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坏疽性脓皮病的临床病理特征:来自印度南部一家三级医疗中心的10年回顾性研究。

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

作者信息

George Anju, Sathishkumar Dharshini, Mathew Lydia, Gupta Ankan, Chiramel Minu Jose, Singh Vartika, Thomas Meera

机构信息

Department of Dermatology, Venereology, Leprosy, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.

Department of Pathology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.

出版信息

Indian Dermatol Online J. 2024 Aug 30;15(5):770-778. doi: 10.4103/idoj.idoj_843_23. eCollection 2024 Sep-Oct.

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 . 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.

摘要

背景

坏疽性脓皮病(PG)是一种反应性中性粒细胞性炎性皮肤病,具有多种临床病理表现。它通常表现为迅速进展的疼痛性溃疡,可模仿多种疾病,包括感染、血管炎和恶性肿瘤,是一种排除性诊断。来自印度次大陆的关于PG的数据很少。

目的

本研究的目的是研究PG患者的临床病理特征及其潜在的全身关联。

材料和方法

在2011年至2021年间进行了一项回顾性观察研究,招募了根据Maverakis提出的诊断工具被诊断为PG的患者,并获取了他们的人口统计学、临床和组织学检查结果。

结果

在54例疑似PG的患者中,17例(8例男性和9例女性)符合诊断标准,疾病发病的平均年龄为32.1岁(范围:3 - 60岁)。溃疡型是最常见的类型(9/17,52.9%),29.4%的患者有全身关联,包括自身炎症综合征。分别有1例患者在面部和手部等非典型部位发病。组织病理学显示大多数(94.1%)为多形性真皮浸润,中性粒细胞占优势。11/17(64.7%)的患者使用了全身用类固醇(剂量范围为0.5 - 1mg/kg泼尼松等效剂量)。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%;氨苯砜和环孢素各占11.7%。10例(58.8%)患者在开始治疗后2周内至3个月内实现缓解,记录到2例死亡(11.7%)。

结论

PG可影响任何年龄组,可能局限于较罕见的非典型部位。除了评估炎症性肠病、血液系统疾病和风湿性疾病等其他疾病外,还应考虑潜在的自身炎症性疾病的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/759a/11444439/3d2b3cd305f2/IDOJ-15-770-g001.jpg

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