WEBOct 25, 2023 · Hailey-Hailey disease is a rare genetic disorder that is characterized by blisters and erosions most often affecting the neck, armpits, skin folds and genitals. The lesions may come and go and usually heal without scarring. Sunlight, heat, sweating and friction often aggravate the disorder.
WEBHailey–Hailey disease (HHD), or familial benign chronic pemphigus [1] : 559 or familial benign pemphigus, [2] : 622 was originally described by the Hailey brothers (Hugh Edward and William Howard) in 1939. [3] [4] It is a genetic disorder that causes blisters to form on the skin. Signs and symptoms.
WEBAug 2, 2017 · Benign familial pemphigus is also known as Hailey–Hailey disease. It is a rare hereditary blistering skin disease first described by the Hailey brothers in 1939. Unrelated to pemphigus vulgaris, Hailey-Hailey disease is not an autoimmune condition and has a differing prognosis.
WEBAug 14, 2023 · Hailey-Hailey disease (HHD; OMIM 169600), also known as benign familial pemphigus, is a rare genodermatoses firstly described in 1939 by the Hailey brothers.[1] HHD is characterized by impaired keratinocyte adhesion resulting in widespread intraepidermal acantholysis.
WEBSep 6, 2016 · Hailey-Hailey disease (HHD; MIM #169600), also called benign familial pemphigus or benign chronic pemphigus, is a rare, autosomal dominant disorder that affects the adhesion of epidermal keratinocytes. Initially described by the Hailey brothers in 1939 [ 1 ], this intraepidermal blistering disorder is characterized by painful …
WEBHailey-Hailey disease, also known as benign chronic pemphigus, is a rare skin condition that usually appears in early adulthood. The disorder is characterized by red, raw, and blistered areas of skin that occur most often in skin folds, such as the groin, armpits, neck, and under the breasts.
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Management of familial benign chronic pemphigus - PMC
WEBSep 14, 2016 · Benign familial chronic pemphigus, also known as Hailey–Hailey disease (HHD), is a genodermatosis that affects the action of desmosomes between keratinocytes in the epidermis. 1, 2 The autosomal dominant mutation takes place in the ATP2C1 gene, which affects the function of calcium-dependent ATPases whose usual …
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GARD Rare Disease Information - Hailey-Hailey disease
WEBDisease Overview. Hailey-Hailey disease is a genetic skin disease that causes blistering. Signs and symptoms include a painful rash and blistering in skin folds, such as the armpits, groin, neck, under the breasts, and between the buttocks. Secondary bacterial infections are not uncommon.
WEBHailey-Hailey disease is also known as familial benign chronic pemphigus. It was originally described by the Hailey brothers. It is a rare inherited skin condition in which there are red scaly areas that can be itchy and sore, that can lead to superficial blisters and eroded (broken) areas of the skin.
WEBSep 6, 2023 · Hailey-Hailey disease (HHD) is caused by loss-of-function variants in the ATP2C1 gene at 3q22.1, which encodes the adenosine triphosphate (ATP)-powered, magnesium-dependent calcium pump protein hSPCA1. Its function is to maintain normal intracellular concentrations of free calcium (Ca 2+) by sequestering Ca 2+ into the Golgi …
