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Erythema multiforme is an acute mucocutaneous syndrome defined entirely by its clinical features. The lack of distinctive histological findings and biological markers contribute to the confusion surrounding its cause.
Since the 1950s, it has been customary to consider erythema multiforme majus (with multipolar mucosal erosions), Stevens-Johnson syndrome and pluriorificial erosive ectodermosis to be one and the same entity, although the two latter disorders were clearly distinguished from erythema multiforme by the authors who originally described them.
A return to the original clinical classification has been suggested with, on the one hand, erythema multiforme minor and major with its distinctive target-like lesions, acral distribution and probable infectious cause and, on the other, Stevens-Johnson syndrome and Toxic Epidermal Necrolysis which are drug-induced and present as erythematous macules which never have the target-like appearance described above, converging to form more or less extensive plaques with a predominantly central distribution on the chest and at the tops of the limbs. Based on the clinical appearance of the skin lesions, this classification does not take into account the forms involving only or primarily the mucosa. It appears logical to refer to these cases, which closely resemble erythema multiforme majus, as pluriorificial erosive ectodermosis (Fuchs syndrome in Germany).
Erythema multiforme is a rare condition but its incidence has not been precisely calculated. It is more common in children and young adults, with a frequency peak between the ages of 20 and 30 and a slight male predominance.
INFORMATION FOR PATIENTS
• Mechanisms : Erythema multiforme is a harmless condition that most often arises as a "reaction" to an infection, especially the herpes virus. A flare sometimes requires a few days in hospital to alleviate pain and other symptoms. No truly effective treatments currently exist but a flare tends to be self-limiting within 2 to 3 weeks. If the disorder is linked to recurrent herpes infection, then further flares are possible.
• Patient compliance and monitoring : if EM recurs, the doctor may prescribe a preventive treatment. This will only be effective if taken regularly, every day, for at least 6 months. The treatment is cumbersome and is therefore only truly of interest for patients experiencing regular, debilitating flares.
• Risks for family and friends : for unknown reasons, only a very small percentage of people with a recurrent form of herpes will present with erythema multiforme. Erythema multiform is not contagious or passed from person to person. Herpes, however, is contagious and can be dangerous for children with atopic dermatitis or immunocompromised adults.
• Impact on social life and work : there is usually little impact. Only the – thankfully rare - recurrent or subintrant forms can affect quality of life. The treatments available for these cases are quite effective.
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