Scleromyxedema and the dermato-neuro syndrome: case report and review of the literature

被引：48

作者：

Fleming, Kirsten E. ^{[1
,2
]}

Virmani, Divya ^{[4
,5
]}

Sutton, Evelyn ^{[2
,6
]}

Langley, Richard ^{[2
,3
]}

Corbin, Jessica ^{[2
,3
]}

Pasternak, Sylvia ^{[1
,2
]}

Walsh, Noreen M. ^{[1
,2
]}

机构：

[1] Capital Dist Hlth Author, Div Anat Pathol, Dept Pathol, Halifax, NS B3H 1V8, Canada

[2] Dalhousie Univ, Halifax, NS, Canada

[3] Capital Dist Hlth Author, Div Dermatol, Dept Med, Halifax, NS B3H 1V8, Canada

[4] Univ Calgary, Dept Med, Div Infect Dis, Calgary, AB, Canada

[5] Alberta Hlth Serv, Calgary, AB, Canada

[6] Capital Dist Hlth Author, Div Rheumatol, Dept Med, Halifax, NS B3H 1V8, Canada

来源：

JOURNAL OF CUTANEOUS PATHOLOGY | 2012年 / 39卷 / 05期

关键词：

dermato-neuro syndrome; lichen myxedematosus; nephrogenic systemic fibrosis; papular mucinosis; scleromyxedema; LICHEN MYXEDEMATOSUS; PAPULAR MUCINOSIS; ENCEPHALOPATHY;

D O I：

10.1111/j.1600-0560.2012.01882.x

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Scleromyxedema is a generalized form of lichen myxedematosus which is characterized by a papular and sclerodermoid skin eruption resulting from dermal fibroblast proliferation and mucin deposition. The majority of patients with scleromyxedema have a monoclonal gammopathy, and other systemic manifestations are common. Herein we describe a case of the dermato-neuro syndrome, a rare and sometimes fatal neurologic manifestation of scleromyxedema which consists of fever, convulsions and coma, often preceded by a flu-like prodrome. In addition, we provide a comprehensive summary of previously published cases of the dermato-neuro syndrome and discuss the current etiopathogenic theories and treatment options for this rare disease.

引用

页码：508 / 517

页数：10

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