Gougerot-Hailey-Hailey disease

Alternative eponyms

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A skin disease affecting both sexes characterized by pemphigus with clusters of small vesicles arising on an erythematous base.

Description

A skin disease affecting both sexes, with onset usually in adolescence or early adulthood. It is characterized by pemphigus with clusters of small vesicles arising on an erythematous base; these change to rupturing flaccid bullae, leaving eroded areas covered by an amber-coloured crust. In their centres, the lesions show healing processes with pigmentation or moist areas. The lesions tend to spread peripherally, involving the neck, axillae, groin, and the genital, perianal, periumbilical and other regions, being limited to a few sites at one time, and occasionally to a single area. After a period of several months, the eruption heals without scarring. Recurrences usually occur in the same locations. Involvement of the oral mucosa, larynx, oesophagus, vulva, and vagina may be observed in some cases. Inheritance is autosomal dominant with variable penetrance.

Bibliography

  • H. Gougerot, Allé:
    Forme de transition entre la dermatite polymorphe douloureuse de Brocq-Duhring et le pemphigus congénital familial héréditaire.
    Annales de dermatologie et de syphiligraphie, Paris, 1933, 5: 255.
  • H. Hailey, H. Hailey:
    Familial benign chronic pemphigus. Report of 13 cases in four generations of a family and report of 9 additional cases in 4 generations of a family.
    Archives of Dermatology and Syphilology, Chicago, 1939, 39: 679-685.
  • H. Gougerot:
    La priorité du pemphigus chronique familial héréditaire bénin.
    Annales de dermatologie et de syphiligraphie, Paris, 1950, 10: 361-363.

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