Cutaneous pemphigus vulgaris with skin features similar to the classic mucocutaneous type: a case report and review of the literature
Cutaneous pemphigus vulgaris with skin features similar to the classic mucocutaneous type: a case report and review of the literature
复制标题
DOI：
10.1111/j.1365-2230.2008.02871.x
复制
发表时间：
2008-11-01
影响因子：
4.1
通讯作者：
Shimizu, H.
中科院分区：
文献类型：
--
作者：
Shinkuma, S.;Nishie, W.;Shimizu, H.
寻常天疱疮（PV）是一种危及生命的自身免疫性起泡性皮肤病，特别是涉及口腔粘膜。最近的研究表明，极少数PV患者没有表现出粘膜受累，尽管他们有针对桥粒芯糖蛋白（Dsg）1和Dsg 3的循环自身抗体，这些抗体与组织病理学基底上棘层松解症相关。这些病例被归类为皮肤型PV。我们在此报告一位50岁男性发生皮肤型肺静脉的病例，临床检查发现患者全身有许多紧张且扩散的水疱和糜烂，与典型的粘膜皮肤型肺静脉相似。有趣的是，先前报告的皮肤PV患者均未表现出与粘膜皮肤PV相似的皮肤特征，而本病例明确显示出与粘膜皮肤PV相似的非常典型的临床特征。
Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering skin disease that specifically involves oral mucosa. It was recently shown that a very small number of patients with PV show no mucous membrane involvement although they have circulating autoantibodies directed against both desmoglein (Dsg)1 and Dsg3 that are associated with histopathological suprabasal acantholysis. These cases are classed as cutaneous-type PV. We report here a case of cutaneous-type PV that occurred in a 50-year-old man. Clinical examination revealed numerous tense and spreading blisters and erosions over the patient's entire body, similar to the classic mucocutaneous-type PV. Interestingly, none of the previously reported patients with cutaneous PV had shown skin features like those of mucocutaneous PV, whereas the present case clearly demonstrated very typical clinical features similar to those in mucocutaneous PV.