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.
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.
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.
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
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.
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.
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 ...