VARIANT_P: a 14-years multicentric follow-up study of atypical pemphigus variants in Italy.

IF 2.8 4区医学 Q1 DERMATOLOGY

Clinical and Experimental Dermatology Pub Date : 2025-10-11 DOI:10.1093/ced/llaf438

Lavinia Quintarelli, Irene Bonanni, Elena Biancamaria Mariotti, Laura Atzori, Clara De Simone, Camilla Vassallo, Giovanni Di Zenzo, Stefano Caccavale, Emanuele Cozzani, Gianpiero Girolomoni, Angelo Valerio Marzano, Andrea Conti, Pamela Vezzoli, Giovanni Damiani, Vito Di Lernia, Riccardo Balestri, Roberto Maglie, Alberto Corrà, Alessandro Magnatta, Marta Elettra Donati, Valentina Ruffo di Calabria, Alice Verdelli, Alessio Coi, Emiliano Antiga, Marzia Caproni

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引用次数: 0

Abstract

Background: The clinical, epidemiological, and immunopathological profile of atypical forms of pemphigus, remains only partially known.

Objectives: To define the clinical, epidemiological and immunological characteristics, therapies and outcome of atypical pemphigus variants.

Methods: A 14-year multicenter retrospective observational study (VARIANT_P) on atypical variants of pemphigus across Italy. We collect demographic, immunopathological, and clinical data, as well as information on comorbidities and prescribed treatments.

Results: We enrolled 61 patients (Sex Ratio=1.77; 13 PNP, 26 PIgA, 22 PH). Median age at diagnosis and onset were 71.1 and 70.6 (PNP), 63.6 and 62.2 (PigA), 52.3 and 49.4 (PH).The average diagnostic delay was 3.0 (PNP), 9.5 (PIgA) and 2.0 months (PH). Mortality rate was 54.5% (PNP), 3.8% (PIgA) and 5.9% (PH). Cutaneous involvement was present in all cases of PIgA and PH, and in 83.3% of PNP cases. In contrast, oral mucosal involvement was observed in all PNP cases, but only in 7.7% of PigA and 21.1% of PH cases. Histology, DIF, IIF and ELISA data were variably consistent with the literature.Comorbidities included mainly solid malignancies for PNP while cardiovascular and metabolic disease were the most prevalent for PIgA and PH. Treatment mostly relied on systemic steroids and rituximab.

Conclusions: The VARIANT_P study contributed to data collection relatively to atypical pemphigus variants in order to promote the development of specific therapeutical guidelines in the future.

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VARIANT_P：一项意大利非典型天疱疮变体的14年多中心随访研究。

背景：临床，流行病学和非典型形式的天疱疮的免疫病理概况，仍然只是部分了解。目的：探讨非典型天疱疮的临床、流行病学、免疫学特征、治疗方法及转归。方法：对意大利各地天疱疮的非典型变异进行为期14年的多中心回顾性观察研究（VARIANT_P）。我们收集人口统计学、免疫病理学和临床数据，以及合并症和处方治疗的信息。结果：我们纳入了61例患者（性别比=1.77；PNP 13例，PIgA 26例，PH 22例）。诊断和发病时的中位年龄分别为71.1和70.6 （PNP）、63.6和62.2 （PigA）、52.3和49.4 （PH）。平均诊断延迟为3.0个月（PNP）， 9.5个月（PIgA）和2.0个月（PH）。死亡率分别为54.5% （PNP）、3.8% （PIgA）和5.9% （PH）。所有PIgA和PH病例以及83.3%的PNP病例均存在皮肤受累。相比之下，在所有PNP病例中均观察到口腔黏膜受累，但仅在7.7%的PigA和21.1%的PH病例中观察到。组织学、DIF、IIF和ELISA数据与文献一致。合并症主要包括PNP的实体恶性肿瘤，而PIgA和ph最常见的是心血管和代谢性疾病。治疗主要依赖全身性类固醇和利妥昔单抗。结论：VARIANT_P研究有助于收集非典型天疱疮变异的相关数据，以促进未来特定治疗指南的制定。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical and Experimental Dermatology 医学-皮肤病学

CiteScore

3.20

自引率

2.40%

发文量

389

审稿时长

3-8 weeks

期刊介绍： Clinical and Experimental Dermatology (CED) is a unique provider of relevant and educational material for practising clinicians and dermatological researchers. We support continuing professional development (CPD) of dermatology specialists to advance the understanding, management and treatment of skin disease in order to improve patient outcomes.