Mayba Julia, Hawkins C Nicole
University of Saskatchewan, Saskatoon, SK, Canada.
SAGE Open Med Case Rep. 2019 Jan 30;7:2050313X19826432. doi: 10.1177/2050313X19826432. eCollection 2019.
We present a case of Sneddon-Wilkinson disease in a 52-year-old female at her first presentation to dermatology. Outlined in the case are various investigations undertaken at this initial presentation, including rheumatologic and hematologic malignancy markers, which identified immunoglobulin A gammopathy. The systemic and topical therapies used to treat the patient's condition are described, as well as her response to these treatments. In this discussion, we explain the epidemiology, pathophysiology, and clinical presentation of Sneddon-Wilkinson disease. Various medical conditions having known association with Sneddon-Wilkinson disease are discussed, including immunoglobulin A or immunoglobulin G monoclonal gammopathies and lymphoproliferative disorders. A comprehensive differential diagnosis for Sneddon-Wilkinson disease is provided, including immunoglobulin A pemphigus, acute generalized exanthematous pustulosis and pustular psoriasis, among others. We describe the systemic and topical therapy options for the treatment of Sneddon-Wilkinson disease, of which first line treatment is systemic dapsone. This patient serves as an excellent case of Sneddon-Wilkinson disease with unexpected immunoglobulin A gammopathy.
我们报告一例52岁女性初诊皮肤科时的Sneddon-Wilkinson病病例。病例中概述了初次就诊时进行的各项检查，包括风湿和血液系统恶性肿瘤标志物检查，结果发现免疫球蛋白Aγ病。文中描述了用于治疗该患者病情的全身和局部治疗方法，以及她对这些治疗的反应。在本讨论中，我们解释了Sneddon-Wilkinson病的流行病学、病理生理学和临床表现。讨论了已知与Sneddon-Wilkinson病相关的各种医学病症，包括免疫球蛋白A或免疫球蛋白G单克隆丙种球蛋白病和淋巴增殖性疾病。提供了Sneddon-Wilkinson病的全面鉴别诊断，包括免疫球蛋白A天疱疮、急性泛发性发疹性脓疱病和脓疱型银屑病等。我们描述了治疗Sneddon-Wilkinson病的全身和局部治疗选择，其中一线治疗是全身使用氨苯砜。该患者是伴有意外免疫球蛋白Aγ病的Sneddon-Wilkinson病的典型病例。
