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.