Bontoux Christophe, Badrignans Marine, Afach Sivem, Sbidian Emilie, Mboumba Diana-Laure, Ingen-Housz-Oro Saskia, Claudel Alexis, Aubriot-Lorton Marie-Hélène, Chong-Si-Tsaon Arnaud, Le Masson Gilles, Attencourt Christophe, Dubois Romain, Beltzung Fanny, Koubaa Wafa, Beltraminelli Helmut, Cardot-Leccia Nathalie, Balme Brigitte, Nguyen Anh Tuan, Bagny Kelly, Legoupil Delphine, Moustaghfir Ibtissam, Denamps Juliette, Mortier Laurent, Hammami-Ghorbel Houda, Skrek Sergey, Rafaa Mostefa, Fougerousse Anne-Claire, Deschamps Thibaut, Dalle Stéphane, D'incan Michel, Chaby Guillaume, Beylot-Barry Marie, Dalac Sophie, Ortonne Nicolas
Department of Pathology, Cancer University Institute of Toulouse-Oncopole, Toulouse University Hospital, Toulouse, France.
OncoSarc, INSERM U1037, Cancer Research Center in Toulouse, Toulouse, France.
Br J Dermatol. 2024 Dec 23;192(1):125-134. doi: 10.1093/bjd/ljae312.
Mycosis fungoides (MF) usually has an indolent course. However, some patients develop more aggressive disease and few prognostic parameters have been identified. Isolated cases of pustular MF (pMF) suggest an unfavourable prognosis.
To describe the clinicopathological characteristics and prognostic value of pMF.
We retrospectively collected data from all patients with MF with histological pustules diagnosed from 2009 to 2020. The outcomes and clinicopathological characteristics of pMF at diagnosis (pMFD) were compared with those of a cohort of patients with nonpustular MF (NpMF).
Thirty-three patients with pMF (including 22 with pMFD) and 86 with NpMF were included. Median age at diagnosis of pMF was 61 years [interquartile range (IQR) 50-75]. The median duration of follow-up for patients with pMFD was 32 months (IQR 14-49). Clinically, 33% of patients with pMF had pustules. Large cell transformation (LCT) occurred in 17 patients. Patients with pMFD had significantly more advanced-stage disease and showed more LCT at diagnosis than those with NpMF [50% vs. 7% (P < 0.001) and 23% vs. 0% (P < 0.001), respectively]. On multivariate Cox analysis, the presence of histological pustules at diagnosis was associated with shorter overall survival (OS) in all patients [hazard ratio (HR) 13.90, 95% confidence interval (CI) 2.40-79.00); P = 0.003] and in patients with early-stage disease (HR 11.09, 95% CI 1.56-78.82; P = 0.02). In multivariate Fine and Gray model analysis, pMFD was associated with a higher cumulative incidence of LCT (subdistribution HR 13.90, 95% CI 2.43-79.00; P = 0.003) in all patients. Median OS after the occurrence of histological pustules during follow-up in all patients with pMF was 37 months, with a 5-year OS rate of 25% (95% CI 0.06-0.50).
pMF often follows an aggressive course, with a high risk of LCT and shorter survival, even for patients with early-stage disease. Histological pustules at diagnosis of MF might represent an independent poor prognostic factor, to be confirmed by further studies. As pustules are not always identified clinically, pustules found on histology should be mentioned in MF pathology reports and should prompt discussion of closer follow-up.
蕈样肉芽肿（MF）通常病程进展缓慢。然而，一些患者会发展为侵袭性更强的疾病，且目前已确定的预后参数较少。孤立性脓疱型MF（pMF）病例提示预后不良。
描述pMF的临床病理特征及预后价值。
我们回顾性收集了2009年至2020年所有经组织学诊断为有脓疱的MF患者的数据。将pMF诊断时（pMFD）的结局和临床病理特征与一组非脓疱型MF（NpMF）患者进行比较。
纳入了33例pMF患者（包括22例pMFD患者）和86例NpMF患者。pMF诊断时的中位年龄为61岁[四分位间距（IQR）50 - 75]。pMFD患者的中位随访时间为32个月（IQR 14 - 49）。临床上，33%的pMF患者有脓疱。17例患者发生了大细胞转化（LCT）。pMFD患者在诊断时疾病分期明显更晚，且LCT发生率高于NpMF患者[分别为50%对7%（P < 0.001）和23%对0%（P < 0.001）]。多因素Cox分析显示，诊断时存在组织学脓疱与所有患者的总生存期（OS）缩短相关[风险比（HR）13.90，95%置信区间（CI）2.40 - 79.00；P = 0.003]，在早期疾病患者中也是如此（HR 11.09，95% CI 1.56 - 78.82；P = 0.02）。在多因素Fine和Gray模型分析中，pMFD与所有患者LCT的更高累积发生率相关（亚分布HR 13.90，95% CI 2.43 - 79.00；P = 0.003）。所有pMF患者在随访期间出现组织学脓疱后的中位OS为37个月，5年OS率为25%（95% CI 0.06 - 0.50）。
pMF通常病程侵袭性强，LCT风险高且生存期较短，即使是早期疾病患者也是如此。MF诊断时的组织学脓疱可能是一个独立的不良预后因素，有待进一步研究证实。由于脓疱并非总能在临床上被识别，MF病理报告中应提及组织学上发现的脓疱，并应促使讨论更密切的随访。
