Department of Dermatology, Brigham and Women's Hospital, Boston, MA 02115, USA; Boston University School of Medicine, Boston, MA 02118, USA.
Department of Dermatology, Brigham and Women's Hospital, Boston, MA 02115, USA.
Dermatol Clin. 2024 Apr;42(2):307-315. doi: 10.1016/j.det.2023.08.010. Epub 2023 Sep 7.
Sneddon-Wilkinson disease (SWD), IgA pemphigus, and bullous systemic lupus erythematosus (BSLE) are superficial and bullous neutrophilic dermatoses. They are all characterized by sterile neutrophilic infiltrate but differ in the level of skin affected and presence of autoantibodies. Both SWD and IgA pemphigus present with grouped flaccid pustules and have epidermal involvement; it is unclear whether they are distinct or exist on a spectrum of the same disease. IgA pemphigus is distinguished from SWD by positive direct immunofluorescence showing intercellular IgA deposition. BSLE presents with tense bullae, dermal neutrophilic infiltrate, and direct immunofluorescence showing linear IgG deposition along the dermal-epidermal junction.
斯奈德-威尔金森病（SWD）、IgA 天疱疮和大疱性系统性红斑狼疮（BSLE）均为浅表性和大疱性中性粒细胞皮肤病。它们均以无菌性中性粒细胞浸润为特征，但在受累皮肤的程度和自身抗体的存在上有所不同。SWD 和 IgA 天疱疮均表现为成群的松弛性脓疱，且累及表皮；目前尚不清楚它们是两种不同的疾病，还是同一种疾病的不同表现形式。IgA 天疱疮与 SWD 的区别在于，直接免疫荧光显示细胞间 IgA 沉积呈阳性。BSLE 表现为紧张性大疱、真皮中性粒细胞浸润，直接免疫荧光显示沿表皮-真皮交界处线状 IgG 沉积。
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