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1/5. A peculiar acantholytic dermatosis.

    This paper reports a peculiar case seen in a 36-year-old woman who every summer since the age of 30 has developed numerous solitary vesicles or crusted papules with intense pruritus in the seborrheic zones. There are no general symptoms and no hereditary relations. The individual rash disappears completely in a week to a month and there is no eruption in winter. The clinical picture resembles that of Darier's disease, but histopathological tests reveal intraepidermal cleft or bulla formation due to acantholysis but no dyskeratosis. Clinicopathological consideration indicates a disease akin to familial benign chronic pemphigus, but as our case does not correspond to any known disease, it is therefore reported as a peculiar acantholytic dermatosis.
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ranking = 1
keywords = eruption
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2/5. Bacterial infection-induced generalized Hailey-Hailey disease successfully treated by etretinate.

    Hailey-Hailey disease (HHD; familial benign chronic pemphigus) is a hereditary blistering disorder characterized by episodic maceration and erosions mainly in intertriginous areas, and generalized eruptions are rarely seen. We report here a 51-year-old woman with generalized HHD who was successfully treated with oral etretinate. The present case suggests that oral etretinate is effective against the generalized eruptions even in cases in which bacterial infection has triggered the generalization of HHD.
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ranking = 2
keywords = eruption
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3/5. Novel mutation in ATP2C1 gene in a Japanese patient with Hailey-Hailey disease.

    Hailey-Hailey disease (HHD) is an autosomal dominant disorder with recurrent eruption of vesicles and bullae involving predominantly the neck, groin and axillary regions. Histopathology shows suprabasal cleavage in epidermal cells. Recent studies have revealed that HHD is caused by mutations in the ATP2C1 gene encoding a novel Ca(2 ) pump. We analyzed mutations of the ATP2C1 gene in 2 Japanese patients with HHD. The diagnosis of HHD was made based on the characteristic clinical features and histopathological evidence. All 27 exons and flanking intron boundaries were amplified by polymerase chain reaction and products analyzed by sequencing. As a result, we identified a novel missense mutation (A1087G) in exon 13 of the ATP2C1 gene in a patient. This mutation led the amino acid change from Thrto Ala in the phosphorylation protein domain. Another patient showed no mutation of the gene. These results demonstrate that a spectrum of ATP2C1 gene mutations is present in Japanese HHD patients.
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ranking = 1
keywords = eruption
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4/5. Bullous drug eruption to griseofulvin in a man with Hailey-Hailey disease.

    Hailey-Hailey disease (chronic benign familial pemphigus) is a blistering disease characterized by episodic maceration and erosions in areas subject to friction and trauma such as the neck, axilla, and other intertriginous areas. We report a case in which a griseofulvin-induced drug eruption led to a generalized vesiculobullous eruption requiring treatment in a burn treatment center. This emphasizes that Hailey-Hailey disease, usually confined to relatively small areas, has the potential to cause blistering throughout the epidermis under certain conditions.
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ranking = 6
keywords = eruption
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5/5. Vesiculobullous Hailey-Hailey disease: successful treatment with oral retinoids.

    A 56-year-old male presented with a pruritic, generalized vesiculobullous eruption. His past history revealed classical symptoms of limited Hailey-Hailey disease for 34 years. Clinically, vesicles, bullae and occasional pustules were present and multiple biopsies confirmed this to be an unusual presentation of Hailey-Hailey disease. Various therapeutic modalities including topical and oral antibiotics, oral prednisone and dapsone failed to achieve sustained remission. Treatment with low-dose oral etretinate (25 mg daily) produced marked clinical improvement with complete suppression of new vesicle formation after 6 weeks.
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ranking = 1
keywords = eruption
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