Cheng S, Edmonds E, Ben-Gashir M, Yu R C
Departments of Dermatology and Histopathology, University College London Hospital, London, UK.
Clin Exp Dermatol. 2008 May;33(3):229-33. doi: 10.1111/j.1365-2230.2008.02706.x. Epub 2008 Mar 18.
We review the key developments in our understanding of subcorneal pustular dermatosis (SCPD, also known as Sneddon-Wilkinson disease) over the past 50 years. SCPD is a rare, chronic, sterile pustular eruption that was first described by Sneddon and Wilkinson in 1956. The primary lesions are pea-sized pustules classically described as half-pustular, half-clear flaccid fluid blisters. Histologically the salient feature is a subcorneal accumulation of neutrophils, suggesting the presence of chemoattractants such as tumour necrosis factor (TNF)alpha in the uppermost epidermis. However, to date its exact pathophysiology is unknown. Cases in association with pyoderma gangrenosum, benign monoclonal IgA gammopathy and multiple myeloma are well documented. There are anecdotal reports of SCPD associated with other internal malignancies such as chronic lymphocytic leukaemia, thymoma, apudoma and epidermoid carcinoma of the lung. The treatment of choice is dapsone. Therapeutic alternatives include retinoids, phototreatment with psoralen ultraviolet (UV) A, broadband or narrow band UVB and corticosteroids. Anecdotal uses of tacalcitol, ketoconazole, azithromycin, tetracycline, minocycline, vitamin E, ciclosporin, colchicine, mizoribine, mebhydrolin, infliximab and adalimumab with mycophenolate mofetil have all been reported.
我们回顾了过去50年里我们对角层下脓疱性皮肤病（SCPD，也称为斯内登-威尔金森病）认识的关键进展。SCPD是一种罕见的慢性无菌性脓疱性皮疹，于1956年由斯内登和威尔金森首次描述。主要皮损为豌豆大小的脓疱，经典描述为半脓疱、半清亮的松弛水疱。组织学上，显著特征是角层下中性粒细胞聚集，提示最上层表皮中存在趋化因子，如肿瘤坏死因子（TNF）α。然而，迄今为止其确切的病理生理学尚不清楚。与坏疽性脓皮病、良性单克隆IgA丙种球蛋白病和多发性骨髓瘤相关的病例已有充分记录。有传闻称SCPD与其他内部恶性肿瘤有关，如慢性淋巴细胞白血病、胸腺瘤、嗜银细胞瘤和肺表皮样癌。治疗的首选药物是氨苯砜。治疗选择还包括维甲酸、补骨脂素紫外线A（PUVA）光疗、宽带或窄带UVB光疗以及皮质类固醇。已有使用他卡西醇、酮康唑、阿奇霉素、四环素、米诺环素、维生素E、环孢素、秋水仙碱、咪唑立宾、美海屈林、英夫利昔单抗和阿达木单抗联合霉酚酸酯的传闻报道。
