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组织病理学上类似硬斑病的Ⅰ型和Ⅱ型硬化性黏液水肿合并的非典型病例。

Atypical Case of Combined Types I and II Scleredema Mimicking Morphea on Histopathology.

作者信息

Alhunaif Sarah A, Alsarheed Abeer, Almutairi Raghad, Almutairi Ghaida

机构信息

Dermatology, King Abdulaziz Medical City - National Guard Health Affairs, Riyadh, SAU.

出版信息

Cureus. 2023 Jan 23;15(1):e34077. doi: 10.7759/cureus.34077. eCollection 2023 Jan.

Abstract

Scleredema is a rare sclerotic skin disorder that typically develops in conjunction with diabetes, monoclonal gammopathy, or infection and commonly involves the neck, back, trunk, and arms. Scleredema can be categorized into three types according to its cause. The local examination of the lesion is characterized by non-pitting induration that follows a symmetrical spread with sparing of the hands and feet. We present a case of a 19-year-old female patient that presented to the outpatient clinic complaining of facial skin lesions that have been progressive for four years. The skin lesions were found to involve the neck, back, trunk and both arms sparing the hands and there was no systemic involvement of the disease. The patient is non-diabetic but reported frequent attacks of tonsillitis over the past months. Initially, punch biopsy showed no signs of scleredema; however, repeated biopsy at follow-up confirmed the presence of mucin deposits which are suggestive features of scleredema. Due to the similarities with various other diseases, the diagnosis requires clinical and histopathological exclusion which makes the diagnosis quite challenging. It almost always starts in the neck but can present initially in rare cases from the face spreading symmetrically. Close follow-up and continuous monitoring are necessary for systemic organ involvement.

摘要

硬化性水肿是一种罕见的硬化性皮肤病,通常与糖尿病、单克隆丙种球蛋白病或感染同时发生,常见于颈部、背部、躯干和手臂。根据病因,硬化性水肿可分为三种类型。病变的局部检查特征为非凹陷性硬结,呈对称性扩散,手部和足部不累及。我们报告一例19岁女性患者,该患者到门诊就诊,主诉面部皮肤病变已持续四年且呈进行性发展。发现皮肤病变累及颈部、背部、躯干和双臂,手部未受累,且该疾病无全身累及情况。该患者无糖尿病,但报告在过去几个月中扁桃体炎频繁发作。最初,穿刺活检未显示硬化性水肿的迹象;然而,随访时重复活检证实存在粘蛋白沉积,这是硬化性水肿的特征性表现。由于与其他多种疾病相似,诊断需要临床和组织病理学排除,这使得诊断颇具挑战性。它几乎总是从颈部开始,但在极少数情况下最初可从面部开始并对称扩散。对于全身器官累及情况,密切随访和持续监测是必要的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b46f/9944634/60fe49166275/cureus-0015-00000034077-i01.jpg

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