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A case of Hailey-Hailey disease accompanied by Cushing's syndrome and adrenal insufficiency due to long-term usage of topical steroids with review of literature.
Endocr J
August 2025
Department of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Okayama 701-0192, Japan.
Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is a rare autosomal dominant disorder characterized by recurrent vesicles and erosions in intertriginous areas. Topical corticosteroids are the primary treatment, but their potential systemic side effects are often overlooked. Prolonged use on compromised skin can lead to excessive absorption, increasing the risk of iatrogenic Cushing's syndrome and adrenal insufficiency.
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Cureus
June 2025
Dermatology, Temple University Hospital, Philadelphia, USA.
Hailey-Hailey disease (HHD) is a rare autosomal dominant genodermatosis caused by mutations in the ATP2C1 gene, leading to impaired calcium homeostasis and epidermal acantholysis. Clinically, it manifests as recurrent, painful erosions in the intertriginous areas and is often resistant to conventional treatments, such as topical corticosteroids, antibiotics, and retinoids. This report describes the case of a 67-year-old woman with refractory HHD who presented with painful, pruritic erosions affecting the axillary, inguinal, and inframammary regions.
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Acta Dermatovenerol Croat
November 2024
Constantin A. Dasanu, Eisenhower Lucy Curci Cancer Center, 39000 Bob Hope Drive, Rancho Mirage, United States;
Familial benign chronic pemphigus, also known as Hailey-Hailey disease, was first described by the Hailey brothers in 1939 (1). It represents a chronic autosomal-dominant genetic skin disorder with incomplete penetrance, usually diagnosed in children and young adults. As a result, family history of this disorder can be elicited in only about 66% of patients.
View Article and Find Full Text PDFTreatment of Hailey-Hailey disease with the Janus kinase inhibitor abrocitinib: A case report.
SAGE Open Med Case Rep
June 2025
Division of Dermatology, Department of Medicine, McGill University Health Centre, Montreal, QC, Canada.
Hailey-Hailey disease is a rare, chronic, autosomal dominant skin disorder characterized by recurrent painful erosions and macerated plaques, primarily affecting intertriginous areas. It is caused by mutations in the gene, leading to impaired calcium homeostasis and keratinocyte adhesion. Many patients experience poor disease control despite conventional therapies.
View Article and Find Full Text PDFRare Ocular Association With Hailey-Hailey Disease.
Ochsner J
January 2025
Department of Ophthalmology, All India Institute of Medical Sciences Rajkot, Rajkot, Gujarat, India.
Background: Hailey-Hailey disease is an autosomal dominant blistering disorder characterized by junctional abnormalities of epidermal keratinocytes. Vesiculobullous eruptions affect the intertriginous areas of individuals with the condition. Ocular involvement associated with Hailey-Hailey disease is rare.
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