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肥胖相关性淋巴水肿性黏液水肿和淤滞性黏液水肿。

Obesity-associated lymphedematous mucinosis and stasis mucinosis.

机构信息

Unit of Dermatology, Department of Medical Science and Public Health, University of Cagliari, Italy.

Unit of Dermatology, Department of Medical Science and Public Health, University of Cagliari, Italy.

出版信息

Clin Dermatol. 2021 Mar-Apr;39(2):229-232. doi: 10.1016/j.clindermatol.2020.10.014. Epub 2020 Oct 16.

DOI:10.1016/j.clindermatol.2020.10.014
PMID:34272015
Abstract

Cutaneous dermal mucinoses are a group of conditions characterized by abnormal deposition of mucin (hyaluronic acid and sulfated glycosaminoglycans) in the dermis. They can be classified either as localized or generalized forms and occur primarily or secondarily to systemic disorders. Obesity-associated lymphedematous mucinosis and pretibial stasis mucinosis are uncommon and relatively newly recognized disorders occurring in obese patients or in patients with venous insufficiency. Clinically, patients present with papules and nodules progressing to plaques and arising in an erythematous and edematous basis on the legs, especially the shins. Histopathologic examination indicates mucin deposition in the superficial portion of the dermis, angioplasia with an increase in small blood and/or lymphatic vessels, vertically running vessels, slight fibrosis, and no inflammation. Laboratory workup and histopathologic testing are helpful in differentiating these entities from pretibial myxedema (thyroid dermopathy), which represents the main differential diagnosis. Obesity-associated lymphedematous mucinosis and stasis mucinosis are not different entities but belong to a spectrum of mucinoses secondary to an increased body mass index, chronic lymphedema, and/or chronic venous insufficiency.

摘要

皮肤真皮黏液增多症是一组以真皮中黏蛋白(透明质酸和硫酸化糖胺聚糖)异常沉积为特征的疾病。它们可以分为局限性或全身性形式,主要或次要发生于系统性疾病。肥胖相关性淋巴水肿性黏液增多症和小腿静脉淤滞性黏液增多症是罕见且新近认识的疾病,发生于肥胖患者或静脉功能不全患者。临床上,患者表现为丘疹和结节,进展为斑块,在腿部,特别是小腿,出现红斑和水肿基础上。组织病理学检查显示黏蛋白沉积于真皮浅层,伴有小血管和/或淋巴管、垂直走行的血管、轻微纤维化而无炎症的血管形成。实验室检查和组织病理学检查有助于将这些病变与胫前黏液水肿(甲状腺皮肤病)相鉴别,后者是主要的鉴别诊断。肥胖相关性淋巴水肿性黏液增多症和静脉淤滞性黏液增多症不是不同的实体,而是属于由于体重指数增加、慢性淋巴水肿和/或慢性静脉功能不全引起的黏蛋白增多症谱。

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