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Master Thesis

Profils cliniques évolutifs de la pemphigoïde bulleuse : étude rétrospective monocentrique de 312 cas

Abstract : Background: bullous pemphigoid (BP) is the most frequent autoimmune bullous disease and a set of 4 clinical criteria highly consistent with BP diagnosis has been previously validated. However, clinical spectrum of diseases may change over time and recently a number of unusual clinical features seem to have emerged in some BP patients. Accordingly a retrospective study was designed to reappraise the validity of the aforementioned criteria over time in a large cohort of well-documented BP in this setting of a possibly increasing frequency of atypical features and to describe possible new clinical patterns of BP and their potential time-related occurrence over the last two decades. Patients and methods: well-documented BP patients diagnosed between January 2001 and April 2017 were included in this retrospective analysis. The following data were collected : baseline characteristics of patients, extent of the disease, distribution and type of cutaneous lesions, presence of mucous lesions, presence of at least one atypical clinical (topographical or lesional) feature, presence or absence of at least 3 of the prior validated diagnostic clinical criteria. In order to identify a possible time-related shift of clinical features, data from 4 chronological groups of 4 years (G1 to G4) were statistically compared. Results: 312 patients were included and analyzed. At least 3 of the 4 classical criteria were present in 278 patients (89.1%), but with a trend toward a steady decrease of relevance over time (G1 93.0% vs G4 83.8%, p 0.067). The most frequent missing criteria were absence of mucosal involvement followed by absence of head and neck involvement. 206 patients (66.0%) displayed at least one atypical clinical feature and chronological analysis confirmed a significant time-related increase of atypical pattern frequency (G1 57.9%, G4 73.7%, p 0.041). Head and neck, palmoplantar, mucosae involvement were observed in 56 (17.9%), 151 (48.4%) and 58 (18.6%) patients respectively, with an upward, although non significant trend between G1 and G4 (p 0.071, p 0.088, p 0.094 for these 3 items respectively). Discussion: clinical presentation of BP seems to have evolved over time with a progressively higher proportion of atypical topographical and/or lesional characteristics, mainly regarding mucous membrane, head and neck and palmo-plantar involvement. This shift probably underlies the progressive reduction of the percentage of BP patients fulfilling at least 3 of the 4 BP previously validated diagnostic criteria even though these criteria remain robust in our study. Environnemental causes, mainly drugs might affect clinical presentation of BP since a higher frequency of mucosal lesions has been recently reported in dipeptidyl peptidase 4 inhibitors (DPP4is)-induced BP compared to other BP patients.
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Master Thesis
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Submitted on : Monday, October 5, 2020 - 3:53:31 PM
Last modification on : Friday, August 5, 2022 - 10:57:51 AM


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  • HAL Id : dumas-02958092, version 1



Élise Dandache. Profils cliniques évolutifs de la pemphigoïde bulleuse : étude rétrospective monocentrique de 312 cas. Médecine humaine et pathologie. 2018. ⟨dumas-02958092⟩



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