Clinicopathological Profile of Pyoderma Gangrenosum: A 10-Year Retrospective Study from a Tertiary Care Center in South India.

IF 1.9 Q3 DERMATOLOGY Indian Dermatology Online Journal Pub Date : 2024-08-30 eCollection Date: 2024-09-01 DOI:10.4103/idoj.idoj_843_23
Anju George, Dharshini Sathishkumar, Lydia Mathew, Ankan Gupta, Minu Jose Chiramel, Vartika Singh, Meera Thomas
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Abstract

Background: Pyoderma gangrenosum (PG) is a reactive neutrophilic inflammatory dermatosis with a varied clinicopathologic presentation. It commonly manifests as rapidly progressive painful ulcers, mimicking varied conditions including infections, vasculitis, and malignancies, and is a diagnosis of exclusion. There are scarce data on PG from the Indian subcontinent.

Aim and objectives: The aim of the study was to study the clinicopathologic profile of patients with PG and their underlying systemic associations.

Materials and methods: A retrospective observational study was done between 2011 and 2021, and patients diagnosed as PG based on the diagnostic tool proposed by Maverakis et al. were recruited and their demographic, clinical, and histological findings were obtained.

Results: Among 54 patients with suspected PG, 17 patients (eight males and nine females) fulfilled the diagnostic criteria, and the mean age of disease onset was 32.1 years (range: 3-60 years). Ulcerative variant was the most common type (9/17, 52.9%), and 29.4% had systemic associations including autoinflammatory syndromes. The onset at atypical sites such as face and hand were noted in one patient each. Histopathology revealed a polymorphous dermal infiltrate with neutrophilic predominance in the majority (94.1%). Systemic steroids (dose ranging from 0.5-1 mg/kg prednisolone equivalent) were used in 11/17 (64.7%) patients. The commonly used alternative drugs included clofazimine (47%), minocycline (29%), thalidomide (23.5%), adalimumab and mycophenolate mofetil in 17.6% each, dapsone and ciclosporine in 11.7% each. Remission was achieved between two weeks and three months in 10 (58.8%) patients after treatment initiation and two mortalities (11.7%) were recorded.

Conclusion: PG can affect any age group and may be localized to rarer, atypical sites. The possibility of underlying autoinflammatory conditions should be considered in addition to the evaluation of other disorders like inflammatory bowel disease, hematological disorders, and rheumatological disorders.

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坏疽性脓皮病的临床病理概况:印度南部一家三级医疗中心的十年回顾性研究
背景:坏疽性脓皮病(PG)是一种反应性嗜中性粒细胞炎症性皮肤病,临床病理表现多种多样。它通常表现为快速进展的疼痛性溃疡,可模拟感染、血管炎和恶性肿瘤等多种疾病,是一种排除性诊断。印度次大陆有关 PG 的数据很少:研究目的:研究 PG 患者的临床病理学特征及其潜在的系统性关联:在2011年至2021年期间进行了一项回顾性观察研究,招募了根据Maverakis等人提出的诊断工具诊断为PG的患者,并获得了他们的人口统计学、临床和组织学结果:在 54 名疑似 PG 患者中,17 名患者(8 男 9 女)符合诊断标准,平均发病年龄为 32.1 岁(范围:3-60 岁)。溃疡变异型是最常见的类型(9/17,52.9%),29.4%与自身炎症综合征等全身性疾病有关。有一名患者的发病部位不典型,如面部和手部。组织病理学显示,大多数患者(94.1%)的真皮呈多形性浸润,以中性粒细胞为主。11/17(64.7%)名患者使用了全身类固醇(剂量为 0.5-1 毫克/千克泼尼松龙当量)。常用的替代药物包括氯法齐明(47%)、米诺环素(29%)、沙利度胺(23.5%)、阿达木单抗和霉酚酸酯(各占17.6%)、达泊松和环孢素(各占11.7%)。10例(58.8%)患者在开始治疗后的两周至三个月内病情得到缓解,有两例(11.7%)患者死亡:结论:PG 可影响任何年龄段的人群,并可能发生在较罕见的非典型部位。除了评估炎症性肠病、血液病和风湿病等其他疾病外,还应考虑潜在自身炎症的可能性。
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CiteScore
2.00
自引率
11.80%
发文量
201
审稿时长
49 weeks
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