Familial “benign” pemphigus? Erythroderma and fatal outcome

Tansini, Paula Baldissera; Boff, Ana Letícia; Weber, Magda Blessmann; Bonamigo, Renan Rangel

doi:10.1016/j.abd.2019.02.006

Cited by 4 publications

(7 citation statements)

References 9 publications

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“…Changes in calcium levels lead to alteration of desmosomes and the cellular adhesion of keratinocytes to the epidermal junction, which will eventually cause acantholysis. 5 Hailey-Hailey disease has an incidence of 1 in 50,000, which makes it a fairly uncommon condition worldwide.…”

Section: Discussionmentioning

confidence: 99%

Spot the Difference: A Case of Hailey-Hailey Disease in a 64-year-old Filipino Female

2021

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Hailey-Hailey disease (HHD) is an uncommon acantholytic disorder of the skin. This is a case of a 64-year-old Filipino female with a chronic history of painful and malodorous intertriginous plaques. Histopathologic evaluation showed overlapping features of pemphigus vulgaris and Hailey-Hailey disease. A negative direct immunofluorescence test clinched the diagnosis of Hailey-Hailey disease. The patient was advised regarding preventive measures and treated with topical antibiotics and corticosteroids with improvement of her lesions.

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Section: Discussionmentioning

confidence: 99%

Spot the Difference: A Case of Hailey-Hailey Disease in a 64-year-old Filipino Female

2021

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“…Bacterial infection caused by Staphylococcus species is a common feature of HHD [ 4 ]. It is of an unknown prevalence [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…A diagnosis is usually made on histopathological grounds, where suprabasal acantholysis is appreciated. 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 ].…”

Section: Discussionmentioning

confidence: 99%

“…Hailey-Hailey disease (HHD), also known as familial benign chronic pemphigus, is a rare autosomal dominant skin disorder [ 1 , 2 ]. It is caused by a mutation in the ATP2C1 gene, which encodes for intracellular calcium pumps that alter the homeostasis of this ion [ 3 , 4 , 5 ]. Excessive sweating, heat, and trauma to the skin may exacerbate HHD and cause separation of keratinocytes, especially in friction-prone areas [ 1 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…It is caused by a mutation in the ATP2C1 gene, which encodes for intracellular calcium pumps that alter the homeostasis of this ion [ 3 , 4 , 5 ]. Excessive sweating, heat, and trauma to the skin may exacerbate HHD and cause separation of keratinocytes, especially in friction-prone areas [ 1 , 3 ]. Therapeutic options range from topical to systemic corticosteroids and antibiotics, among others [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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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