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探索一例罕见的黑棘皮病病例:了解脆弱皮肤的表现。

Exploring a Unique Case of Hailey-Hailey Disease: Understanding the Manifestation of Fragile Skin.

作者信息

Priya Asokan Lakshmi, Arumugam Sumithra, Sonti Sulochana

机构信息

Department of Pathology, Saveetha Medical College and Hospital, Saveetha Institute of Medical and Technical Sciences, Saveetha University, Chennai, IND.

出版信息

Cureus. 2024 Sep 14;16(9):e69432. doi: 10.7759/cureus.69432. eCollection 2024 Sep.

DOI:10.7759/cureus.69432
PMID:39411604
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11479426/
Abstract

Hailey-Hailey disease (HHD) is a rare autosomal dominant chronic blistering disorder. It presents with eruption of small vesicles, erosions, and crusted plaques frequently in the intertriginous areas. Family history is present in some cases. Histopathology plays a crucial role in diagnosing HHD, which is identified by its distinct "dilapidated brick wall" appearance. Due to its uncommon nature and similarity to other skin conditions in intertriginous areas, HHD can often be misdiagnosed by clinicians. We present a case of a 46-year-old female with crusted lesions in the neck, antecubital fossa, submammary folds, and inguinal regions. There were recurrent exacerbations and remissions. Biopsy was sent for histopathological examination, which helped in confirming the diagnosis of HHD.

摘要

海利-海利病(HHD)是一种罕见的常染色体显性慢性水疱性疾病。其特征为在皮肤褶皱部位频繁出现小水疱、糜烂及结痂斑块。部分病例有家族病史。组织病理学在HHD的诊断中起着关键作用,其具有独特的“破败砖墙”外观可资鉴别。由于其罕见性以及与皮肤褶皱部位其他皮肤病的相似性,临床医生常对HHD误诊。我们报告一例46岁女性病例,其颈部、肘前窝、乳房下皱襞及腹股沟区出现结痂性皮损,病情反复加重与缓解。已送检活检进行组织病理学检查,这有助于确诊HHD。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/e4bf5c9316c7/cureus-0016-00000069432-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/22516fa9a805/cureus-0016-00000069432-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/6b918bc4fb12/cureus-0016-00000069432-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/3aafe6d81946/cureus-0016-00000069432-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/334ec56dc698/cureus-0016-00000069432-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/321b75f5cbb0/cureus-0016-00000069432-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/e4bf5c9316c7/cureus-0016-00000069432-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/22516fa9a805/cureus-0016-00000069432-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/6b918bc4fb12/cureus-0016-00000069432-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/3aafe6d81946/cureus-0016-00000069432-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/334ec56dc698/cureus-0016-00000069432-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/321b75f5cbb0/cureus-0016-00000069432-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9005/11479426/e4bf5c9316c7/cureus-0016-00000069432-i06.jpg

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