMF 患者被纳入研究，并与 43 名年龄和阶段匹配的经典患者进行比较亲表皮性蕈样肉芽肿（MF）的随访时间相似。结果：亲毛囊性蕈样肉芽肿具有明显的临床特征，其中37例患者出现面部受累（86%），仅6例患者病变仅限于躯干（14%）。皮损的形态范围广泛，包括红斑丘疹和毛囊突出斑块，伴或不伴脱发；粉刺、痤疮样和囊性病变；有或没有疤痕的脱发斑块；以及结节性和痒疹样病变。 65% 的患者患有脱发，其中 71% 的病例涉及面部。 68%的患者出现严重瘙痒。一般来说，患者对皮肤定向治疗反应不佳，几乎所有情况下都需要全身药物才能诱导部分缓解，包括早期疾病患者。总体生存率很差。早期疾病 (= IIB) 患者的结果与具有相似阶段的传统亲表皮性 MF 的对照组患者相似。结论：FMF 临床表现的形态学范围广泛，并且与传统 MF 不同。这至少部分归因于 FMF 能够模拟影响毛囊单位的各种炎症状况。该病程具有侵袭性，许多患者（包括早期疾病患者）的预后较差，尤其是在初次发病后 10 至 15 年内。即使在疾病早期，对皮肤定向治疗的反应也很差，我们的最佳结果是采用口服贝沙罗汀的补骨脂素加 UV-A (PUVA) 治疗或 PUVA 与干扰素 α 治疗。这些发现证实了荷兰皮肤淋巴瘤组的发现，并进一步验证了 FMF 作为一个独特实体的分类。
Objectives: To study the clinical features, therapeutic responses, and outcomes in patients with folliculotropic mycosis fungoides (FMF) and to compare our single-center experience of 43 patients with the findings from the Dutch Cutaneous Lymphoma Group.Setting: A single-center experience from the Northwestern University Multidisciplinary Cutaneous Lymphoma Group.Patients: Forty-three patients with FMF were included in the study and compared with 43 age- and stage-matched patients with classic epidermotropic mycosis fungoides (MF) with similar follow-up time.Results: Folliculotropic mycosis fungoides showed distinct clinical features, with 37 patients having facial involvement (86%) and only 6 having lesions limited to the torso (14%). The morphologic spectrum of lesions is broad and includes erythematous papules and plaques with follicular prominence with or without alopecia; comedonal, acneiform, and cystic lesions; alopecic patches with or without scarring; and nodular and prurigolike lesions. Sixty-five percent of patients had alopecia, which in 71% of cases involved the face. Severe pruritus was seen in 68% of patients. In general, patients responded poorly to skin-directed therapy and in almost all cases required systemic agents to induce even a partial remission, including patients with early-stage disease. Overall survival was poor. Patients with early-stage disease (= IIB) had an outcome similar to those patients in the control group with conventional epidermotropic MF of a similar stage.Conclusions: The morphologic spectrum of clinical presentation for FMF is broad and distinct from those in conventional MF. This is at least partially attributed to the ability of FMF to simulate a variety of inflammatory conditions afflicting the follicular unit. The disease course is aggressive, and many patients, including those with early disease, show a poor outcome particularly between 10 and 15 years after the initial onset of disease. Response to skin-directed therapy is poor even in early-stage disease, and our best results were seen with psoralen plus UV-A (PUVA) therapy with oral bexarotene or PUVA with interferon alfa. These findings corroborate those of the Dutch Cutaneous Lymphoma Group and further validate the classification of FMF as a distinct entity.