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Related to Hailey-Hailey disease: Darier's disease, pemphigus, familial benign chronic pemphigus


An acute or chronic disease of the skin characterized by the appearance of bullae, which develop in crops or in continuous succession.



a chronic disease characterized by a crop of blisters, or bullae, having a flaccid covering and serous-hemorrhagic contents; the blisters form on the apparently intact skin or mucosa of the oral cavity, larynx, eyes, and genitals. The blisters rapidly enlarge and multiply, bursting to form extensive ulcerated surfaces. The patient’s general condition is disturbed; there is general weakness and elevation of body temperature. Infection of the mouth and larynx makes food intake difficult.

The causes of pemphigus have not been conclusively determined. The disease usually afflicts middle-aged and elderly persons. The mechanism of formation of pemphigus vulgaris is acantholysis, a type of degenerative change in epidermal cells. It involves the dissolution of the intercellular bridges, degenerative change of the nuclei, and loss of part of the cell protoplasm. As a result, communication between the layers of epidermis is disrupted. In other forms of pemphigus, the blisters form as a result of an inflammatory process.

Pemphigus vulgaris progresses gradually, and cachexia develops. The prognosis for persons suffering from other forms of pemphigus is relatively favorable. Treatment includes the ingestion of hormonal preparations, antimicrobial agents, or analgesics. Disinfecting solutions may be applied externally.


References in periodicals archive ?
A Swiss dermatologist from Bern rose from the audience to confirm the benefit of botulinum toxin in patients with Hailey-Hailey disease.
The diagnosis of Hailey-Hailey disease can be confirmed with a biopsy of perilesional skin that shows large numbers of keratinocytes in the suprabasal epidermis with incomplete acantholysis.
In dermatology, gene mapping has been used to map several inherited disorders in recent years, including different forms of epidermolysis bullas (blistering diseases), ichthyoses (scaling diseases), keratodermas, Darier's disease, Hailey-Hailey disease and PXE, among others.