Erythema multiforme is a skin disorder resulting from an allergic reaction.
Alternate Names: Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis, Lyell?s Syndrome
Stevens-Johnson syndrome has similar skin lesions with the additional involvement of at least two mucous membranes, and fever. The appearance of the mucosal lesion is erythema and edema, which progresses to erosions and pseudo-membrane formation. In a review of the mucosal involvement of 34 patients with Stevens-Johnson, 100% had stomatitis, 86% had ocular involvement, 41% had genital mucosal or urethral involvement, but only 3% had involvement of the anal mucosa. Although not reported in this series, upper airway mucosal involvement and pneumonia may be seen in up to 30% of cases. In addition to the target lesions, virtually all patients will develop a characteristic maculo-papular rash, usually early in the disease. Prodromal symptoms, such as fever, malaise, and cough are sometimes reported as a feature, and they usually occur seven to ten days prior to full-blown presentation. The majority of cases of erythema multiforme and Stevens-Johnson syndrome are between ages 20 and 40, and 20% of cases occur in children and adolescents. The mortality of Stevens-Johnson syndrome is reported as 3 to 19%.
Toxic epidermal necrolysis (TEN)
It is distinguished by larger body surface area involvement, and the development of bullae. The epidermis of the skin peels off in sheets greater than 3 cm, and the skin becomes tender within 48 hours. TEN should be distinguished from staphylococcal scalded skin syndrome. In the largest retrospective series of TEN in the literature, the mean age was 45, although there were several cases in children. Body surface area involved was 47%, and 43% of survivors had some permanent sequelae, most of which were ocular (including three cases of blindness), or permanent skin pigmentary changes. The most common cause of death was sepsis, mostly from Staph aureus or Pseudomonas aeruginosa infections. The mortality rate is 30% to 70%.
Small-vessel vasculitis syndromes
Lupus erythematosus, pemphigus
Pemphigoid, epidermolysis bullosa
It is a type of hypersensitivity (allergic) reaction that occurs in response to medications, infections, or illness.
Medications associated with erythema multiforme include sulfonamides, penicillins, barbiturates, and phenytoin.
Associated infections include herpes simplex and mycoplasma infections.
The exact cause is unknown. The disorder is believed to involve damage to the blood vessels of the skin with subsequent damage to skin tissues. Approximately 90% of erythema multiforme cases are associated with herpes simplex or Mycoplasma infections. The disorder occurs primarily in children and young adults.
Patients are often given antibiotics for an infection, and it is difficult to determine whether the antibiotic or the infection was responsible for the disease. Viral upper respiratory infections, Mycoplasma pneumonia, pharyngitis and Herpes simplex infection are also reported to cause erythema multiforme. The list of other possible etiologies is extensive, and includes systemic lupus erythematosus, histoplasmosis, pregnancy, malignancy and external-beam radiation. In most series, some cases remain idiopathic. Several authors have postulated an immunologic etiology for erythema multiforme, although no one has been able to demonstrate conclusively the pathogenesis of erythema multiforme.
It may present with a classic skin lesion with or without systemic (whole body) symptoms. In Stevens-Johnson syndrome, the systemic symptoms are severe and the lesions are extensive, involving multiple body areas (especially the mucous membranes). Toxic epidermal necrolysis (TEN syndrome, or Lyell?s syndrome) involves multiple large blisters (bullae) that coalesce, followed by sloughing of all or most of the skin and mucous membranes.
With sudden onset, may recur
May appear as nodule, papule, or macule
Central lesion surrounded by concentric rings of pallor and redness (“target”, “iris”, “bull?s eye” shape)
May have vesicles and bullae (blisters of various sizes)
Located on the legs, arms, palms, hands, or feet
May involve the face or lips
Trunk is usually not involved
Itching of the skin may be present
General ill feeling
Additional symptoms that may be associated with this disease:
Eye burning, itching and discharge
Diagnosis & Tests
The diagnostic criteria for erythema multiforme (EM) is individual “target” skin lesions less than 3 cm in diameter, less than 20% of body surface area involved, with minimal mucous membrane involvement, and biopsy compatible with EM. The cutaneous lesions are typically symmetric, and involve the extremities, with the dorsal hands and extensor aspects most commonly involved.
It is primarily based on the appearance of the skin lesion and its typical symmetrical distribution, especially if there is a history of risk factors or associated diseases.
There may be a positive Nikolsky?s sign.
A skin lesion biopsy and microscopic examination may be helpful to differentiate erythema multiforme from other disorders. Erythema multiforme may show tissue death and other changes. Microscopic examination of the tissue may also show antibody deposits.
Treatment includes control of the underlying causes or illnesses, treatment of the symptoms, and prevention of infection. Suspected medications should be discontinued.
Treatment of mild symptoms:
Moist compresses applied to skin lesions.
Medications such as antihistamines to control itching.
Over-the-counter medications (such as acetaminophen) to reduce fever and discomfort.
Topical anesthetics (especially for mouth lesions) to ease discomfort that interferes with eating and drinking.
Treatment of severe symptoms:
Hospitalization and treatment in an intensive care or burn care unit for severe cases, Stevens-Johnson syndrome, and toxic epidermal necrolysis
Systemic corticosteroids to control inflammation
Intravenous immunoglobulins (IVIG) to stop the process
Antibiotics to control secondary skin infections
Good hygiene and isolation from others may be required to prevent secondary infections.
Extensive skin involvement may cause the loss of large quantities of body fluids, causing shock in addition to the risk of infection. Intensive care with support of body systems may be required.
Skin grafting may be helpful in cases in which large areas of the body are affected.
In cases that are caused by the herpes virus, daily antiviral medications may be prescribed to prevent recurrences of erythema multiforme.
Mild forms of erythema multiforme usually resolve without difficulty in 2 to 6 weeks, but they may recur. More severe forms may be difficult to treat. Stevens-Johnson syndrome and toxic epidermal necrolysis are associated with high death rates.
Permanent skin damage and scarring
Occasionally, lesions on internal organs causing:
Pneumonitis (lung inflammation)
Myocarditis (heart inflammation)
Nephritis (kidney inflammation)
Hepatitis (liver inflammation)
Secondary skin infection (cellulitis)
Systemic infection, sepsis
Loss of body fluids, shock
The most common and serious long-term sequelae of Stevens-Johnson and TEN are the ocular complications. The conjunctivitis damages or completely destroys the goblet cells of the conjunctiva, which results in instability of the precorneal tear film, and corneal drying and opacification. The corneal damage can lead to decreased visual acuity and even blindness. The incidence of long-term ocular complications from Stevens-Johnson and TEN are reported at 10 to 27% of patients.
ars., rhus-t., merc., ant-c., sulph., sil., sep., hep., nit-ac. and dulc, stated in order of their importance.
? Dr. Rajneesh Kumar Sharma
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