Christophe Bontoux, Marine Badrignans, Sivem Afach, Emilie Sbidian, Diana-Laure Mboumba, Saskia Ingen-Housz-Oro, Alexis Claudel, Marie-Hélène Aubriot-Lorton, Arnaud Chong-Si-Tsaon, Gilles Le Masson, Christophe Attencourt, Romain Dubois, Fanny Beltzung, Wafa Koubaa, Helmut Beltraminelli, Nathalie Cardot-Leccia, Brigitte Balme, Anh Tuan Nguyen, Kelly Bagny, Delphine Legoupil, Ibtissam Moustaghfir, Juliette Denamps, Laurent Mortier, Houda Hammami-Ghorbel, Sergey Skrek, Mostefa Rafaa, Anne-Claire Fougerousse, Thibaut Deschamps, Stéphane Dalle, Michel D'incan, Guillaume Chaby, Marie Beylot-Barry, Sophie Dalac, Nicolas Ortonne
{"title":"脓疱型真菌病预后不佳：一项多中心临床病理和分子病例系列研究。","authors":"Christophe Bontoux, Marine Badrignans, Sivem Afach, Emilie Sbidian, Diana-Laure Mboumba, Saskia Ingen-Housz-Oro, Alexis Claudel, Marie-Hélène Aubriot-Lorton, Arnaud Chong-Si-Tsaon, Gilles Le Masson, Christophe Attencourt, Romain Dubois, Fanny Beltzung, Wafa Koubaa, Helmut Beltraminelli, Nathalie Cardot-Leccia, Brigitte Balme, Anh Tuan Nguyen, Kelly Bagny, Delphine Legoupil, Ibtissam Moustaghfir, Juliette Denamps, Laurent Mortier, Houda Hammami-Ghorbel, Sergey Skrek, Mostefa Rafaa, Anne-Claire Fougerousse, Thibaut Deschamps, Stéphane Dalle, Michel D'incan, Guillaume Chaby, Marie Beylot-Barry, Sophie Dalac, Nicolas Ortonne","doi":"10.1093/bjd/ljae312","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Mycosis fungoides (MF) has usually an indolent course. However, some patients develop a more aggressive disease and few prognostic parameters have been identified. Isolated cases of pustular MF (pMF) suggest an unfavourable prognosis.</p><p><strong>Objectives: </strong>We aim to describe the clinico-pathological characteristics and prognostic value of pMF.</p><p><strong>Methods: </strong>We retrospectively collected data of all cases of MF with histological pustules diagnosed from 2009 to 2020. The outcomes and clinico-pathological characteristics of pMF at diagnosis (pMFD) were compared to those of a cohort of non-pustular MF (NpMF).</p><p><strong>Results: </strong>33 pMF (including 22 pMFD) and 86 NpMF cases were included. The median age at diagnosis of pMF was 61 years [IQR=50-75]. The median follow-up of pMFD was 32 months [IQR=14-49]. Clinically, 33% of pMF had pustules. Large-cell transformation (LCT) occurred in 17 cases. pMFD were at a significantly more advanced-stage and more showed LCT at diagnosis than NpMF (50% vs 7%, p<0.001 and 23% vs 0%, p<0.001, respectively). In multivariate Cox analysis, the presence of histological pustule at diagnostic was associated with shorter OS in all patients (HR=13.90, CI95%[2.43-79]; p=0.003), and in early-stage patients (HR=11.09, CI95%[1.56-78.82]; p=0.02). In multivariate Fine and Gray model analysis, pMFD was associated with a higher cumulative incidence of LCT (SHR=13.90, CI95% [2.43-79]; p=0.003) in all patients. Median OS after the occurrence of histological pustules during follow-up of all pMF patients was 37 months, with a five-year OS of 25% (CI95% [0.06-0.5]).</p><p><strong>Conclusion: </strong>pMF often follows an aggressive course, with a high risk of LCT and shorter survival, even for early-stage patients. Histological pustules at diagnostic of MF might represent an independent poor prognostic factor, to be confirmed by further studies. Because pustules are not always clinically identified, histological pustules should be mentioned in pathology reports of MF and prompt discussion of a closer follow-up.</p>","PeriodicalId":9238,"journal":{"name":"British Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":11.0000,"publicationDate":"2024-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Pustular mycosis fungoides has a poor outcome: a multicentric clinico-pathological and molecular case series study.\",\"authors\":\"Christophe Bontoux, Marine Badrignans, Sivem Afach, Emilie Sbidian, Diana-Laure Mboumba, Saskia Ingen-Housz-Oro, Alexis Claudel, Marie-Hélène Aubriot-Lorton, Arnaud Chong-Si-Tsaon, Gilles Le Masson, Christophe Attencourt, Romain Dubois, Fanny Beltzung, Wafa Koubaa, Helmut Beltraminelli, Nathalie Cardot-Leccia, Brigitte Balme, Anh Tuan Nguyen, Kelly Bagny, Delphine Legoupil, Ibtissam Moustaghfir, Juliette Denamps, Laurent Mortier, Houda Hammami-Ghorbel, Sergey Skrek, Mostefa Rafaa, Anne-Claire Fougerousse, Thibaut Deschamps, Stéphane Dalle, Michel D'incan, Guillaume Chaby, Marie Beylot-Barry, Sophie Dalac, Nicolas Ortonne\",\"doi\":\"10.1093/bjd/ljae312\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Mycosis fungoides (MF) has usually an indolent course. However, some patients develop a more aggressive disease and few prognostic parameters have been identified. Isolated cases of pustular MF (pMF) suggest an unfavourable prognosis.</p><p><strong>Objectives: </strong>We aim to describe the clinico-pathological characteristics and prognostic value of pMF.