文献简介

出版社:Arch Dermatol

作  者:Peng Wang, MD; Hanjun Yang, MD; Yuping Ran, MD, PhD

编  号:

关键字:papular mucinosis with IgA nephropathy

年  份:2011   点击量:391

文献摘要 全文翻译

Background: Papular mucinosis is an idiopathic cutaneous mucinosis. It includes 3 clinicopathologic subsets according to the recent Rongioletti classification: a generalized papular form, a localized form, and atypical forms. The localized form is subdivided into 5 subtypes. The atypical form does not meet the criteria for either the generalized papular form or the localized forms and includes localized forms with monoclonal gammopathy and/or systemic symptoms.
Observations: We describe a 37-year-old man who had multiple small, waxy papular lesions on the neck for 4 years. One year after the lesions first appeared, he developed IgA nephropathy. No paraproteinemia or thyroid dysfunction was observed. Histologic examination of the skin lesions showed mucin deposits in the upper dermis. Alcian blue–positive mucin was also found in the renal interstitium and around some vessels and capillary loops. A diagnosis of localized papular mucinosis with IgA nephropathy (atypical form) was made after the biopsy.
Conclusions: The patient showed an unusual manifestation of localized papular mucinosis with the lesions confined to the neck and IgA nephropathy. Alcian blue staining is a useful way to determine kidney involvement of papular mucinosis.
背景:丘疹性黏蛋白病是一种特发性皮肤粘蛋白疾病。根据Rongioletti最新分类,其包括3种临床病理类型:泛发性丘疹型、局部型以及非典型。局部型可分为5种亚型。非典型既不符合泛发型丘疹型,也不符合局部类型,但包括单克隆丙种球蛋白病和/或全身性症状的局部类型。
观察:本文报道了一例37岁男性患者病例,颈部多个小而柔软的丘疹4年。皮损出现一年后,发展成IgA肾病。未观察到副球蛋白血症或甲状腺功能减退。皮损组织学检查显示,真皮上部黏蛋白沉积。在肾间质和周围血管以及毛细血管袢可见,阿新蓝染色阳性。活组织检查后确诊为伴有IgA肾病的局部丘疹性黏蛋白病(非典型)。
结论:本例患者伴有局部丘疹性黏蛋白病罕见的临床症状,其皮损局限于颈部并伴有IgA肾病。阿新蓝染色是确诊肾脏受累的丘疹性黏蛋白病的有效方法。