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与IgG-κ副蛋白血症相关的Buschke硬化性黏液水肿的全身受累。

Systemic involvement in scleredema of Buschke associated with IgG-kappa paraproteinaemia.

作者信息

Basarab T, Burrows N P, Munn S E, Russell Jones R

机构信息

Department of Dermatology, Ealing Hospital, Southall, Middlesex, UK.

出版信息

Br J Dermatol. 1997 Jun;136(6):939-42.

PMID:9217831
Abstract

Scleredema is a rare primary cutaneous mucinosis. Systemic involvement is uncommon and histological confirmation is often lacking. We report a case of a 60-year-old man with scleredema and evidence of mucin deposition on biopsies from multiple extracutaneous sites. The bone marrow, nerve, hepatic and salivary gland involvement seen on histology in our patient has not, to our knowledge, been previously reported in this condition.

摘要

硬化性水肿是一种罕见的原发性皮肤粘蛋白病。系统性受累并不常见,且往往缺乏组织学确诊依据。我们报告一例60岁男性硬化性水肿患者,其多处皮肤外活检显示有粘蛋白沉积证据。据我们所知,我们患者组织学所见的骨髓、神经、肝脏及唾液腺受累情况在该病中此前未见报道。

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Systemic involvement in scleredema of Buschke associated with IgG-kappa paraproteinaemia.与IgG-κ副蛋白血症相关的Buschke硬化性黏液水肿的全身受累。
Br J Dermatol. 1997 Jun;136(6):939-42.
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From scleredema to AL amyloidosis: disease progression or coincidence? Review of the literature.从硬化性水肿到AL淀粉样变性:疾病进展还是巧合?文献综述
Clin Rheumatol. 2006 Feb;25(1):3-15. doi: 10.1007/s10067-004-1076-3. Epub 2005 Mar 2.