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

被引:0
作者
Asokan, Lakshmi Priya [1 ]
Arumugam, Sumithra [1 ]
Sonti, Sulochana [1 ]
机构
[1] Saveetha Univ, Saveetha Med Coll & Hosp, Saveetha Inst Med & Tech Sci, Dept Pathol, Chennai, India
关键词
autosomal dominant; pemphigus; acantholysis; intraepidermal blister; hailey-hailey disease;
D O I
10.7759/cureus.69432
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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 46year-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.
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