</p><p><strong>Methods: </strong>We retrospectively collected data of all cases of MF with histological pustules diagnosed from 2009 to 2020. The outcomes and clinico-pathological characteristics of pMF at diagnosis (pMFD) were compared to those of a cohort of non-pustular MF (NpMF).</p><p><strong>Results: </strong>33 pMF (including 22 pMFD) and 86 NpMF cases were included. The median age at diagnosis of pMF was 61 years [IQR=50-75]. The median follow-up of pMFD was 32 months [IQR=14-49]. Clinically, 33% of pMF had pustules. Large-cell transformation (LCT) occurred in 17 cases. pMFD were at a significantly more advanced-stage and more showed LCT at diagnosis than NpMF (50% vs 7%, p<0.001 and 23% vs 0%, p<0.001, respectively). In multivariate Cox analysis, the presence of histological pustule at diagnostic was associated with shorter OS in all patients (HR=13.90, CI95%[2.43-79]; p=0.003), and in early-stage patients (HR=11.09, CI95%[1.56-78.82]; p=0.02). In multivariate Fine and Gray model analysis, pMFD was associated with a higher cumulative incidence of LCT (SHR=13.90, CI95% [2.43-79]; p=0.003) in all patients. Median OS after the occurrence of histological pustules during follow-up of all pMF patients was 37 months, with a five-year OS of 25% (CI95% [0.06-0.5]).</p><p><strong>Conclusion: </strong>pMF often follows an aggressive course, with a high risk of LCT and shorter survival, even for early-stage patients. Histological pustules at diagnostic of MF might represent an independent poor prognostic factor, to be confirmed by further studies. Because pustules are not always clinically identified, histological pustules should be mentioned in pathology reports of MF and prompt discussion of a closer follow-up.</p>\",\"PeriodicalId\":9238,\"journal\":{\"name\":\"British Journal of Dermatology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":11.0000,\"publicationDate\":\"2024-08-12\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"British Journal of Dermatology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1093/bjd/ljae312\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"DERMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"British Journal of Dermatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1093/bjd/ljae312","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"DERMATOLOGY","Score":null,"Total":0}
Pustular mycosis fungoides has a poor outcome: a multicentric clinico-pathological and molecular case series study.
Background: Mycosis fungoides (MF) has usually an indolent course. However, some patients develop a more aggressive disease and few prognostic parameters have been identified. Isolated cases of pustular MF (pMF) suggest an unfavourable prognosis.
Objectives: We aim to describe the clinico-pathological characteristics and prognostic value of pMF.
Methods: We retrospectively collected data of all cases of MF with histological pustules diagnosed from 2009 to 2020. The outcomes and clinico-pathological characteristics of pMF at diagnosis (pMFD) were compared to those of a cohort of non-pustular MF (NpMF).
Results: 33 pMF (including 22 pMFD) and 86 NpMF cases were included. The median age at diagnosis of pMF was 61 years [IQR=50-75]. The median follow-up of pMFD was 32 months [IQR=14-49]. Clinically, 33% of pMF had pustules. Large-cell transformation (LCT) occurred in 17 cases. pMFD were at a significantly more advanced-stage and more showed LCT at diagnosis than NpMF (50% vs 7%, p<0.001 and 23% vs 0%, p<0.001, respectively). In multivariate Cox analysis, the presence of histological pustule at diagnostic was associated with shorter OS in all patients (HR=13.90, CI95%[2.43-79]; p=0.003), and in early-stage patients (HR=11.09, CI95%[1.56-78.82]; p=0.02). In multivariate Fine and Gray model analysis, pMFD was associated with a higher cumulative incidence of LCT (SHR=13.90, CI95% [2.43-79]; p=0.003) in all patients. Median OS after the occurrence of histological pustules during follow-up of all pMF patients was 37 months, with a five-year OS of 25% (CI95% [0.06-0.5]).
Conclusion: pMF often follows an aggressive course, with a high risk of LCT and shorter survival, even for early-stage patients. Histological pustules at diagnostic of MF might represent an independent poor prognostic factor, to be confirmed by further studies. Because pustules are not always clinically identified, histological pustules should be mentioned in pathology reports of MF and prompt discussion of a closer follow-up.
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The British Journal of Dermatology (BJD) is committed to publishing the highest quality dermatological research. Through its publications, the journal seeks to advance the understanding, management, and treatment of skin diseases, ultimately aiming to improve patient outcomes.
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