Stevens-Johnson syndrome

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A syndrome characterized by severe erythema multiforme-like eruption of the skin and lesions of the oral, genital and anal mucosa, and haemorrhagic crusting on the lips, associated with fever, headache and arthralgia.

Description

A syndrome characterized by severe erythema multiforme-like eruption of the skin and lesions of the oral, genital and anal mucosa, and haemorrhagic crusting on the lips, associated with fever, headache and arthralgia. The macular eruptions are usually on extremities, appearing in successive eruptions of short duration. No itching, burning, or rheumatic pains. May appear in separate rings, concentric rings, disk-shaped patches, distributed elevations, and figured arrangements. A very long list of symptoms and signs affecting the eyes, the gastrointestinal tract, etc. It affects all ages, with highest incidence in first or third decades, rarely in infants, or adults over 50 years of age. Prevalent in men. The more severe forms of the disorder occur in boys and young adults. It can be associated with malignant disease and systemic lupus erythematosus. Attacks usually come during the spring and fall.

The syndrome has been classified into two forms. Erythema multiforme exudativum minor is a mild form characterized primarily by cutaneous and oromucosal lesions, while erythema multiforme exudativum major is a severe form characterized by fever, and ocular, genital, cutaneous, and oral mucosal lesions.

Some authors believe this syndrome to be merely a severe form of Hebra’s erythema multiforme exudativum.

Stevens and Johnson reported two patients, boys aged 7 and 8 years.

Bibliography

  • Ferdinand von Hebra (1816-1880):
    Das umschriebene Eczem. Eczema marginatum.
    In: Virchow’s Handbuch der speciellen Pathologie und Therapie. Erlangen, 1860, 1, 1 Abt., pp. 361-363.
  • E. Fuchs:
    Herpes oris conjunctivae.
    Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1876, 14: 333-351.
  • R. Rendu:
    Sur un syndrome caractérisé par l’inflammation simultanée de toutes les muqueuses externes (conjunctivale, nasale, linguale, buccopharyngée, orale et balanopréputiale) coexistant avec une éruption varicelliforme puis purpurique des quatre mebres.
    Rev Gen Clin Ther, 1916, 30: 351-358.
  • N. Fiessinger, R. Rendu:
    Sur un syndrome caractérisé par l’inflammation simultanée de toutes les muqueuses externes coexistant avec une éruption vésiculeuse des quatre membres, non douloureuse et non recidivante.
    Paris médical, 1917, 25: 54-58.
  • A. M. Stevens, F. C. Johnson:
    A new eruptive fever associated with stomatitis and ophthalmia; report of two cases in children.
    American Journal of Diseases of Children, Chicago, 1922, 24: 526-533.
  • E. Baader:
    Dermatostomatitis.
    Archiv für Dermatologie und Syphilis, Berlin, 1925, 149: 261-268.
  • J. V. Klauder:
    Ectodermosis pluriorificialis. Its resemblance to the human form of foot and mouth disease and its relation to erythema multiforme.
    Archives of Dermatology and Syphilology, Chicago, 1937, 36: 1067-1077.

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