Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.
UOC Dermatologia, IRCCS Fondazione Ca' Granda Ospedale Maggiore Policlinico, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Milan, Italy.
Arch Dermatol Res. 2019 Aug;311(6):425-434. doi: 10.1007/s00403-019-01912-1. Epub 2019 Mar 29.
Pyoderma Gangrenosum (PG) is an inflammatory neutrophilic dermatosis (ND) associated with underlying chronic inflammation and/or malignancy. Diagnosis remains to be challenging as a gold standard diagnostic test is lacking. Initial manifestations may include papules, vesicles, or pustules that subsequently develop into ulceration with features of undermining and violaceous borders. Timely recognition of pyoderma gangrenosum is impeded by clinical findings shared with other etiologies, such as granulomatosis with polyangiitis, polyarteritis nodosa, and antiphospholipid syndrome. As with any other ND, extracutaneous involvement may also occur preceding, during, or following the appearance of skin lesions. Sterile neutrophilic infiltrates have been found to affect internal organs supporting the concept of PG being a systemic disease, with lung being the most common extracutaneous manifestation followed by ocular and visceral compromise. Therefore, in this review, we describe the current knowledge of extracutaneous involvement of PG and its respective clinical manifestations to aid dermatologists in diagnosis, management, and determining prognosis.
坏疽性脓皮病（PG）是一种与潜在慢性炎症和/或恶性肿瘤相关的炎症性中性粒细胞皮肤病（ND）。由于缺乏金标准诊断测试，因此诊断仍然具有挑战性。最初的表现可能包括丘疹、水疱或脓疱，随后发展为溃疡，具有潜行和暗紫色边缘的特征。由于与其他病因（如肉芽肿性多血管炎、结节性多动脉炎和抗磷脂综合征）具有共同的临床特征，因此及时识别坏疽性脓皮病受到阻碍。与任何其他 ND 一样，皮肤病变出现之前、期间或之后也可能发生皮肤外受累。已经发现无菌性中性粒细胞浸润会影响内脏器官，支持 PG 是一种全身性疾病的概念，肺部是最常见的皮肤外表现，其次是眼部和内脏受累。因此，在这篇综述中，我们描述了 PG 的皮肤外受累及其各自的临床表现的最新知识，以帮助皮肤科医生进行诊断、管理和确定预后。
