Department of Dermatology, Research Unit Dermatopathology, Medical University of Graz, Graz, Austria.
Department of Biomedicine and Prevention, Division of Anatomic Pathology, University of Rome Tor Vergata.
Am J Surg Pathol. 2019 Aug;43(8):1129-1134. doi: 10.1097/PAS.0000000000001256.
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.
蕈样肉芽肿（MF）的丘疹型变体（PMF）是一种罕见的 MF 变体。PMF 的确切分类和预后尚不清楚。我们回顾性地从奥地利格拉茨医科大学皮肤科的文件中确定了 PMF 病例，并检查了随访数据。这些患者包括 13 名男性和 5 名女性（中位年龄：57.5 岁；范围 13 至 77 岁）。4 例患者最初的临床病理诊断为非典型苔藓样糠疹；由于在随访过程中出现常规 MF 斑块，这些病例随后被重新分类为 PMF。我们病例的随访数据显示，2 名患者分别在首次就诊后 50 和 199 个月因疾病进展而死亡。2 名患者分别在 215 和 300 个月后疾病仍在进展且存活。10 名患者病情稳定（中位：70 个月）。最后一次随访时，4 名患者处于完全缓解（中位：215 个月；其中 2 名死于无关原因）。我们的数据证实，PMF 代表 MF 的早期临床病理变异，其预后与疾病的常规表现相似。熟悉 PMF 并与其他皮肤丘疹性淋巴样增生区分开来，对于这些患者的准确诊断和管理是必要的。
