Papular Mycosis Fungoides Is a Distinctive Variant of Early-stage Mycosis Fungoides
Papular Mycosis Fungoides Is a Distinctive Variant of Early-stage Mycosis Fungoides
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丘疹性蕈样肉芽肿是早期蕈样肉芽肿的一种独特变体
DOI：
10.1097/pas.0000000000001256
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发表时间：
2019
期刊：
影响因子：
--
通讯作者：
L. Cerroni
中科院分区：
文献类型：
--
作者：
A. Saggini;R. Fink‐Puches;C. Cota;V. Lora;H. Potzinger;C. Massone;L. Cerroni
丘疹样肉芽肿(PMF)是真菌样肉芽肿(MF)的一种罕见变种。PMF的确切发病机制和预后尚不清楚。我们从奥地利格拉茨医科大学皮肤科的档案中回顾了PMF的病例，并检查了随访数据。患者包括13名男性和5名女性(中位数年龄：57.5 y；范围13至77 y)。在4例患者中，最初的临床病理诊断为不典型的苔藓样糠疹；这些病例随后由于在随访中出现传统的MF斑块而被重新分类为PMF。我们病例的随访数据显示，2名患者分别在首次出现症状后50个月和199个月死于疾病进展。两名患者分别在215个月和300个月后仍有进展性疾病。10名患者生活在稳定型疾病中(中位数：70 mo)。4例患者在最后一次随访时完全缓解(中位数：215 mo；其中2例死于无关原因)。我们的数据证实，PMF是早期MF的一种临床病理变体，其预后类似于该疾病的常规表现。熟悉PMF，并与其他皮肤丘疹淋巴组织增生症区分开来，对于这些患者的准确诊断和治疗是必要的。
Papular mycosis fungoides (PMF) is a rare variant of mycosis fungoides (MF). The exact nosology and prognosis of PMF are still unclear. We retrospectively identified cases of PMF from the files of the Department of Dermatology of the Medical University of Graz, Austria, and checked the follow-up data. The patients comprised 13 men and 5 women (median age: 57.5 y; range 13 to 77 y). In 4 patients, an initial clinicopathologic diagnosis of atypical pityriasis lichenoides was made; these cases were subsequently reclassified as PMF due to the onset of conventional patches of MF during follow-up. Follow-up data of our cases showed that 2 patients died of disease progression 50 and 199 months after the first presentation, respectively. Two patients are alive with progressive disease after 215 and 300 months, respectively. Ten patients are alive with stable disease (median: 70 mo). Four patients were in complete remission at last follow-up visit (median: 215 mo; 2 of them died of unrelated causes). Our data confirm that PMF represents a clinicopathologic variant of early MF with prognosis similar to conventional presentations of the disease. Familiarity with PMF and distinction from other cutaneous papular lymphoid proliferations is necessary for a precise diagnosis and management of these patients.