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Severe allergic syndrome caused by an allergic reaction (hypersensitivity) to pharmacological agents, infections, or illnesses such as lymphoma and graft-versus-host disease. It is characterized by extensive bullous eruption of the skin and mucous membranes, fever, malaise, conjunctivitis, and diffuse erythema; often lethal in children.

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Acute Toxic Epidermolysis Syndrome; Brocq-Debré-Lyell Syndrome; Bullous Erythroderma Epidermolysis; Debré-Lamy-Lyell Syndrome; Dermatitis Medicamentosa Syndrome; Epidermolysis Acuta Combustiformis; Epidermolysis Combustiformis; Epidermolysis Acuta Toxica; Epidermolysis Necroticans Combustiformis; Erythrodermia Bullosa with Epidermolysis; Fuchs-Salzmann-Terrier Syndrome; Scalded Skin Syndrome; Toxic Epidermal Necrolysis, Toxic-Allergic Epidermal Necrolysis.

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Not a genetic syndrome.

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Hypersensitivity reaction in response to medications (sulfonamides, penicillins, barbiturates, and phenytoin), infections (herpes simplex, mycoplasma, staphylococcal, viral and fungal infections), malignant diseases, radiation, or vaccination.

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Primarily based on skin lesion appearance and symmetrical distribution in the context of history of risk factors or associated diseases. Positive Nikolsky sign (i.e., separation of the center layer of the epidermis from the basal layer with sloughing of the skin produced by minor trauma). Skin biopsy occasionally is useful to exclude other skin disorders and may show significant deposits of immune complexes.

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Development of multiple and large blisters (bullae) that coalesce and result in sloughing of most of the skin and mucous membranes. Associated symptoms may consist of fever, itching, painful joints, visual disorders and pain, and general ill feeling. Lesions of internal organs may occur (pneumonitis, myocarditis, hepatitis, nephritis). Secondary skin infection (cellulitis), septicemia, loss of body fluid (as in extended burns), and shock are common complications. The death rate is high in children and in elderly patients.

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Assess airway patency with regard to the presence of mucosal lesions and bullae. Protect skin lesions with moist compresses. Consider symptomatic treatment to prevent further aggravation: antihistamines to control itching, acetaminophen to reduce fever, pain killer including opiates to treat pain, topical anesthetics (especially for oral lesions) to ease discomfort. Severe cases should be managed in an intensive care or burn care unit. Intravenous corticosteroids (to control inflammation), immunoglobulins (to stop the allergic process), and antibiotics (to control secondary skin infections) must be used. Considerable precaution should be taken to prevent bacterial contamination. Extended skin lesions may cause loss of large quantities of body fluids with shock and require intensive care with support of body systems.

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Prevent any trauma to skin and mucosal surfaces by appropriate positioning and padding. Monitoring should be applied without use of adhesive gels or tapes; the sticky surround of ECG electrodes must be removed. The use of ECG needles might be considered as alternatives. Wrap limb in soft padding prior to use of automated blood pressure recording devices. Venous access may be difficult, and intravenous (IV) cannulas may need to be sutured in place. The pressure from an anesthetic face mask may be damaging, so it should be held lightly just above the face and spontaneous ventilation maintained. The application of vaseline ointment to reduce shearing forces between ...

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