Ectodermosis erosiva pluriorificialis
More often called the Stevens-Johnson syndrome (SJS), this is a systemic (bodywide) disease with a characteristic rash involving the skin and mucous membranes, including the buccal mucosa (inside of the mouth). The disease is due to a hypersensitive (allergic) reaction to one of a number of immunologic stimuli including drugs and infectious agents. SJS is sometimes fatal. The skin rash of SJS consists of erythematous (red) papules, vesicles, bullae. There may also be iris lesions. The mucosal lesions include conjunctivitis as well as oral and genital ulcers. The most frequent complications of SJS are keratitis, uveitis, and perforation of the globe of the eye — all of which may result in permanent visual impairment. In addition, hepatitis, nephritis, gastrointestinal bleeding, pneumonia, arthritis, arthralgia, fever, and myalgia can be part of SJS. The differential diagnosis includes other diseases that can result in cutaneous and mucous membrane lesions. Diseases such as pemphigus vulgaris, erosive lichen planus, and varicella zoster may mimic SJS. Behcet syndrome and Reiter syndrome may have ocular and genital lesions that can be confused with those seen in SJS. The diagnosis of SJS is usually made when the characteristic rash appears 1 to 3 weeks after exposure to a known stimulus and it cannot be explained by another diagnosis. The treatment of SJS depends, in part, on the suspected precipitating cause. Any drug, such as one of the sulfa drugs, penicillin, or an anticonvulsants, especially phenytoin, should be discontinued. Infectious agents such as herpes simplex virus 1 or 2, or Mycoplasma pneumoniae, should be identified and appropriately treated. The most commonly prescribed medication for SJS is corticosteroids. SJS is also called erythema multiforme (or erythema multiforme majus) and the Johnson-Stevens syndrome.

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Stevens-Johnson syndrome.

Medical dictionary. 2011.

Look at other dictionaries:

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  • Ectodermose pluriorrificielle — Syndrome de Stevens Johnson Syndrome de Stevens Johnson CIM 10 : L51.1 Le syndrome de Stevens Johnson (erythema exsudativum multiforme ou ectodermosis erosiva pluriorificialis ou dermatostomatite ou ectodermose érosive pluriorificielle de… …   Wikipédia en Français

  • Syndrome de Stevens-Johnson — Classification et ressources externes Personne atteinte du syndrome de Stevens Johnson. CIM 10 …   Wikipédia en Français

  • Syndrome de stevens-johnson — CIM 10 : L51.1 Le syndrome de Stevens Johnson (erythema exsudativum multiforme ou ectodermosis erosiva pluriorificialis ou dermatostomatite ou ectodermose érosive pluriorificielle de Fiessinger Rendu) est une maladie orpheline grave, de survenue… …   Wikipédia en Français

  • Erythema multiforme — This entry on erythema multiforme pertain specifically to the Stevens Johnson syndrome (SJS). It is a systemic (bodywide) disease with a characteristic rash involving the skin and mucous membranes, including the buccal mucosa (inside of the… …   Medical dictionary

  • Johnson-Stevens syndrome — More often called the Stevens Johnson syndrome (SJS), this is a systemic (bodywide) disease with a characteristic rash involving the skin and mucous membranes, including the buccal mucosa (inside of the mouth). The disease is due to a… …   Medical dictionary

  • Stevens-Johnson syndrome (SJS) — A systemic (bodywide) disease with a characteristic rash involving the skin and mucous membranes, including the buccal mucosa (inside of the mouth). The disease is due to a hypersensitive (allergic) reaction to one of a number of immunologic… …   Medical dictionary

  • Syndrome, Johnson-Stevens — More often called the Stevens Johnson syndrome (SJS), this is a systemic (bodywide) disease with a characteristic rash involving the skin and mucous membranes, including the buccal mucosa (inside of the mouth). The disease is due to a… …   Medical dictionary

  • Syndrome, Stevens-Johnson (SJS) — A systemic (bodywide) disease with a characteristic rash involving the skin and mucous membranes, including the buccal mucosa (inside of the mouth). The disease is due to a hypersensitive (allergic) reaction to one of a number of immunologic… …   Medical dictionary

  • Síndrome de Stevens-Johnson — El síndrome de Stevens Johnson (también conocido como ectodermosis erosiva pluriorificialis, eritema multiforme exudativo, y eritema multiforme flictenular), es una patología severa que afecta piel y mucosa, además de otros órganos internos, y… …   Enciclopedia Universal

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