Bennett Adam E, Fenske Neil A, Rodriguez-Waitkus Paul, Messina Jane L
Department of Dermatology and Cutaneous Surgery, University of South Florida Morsani College of Medicine, Tampa, FL, USA.
Department of Pathology and Cell Biology, University of South Florida Morsani College of Medicine, Tampa, FL, USA.
Int J Dermatol. 2016 Nov;55(11):1184-1195. doi: 10.1111/ijd.13369.
IgG4-related disease (IgG4-RD) is an increasingly prevalent protean multisystem disorder characterized by single or multi-organ infiltration of IgG4-bearing plasma cells. Skin involvement has been recognized and is relevant to proper diagnosis. A systematic literature review of 50 cases involving the skin reveals that patients with IgG4-related skin disease show predominant involvement of the head and neck and have a distinct pattern of systemic involvement, also favoring the head and neck - lymphatics, orbit, salivary, and lacrimal glands - but generally lacking pancreaticobiliary involvement (16% of cases), which by contrast is a predominant manifestation in systemic IgG4-RD (60% with pancreaticobiliary involvement). We summarize clinical and pathologic descriptive data from this systematic review. We review differential diagnosis and propose a diagnostic scheme for stratifying probability of disease based upon comprehensive integration of clinical, histopathologic, and laboratory data. Plasmacyte infiltration and storiform fibrosis are prominent in IgG4-related skin disease, but obliterative venulitis is less common than in the prototypical IgG4-related disease manifestation of autoimmune pancreatitis. IgG4 tissue and serum values, with a mean (±95% CI) in the reviewed cases of 132.8 ± 32.6 IgG4-positive plasma cells per high-power field and 580 ± 183.8 mg/dl, respectively, are incorporated into the suggested criteria. The distinct set of manifestations identified by this systematic review and the proposed diagnostic considerations, while requiring further validation in prospective studies, highlight the need to consider that IgG4-related skin disease defines a unique systemic disease complex along the spectrum of IgG4-RD.
IgG4相关疾病（IgG4-RD）是一种日益常见的具有多种表现的多系统疾病，其特征为携带IgG4的浆细胞单器官或多器官浸润。皮肤受累已得到认识且与正确诊断相关。一项对50例皮肤受累病例的系统性文献综述显示，IgG4相关皮肤疾病患者主要累及头颈部，且具有独特的全身受累模式，也倾向于累及头颈部的淋巴管、眼眶、唾液腺和泪腺，但一般无胰腺胆管受累（16%的病例），相比之下，胰腺胆管受累是系统性IgG4-RD的主要表现（60%有胰腺胆管受累）。我们总结了该系统性综述中的临床和病理描述性数据。我们回顾了鉴别诊断，并基于临床、组织病理学和实验室数据的综合整合提出了一种用于分层疾病可能性的诊断方案。浆细胞浸润和席纹状纤维化在IgG4相关皮肤疾病中较为突出，但闭塞性静脉炎比自身免疫性胰腺炎这种典型的IgG4相关疾病表现中少见。IgG4组织和血清值分别纳入了建议标准，在所综述病例中的均值（±95%CI）分别为每高倍视野132.8±32.6个IgG4阳性浆细胞和580±183.8mg/dl。该系统性综述所确定的独特表现集以及所提出的诊断考量，虽然需要在前瞻性研究中进一步验证，但突出了需要考虑IgG4相关皮肤疾病在IgG4-RD范围内定义了一种独特的系统性疾病复合体。
