Familial benign chronic pemphigus (Hailey-Hailey disease) treated with electron beam radiation

Graham, Paul M.; Melkonian, Allison A.; Fivenson, David P.

doi:10.1016/j.jdcr.2016.02.008

Cited by 9 publications

(6 citation statements)

References 10 publications

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“…Secondary infection of the skin lesions can occur and may cause an unpleasant odor. Sunlight, heat, sweating, and friction often aggravate the disorder [3,4].…”

Section: Discussionmentioning

confidence: 99%

“…Genetic defect results in altered cellular connections within desmosomes and adherens junctions of epidermis, secondary to high cytosolic calcium levels. Other manifestations include oxidative stress and microRNAs [4,5].…”

Section: Discussionmentioning

confidence: 99%

“…Its management involves control of exacerbating factors, secondary infections, and cutaneous inflammation. Topical therapy includes antibiotics, steroids, tacrolimus, and calcitriol, whereas systemic therapy includes antibiotics (clindamycin, gentamicin, and mupirocin), steroids, methotrexate, tacrolimus, dapsone, and thalidomide [3][4][5].…”

Section: Discussionmentioning

confidence: 99%

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Hailey-Hailey Disease – A Case Report

NAIR¹,

JOSEPH²,

LAKSHMI³

et al. 2020

Asian J Pharm Clin Res

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Hailey-Hailey disease (HHD) is a rare autosomal dominant skin condition discovered in 1939 by Hailey brothers. Prevalence is found to be around 1 in 50,000 and is relatively uncommon in India. First onset of disease occurs between 20 to 40 years of age, usually presented in the 3rd and 4th decades of life. Here, we report a case of a 50-year-old female who presented with clinical features of the HHD and showed greater response to the treatment.

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“…Secondary infection of the skin lesions can occur and may cause an unpleasant odor. Sunlight, heat, sweating, and friction often aggravate the disorder [3,4].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Hailey-Hailey Disease – A Case Report

NAIR¹,

JOSEPH²,

LAKSHMI³

et al. 2020

Asian J Pharm Clin Res

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“…HHD is a rare inherited disease that is caused by a mutation of ATP2C1 , which encodes for a magnesium-dependent calcium pump [ 6 , 7 ]. It was first described in 1939 by brothers Hailey and Hailey as an autosomal dominant disorder which manifests as recurrent episodes of pruritic vesicles and erosions [ 4 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…A dilapidated brick wall appearance is often present [ 3 ]. The treatments of choice include oral and topical antibiotics, along with systemic and topical corticosteroids [ 7 , 8 , 9 ]. Surgical therapies have also been suggested, such as the use of ablation as well as of diode and pulsed dye lasers [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

Recurrent Ulcerations in an 84-Year-Old Male Diagnosed with Hailey-Hailey Disease

Qooz¹,

Almuharraqi²,

Salam³

et al. 2020

Case Rep Dermatol

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Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is a rare inherited acantholytic dermatosis. It is an autosomal dominant disease affecting the intertriginous areas. HHD has been characterized by flaccid blisters, erosions, and macerations that are limited to flexural (friction-prone) areas. The painful blisters and erosions significantly decrease patients’ quality of life. There are multiple types of therapy related to this disorder. Many of the studies have suggested benefits from steroid therapy in addition to oral antibiotics.

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Inherited Acantholytic Disorders

Zamiri

2024

Rook's Textbook of Dermatology

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Darier disease (DD) and Hailey–Hailey disease (HHD) are autosomal dominant disorders characterised by epidermal acantholysis. They have a similar molecular pathogenesis, with mutations in intracellular calcium pumps of the endoplasmic reticulum ( SERCA2 ) and Golgi ( SPCA1 ), respectively. DD presents with persistent, hyperkeratotic papules in a seborrhoeic distribution, associated with nail changes and palmar pitting. HHD presents with painful, mainly flexural, erosions. In both, secondary infection is common and hypertrophic flexural disease can be disabling. Histologically, both are characterised by loss of epidermal intercellular adhesion (acantholysis). DD may be accompanied by neuropsychiatric symptoms such as depression, epilepsy, learning disabilities or bipolar disease. First line topical treatment is with emollients, antimicrobials and corticosteroids. Oral antibiotics are often necessary for exacerbations; other systemic options include retinoids and ciclosporin. Physical measures such as laser resurfacing, photodynamic therapy, botulinum toxin and surgical interventions can be considered in refractory cases.

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Familial benign chronic pemphigus (Hailey-Hailey disease) treated with electron beam radiation

Cited by 9 publications

References 10 publications

Hailey-Hailey Disease – A Case Report

Hailey-Hailey Disease – A Case Report

Recurrent Ulcerations in an 84-Year-Old Male Diagnosed with Hailey-Hailey Disease

Inherited Acantholytic Disorders

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