Skip to Main Content

At a glance

It is a 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. Complications include severe dehydration with cardiovascular shock, sepsis, pneumonia, and ultimately multiple organ failure. It is often lethal in children with a mortality rate established between 20 and 50%.

Synonyms

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.

Incidence

This medical condition combined with the Steven Johnson Syndrome affect 1 to 2 million people per year worldwide. It is more common in females than males. Individuals with Human Immunodeficiency Virus positive (HIV-positive) have an increased risk of developing this medical condition and Steven Johnson Syndrome of 1,000 times when compared to the general population. The reason for this increased risk remains unknown. The onset is typically seen in individuals older than 40 years. Patients that survive usually grow skin over 2 to 3 weeks and full recovery usually take months and most are left with chronic problems.

Genetic inheritance

Although it is not a genetic syndrome, nevertheless there are certain genetic factors that seems associated with increased risk of toxic epidermal necrolysis (TEN). It was reported that some human leukocte antigen (HLA-types) have been linked to this medical condition.

Pathophysiology

Hypersensitivity reaction in response to medications. The most common associated with this medical condition are sulfonamides, allopurinol, carbamazepine, penicillins, nevirapine, barbiturates, and phenytoin. Infections with herpes simplex, mycoplasma pneumonia, cytomegalovirus, staphylococcal, viral, and fungal infections are established. There are risks with HIV/AIDS (Human Immunodeficiency Virus/Acquired Immune Deficiency Syndrome) and systemic lupus erythematosus. Malignant diseases, radiation, or vaccination have also been implicated.

Diagnosis

Primarily based on skin lesion appearance and symmetrical distribution in the context of history of risk factors or associated diseases. A positive Nikolsky sign (ie, separation of the center layer of the epidermis from the basal layer with sloughing of the skin produced by minor trauma) is an indication of the disease. Skin biopsy occasionally is useful to exclude other skin disorders and may show significant deposits of immune complexes. A skin biopsy associated with skin involvement greater than 30% are confirming the diagnosis. It is called a Steven Johnson Syndrome when less than 10% of the skin is involved and established as an intermediate form with 10 to 30% of the skin is involved.

Clinical aspects

A development of multiple and large blisters (bullae) that coalesce and result in sloughing ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.