Isolated Familial Benign Chronic Pemphigus

Hazelrigg, Donald E.

doi:10.1001/archderm.1977.01640090150051

Cited by 14 publications

(7 citation statements)

References 2 publications

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“…Negative immunofiuorescence was recorded in two instances (1,3). An absence of Cooper similar lesions elsewhere on the body was recorded in reports of 5 patients (2,4,6), and negative family histories were noted in descriptions of 6 patients (2,(4)(5)(6), Solitary lesions were successfully treated by excision, but the remaining eruptions were resistant to various therapies.…”

Section: Commentmentioning

confidence: 97%

“…The lesions were papular in 4 patients (1,3-5), consisted of solitary keratotic nodules in 3 cases (6), and presented as a single plaque in one instance (2), In 2 patients, the abtiormalities were first noted during therapy for candidal vaginitis (2,5). The primary pathologic finding was either acantholytic dyskeratosis (3,6) or acantholysis (2,4,5). In one instance, separate biopsies showed either acantholytic dyskeratosis or acantholysis (1).…”

Section: Commentmentioning

confidence: 99%

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Acantholytic dermatosis localized to the vulvocrural area

Cooper

1989

J Cutan Pathol

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This report describes 6 women with acantholytic lesions localized to the vulvocrural area. Clinically, multiple papules or solitary papules or plaques were situated on the labia majora or adjacent skin. None of the patients had similar abnormalities elsewhere on the body or family histories of similar eruptions. Two patients had concurrent candidal vaginitis. Microscopic findings consisted of acantholytic dyskeratosis resembling that observed in Darier's disease or acantholysis with minimal dyskeratosis similar to that seen in Hailey-Hailey disease. In one patient, acantholytic dyskeratosis with a cup-like configuration resembled the microscopic appearance of warty dyskeratoma. Immunofluorescence microscopy was negative in one patient. Solitary lesions were cured by local excision, but the papular eruption of one patient was recalcitrant to therapy. The literature contains descriptions of eight additional women with comparable lesions. Acantholytic reactions localized to the vulvocrural area are not apparently rare and need to be distinguished from other acantholytic dermatoses that can affect this anatomic site.

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Section: Commentmentioning

confidence: 97%

Section: Commentmentioning

confidence: 99%

Acantholytic dermatosis localized to the vulvocrural area

Cooper

1989

J Cutan Pathol

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“…There are reports in which a relationship to Hailey–Hailey disease has been favored over Darier disease . Predominantly Hailey–Hailey disease‐like histopathology has been seen in a minority of cases . Mutation in the ATP2C1 gene (affected in Hailey–Hailey disease) has been demonstrated in tissue from a patient with an extensive vulvocrural eruption showing Hailey–Hailey disease‐like pathology; however sequencing of blood was not performed, therefore, it is not possible to exclude a constitutional mutation in this case .…”

Section: Discussionmentioning

confidence: 89%

Somatic ATP2A2 mutation in a case of papular acantholytic dyskeratosis: mosaic Darier disease

et al. 2015

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Papular acantholytic dyskeratosis (PAD), also known as acantholytic dermatosis of the vulvocrural (or anogenital) area, is an uncommon eruption reported predominantly in women. This entity manifests with pruritic papules in the groin/anogenital area and less commonly on the chest. The pathobiology of PAD is uncertain. A 62-year old woman presented with multiple verrucous-appearing lesions in the groin and on the chest showing acantholytic dyskeratosis on histopathology. Given histological similarity of these PAD lesions to Darier disease (DD) due to inherited ATP2A2 mutation, we screened affected and normal tissue and peripheral blood in our patient for mutations in ATP2A2. We found an identical ATP2A2 p.706D>N mutation in multiple independent PAD lesions that was not present in uninvolved skin or peripheral blood DNA. These findings establish somatic mosaicism of ATP2A2 mutations as a genetic cause for PAD.

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“…[7][8][9] One or both of these patterns may be observed in biopsies from patients with ADGR. The patterns of acantholysis observed in this condition have varied.…”

Section: Discussionmentioning

confidence: 99%