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莫尔比昂病合并皮肤垢着病:一种独特的表现。

Morbihan disease complicated by dermatosis neglecta: An unique presentation.

作者信息

Eber Ariel E, Mlacker Stephanie, Verne Sebastian, Griffith Robert, Ledon Jennifer, Perper Marina, Nouri Keyvan, Cho-Vega Jeong Hee

机构信息

Department of Dermatology and Cutaneous Surgery, Miller School of Medicine,University of Miami, Miami, Florida.

Dermatopathology Division, Department of Pathology, Sylvester Comprehensive Cancer Center, Miami, Florida.

出版信息

J Cutan Pathol. 2017 May;44(5):470-473. doi: 10.1111/cup.12902. Epub 2017 Feb 16.

Abstract

Morbihan disease, also referred to as solid facial edema, or rosacea lymphedema, is a rare disorder that involves chronic erythema and solid edema of the cheeks, eyelids, forehead and glabella and may arise as a complication of acne vulgaris or rosacea. Of note, it may be the only initial presenting symptom of these associated diseases. Few cases have been described in the literature, as its first description by Robert Degos in 1957. The condition is characterized by its chronicity, a typical clinical appearance and the lack of specific histopathologic or laboratory findings. The condition may wax and wane but typically does not resolve without treatment. Many cases of this condition tend to be recalcitrant to therapy, with topical and oral antibiotics regimens commonly used for rosacea generally being ineffective. The disease may easily go undiagnosed, as it mimics other more common skin conditions. We present a case of originally undiagnosed Morbihan disease mistaken for an atypical allergic rash, resistant to treatment, and complicated by dermatosis neglecta.

摘要

莫尔比昂病,也称为实性面部水肿或酒渣鼻性淋巴水肿,是一种罕见的疾病,表现为脸颊、眼睑、前额和眉间的慢性红斑和实性水肿,可能作为寻常痤疮或酒渣鼻的并发症出现。值得注意的是,它可能是这些相关疾病唯一的初始表现症状。自1957年罗伯特·德戈斯首次描述以来,文献中报道的病例很少。该疾病的特点是病程慢性、典型的临床表现以及缺乏特异性的组织病理学或实验室检查结果。病情可能会反复,但通常未经治疗不会缓解。这种疾病的许多病例往往对治疗有抵抗性,通常用于治疗酒渣鼻的局部和口服抗生素方案一般无效。由于它与其他更常见的皮肤疾病相似,这种疾病很容易被漏诊。我们报告一例最初未被诊断出的莫尔比昂病,被误诊为非典型过敏性皮疹,对治疗有抵抗性,并伴有皮肤垢着病。

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