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[黏液水肿性苔藓和硬化性黏液水肿]

[Lichen myxedematosus and scleromyxedema].

作者信息

Hödl S

出版信息

Hautarzt. 1982 Jul;33(7):359-65.

PMID:7107280
Abstract

Review of the clinical picture, dynamics of the disease, associated symptoms and signs, etiopathology as well as treatment and prognosis of lichen myxedematosus and scleromyxedema. Diagnostic criteria are wax-like papules and leather-like skin thickening, an increase in concentration of acid mucopolysaccharides consisting mainly of hyaluronic acid in the dermis, lympho-plasmacytoid infiltrates in the skin and the bone marrow, normal thyroid function, and paraproteinemia of light chain type lambda or kappa. The nosologic connection of both manifestations implying a single disease entity is emphasized by two of our clinical observations of the transition of lichen myxedematosus to scleromyxedema. An attempt will be made to interpret this as a certain kind of myelomesenchymal syndrome.

摘要

黏液性苔藓和硬化性黏液水肿的临床表现、疾病动态、相关症状和体征、病因病理学以及治疗和预后的综述。诊断标准为蜡样丘疹和皮革样皮肤增厚、真皮中主要由透明质酸组成的酸性黏多糖浓度增加、皮肤和骨髓中的淋巴细胞 - 浆细胞样浸润、甲状腺功能正常以及λ或κ轻链型副蛋白血症。我们对黏液性苔藓转变为硬化性黏液水肿的两项临床观察强调了这两种表现的疾病学联系,意味着它们是单一的疾病实体。将尝试把这解释为某种骨髓间充质综合征。

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