Lyell Syndrome

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.

Not a genetic syndrome.

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 complexes.

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 the face mask and the skin is another option. Oral airways should be avoided if possible. Intratracheal lesions do not occur often if intubation proceeds cautiously with a well-lubricated undersized tube. Because endotracheal tube securing may be complicated, suturing the tube in place may be the best choice. Whenever possible, spontaneous ventilation without tracheal intubation or placement of an airway (including a laryngeal mask) is mandatory; use of IV ketamine or volatile agents delivered through a head box (or a gently applied face mask) is recommended when applicable. Because hypothermia may develop rapidly, temperature should be carefully monitored and every measure taken to ensure normothermia. It is mandatory to avoid oropharyngeal suctioning because it can lead to bullae formation and postoperative airway obstruction. Regional anesthesia is not contraindicated if the skin over the region is unaffected, but it is imperative to use a sterile technique because these patients are more prone to infection. Because of the esophageal scarring, there is a danger of regurgitation and aspiration; consequently, antacid prophylaxis is recommended prior to surgery. Infection is more common because of the broken epidermal barrier, which should be treated prior to the patient going to the operating room. Because there is a greater risk of corneal abrasion, lubricant and petroleum jelly gauzes should be applied.

It might be prudent to avoid succinylcholine in case of hyperkalemia caused by acute renal failure and shock.

Epidermolysis Bullosa: Group of skin diseases characterized by recurring painful blisters and open sores, often in response to minor trauma, as a result of the unusually fragile nature of the skin. There are three major forms: epidermolysis bullosa simplex, the most common; junctional epidermolysis bullosa; and dystrophic epidermolysis bullosa. In epidermolysis bullosa simplex (nonscarring), the blisters occur within the epidermis. In the Weber-Cockayne variant, blisters are usually confined to the hands and the feet. The Dowling-Meara variant is characterized by the presence of blisters on the entire body. About one third of all epidermolysis bullosa simplex patients experience blisters and sores within the oral cavity that impair feeding and swallowing. Growth retardation and anemia can be present. Children usually have normal mental and physical development. In junctional epidermolysis bullosa (JEB), the blisters occur deeper within the lamina lucida of the basement membrane zone. Herlitz JEB and non-Herlitz JEB are the two subtypes. Patients present with widespread painful blisters and skin erosions. The skin is very fragile and may peel off easily. Dystrophic epidermolysis bullosa (DEB) is characterized by blisters that develop beneath the basement membrane zone and are present at, or shortly after, birth. Patients have widespread painful blisters and sores. The two major subtypes are dominant DEB and recessive DEB. The Hallopeau-Siemens variant, a recessive form, is the most severe. Blisters appear on the arms and legs and are widespread, affecting mucous membranes and skin. Blisters leave scars and small “seed-like” cysts after healing.

Erythema Multiforme (EM): Characterized by inflammation of the oropharynx and hypopharynx membranes, anogenital region, intestinal tract, and conjunctiva. Macules or papules are present. Bullae or blisters may develop. Abnormalities of the eyes may lead to ocular damage as a result of purulent conjunctivitis.