Behçet's syndrome

Alternative eponyms

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Recurrent systemic disease characterized by uveitis with hypopyon, recurrent ulceration of the mucous membranes of the mouth and pharynx, and ulceration of the genitalia.

Description

Recurrent systemic disease characterized by uveitis with hypopyon, recurrent ulceration of the mucous membranes of the mouth and pharynx, and ulceration of the genitalia. Joint pain is a trait that usually comes later in the history of the disease than the ulcers of the mouth and genitalia.

Iritis frequently is accompanied by conjunctivitis, episcleritis, keratitis, retinal thrombophlebitis, and optic atrophy. The central nervous system, heart, and intestinal tract may be involved. Onset is usually between 10 and 30 years of age, but may occur as late as age 45, and males are affected approximately 5 times more frequently than females. Recurrent every 2 to 3 months and lasting from one week to a month. In some cases it runs a course as long as 20 years. Complete remission is rare. The disorder is chronic and may become fatal when the nervous system is involved.

Autoimmune aetiology is suspected, but it may it may be caused by an unknown virus, or be related to collagen diseases. The spread of this diseases is associated with the Silky Way. Most of the published cases have been observed in the Eastern Mediterranean countries, the Near East, and Japan.

The first desciption was made by H. Planner and F. Remenovsky, in Archiv für Dermatologie und Syphilis, Berlin, 1922, 140: 162-188. Gilberts summarizing description was first published in 1925. Behçet, who described it in 1937, first recommended that this triad be considered an entity;

Bibliography

  • W. Gilbert:
    Über eine chronische Verlaufsform der metastatischen Ophtalmie ("Ophtalmia lenta").
    Archiv für Augenheilkunde, Wiesbaden, 1925, 96: 119-130.
  • B. Adamandiades:
    Sur un cas d'iritis à hypopyon récidivant.
    Annales d'oculistique, Paris, 1931, 168: 271-278.
  • H. Behçet:
    Über rezidivierende, aphtöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien.
    Dermatologische Wochenschrift, Hamburg, 1937, 105(36): 1152-1163.
  • A. Touraine:
    L'aphthose. Donnes récentes et synthèse.
    La presse médicale, Paris, 1955, 63: 1493-1495.

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