Erythema exsudativum multiforme (EEM) is an inflammatory rash on the skin or mucosa. Because of the visual similarity of the rosette-shaped skin foci to military cockades, erythema exsudativum multifome is also called cocard erythema and the foci are called gunshot lesions.
What is erythema exsudativum multiforme?
In erythema exsudativum multiforme, annular skin lesions appear on patients’ skin, often with a purple center surrounded by a bluish halo. Between them, a pale ring is visible, like a cockade or shooting target. Often the foci appear like juxtaposed coins, singly or in groups. There are two forms, differing in severity and course. In the mild form , EEM Minor, patients usually do not feel ill. The cocardiform foci are mainly found on the skin of the back of the hand and the extensor side of the forearm. There is no or only weak blistering, the mucous membranes are not affected. In the severe manifestation (EEM Major), the general condition of the patient is deteriorated. The entire body including feet, palms and oral mucosa may be affected by rash, blisters have formed in the center of the foci. The transition to Stevens-Johnson syndrome is smooth, a variant of EEM major with severe skin involvement of the entire body. Here, the mucous membranes of the mouth, eyes or genitals are also affected. The risk of secondary infection is high, and often scars remain after the erythema heals.
Causes
Many erythema exsudativum multiforme conditions follow infection with herpes simplex virus. Fragments of herpes simplex DNA are thought to cause a cell-damaging reaction in cells of the epidermis. However, this is rare compared to the total number of herpes simplex infections. Therefore, an additional genetic predisposition is assumed. Other viral or bacterial infections, mycoses, vaccines, preservatives or certain medications are also listed among the causes. Antibiotics, so-called hydantoins (partly contained in antiepileptic drugs) or pyrazolones (partly contained in painkillers) are suspected to cause erythema exsudativum multiforme. In the EEM minor form, the trigger cannot be determined in 60 percent of cases. If the herpes simplex virus is the cause, a chronic form with recurrent episodes may develop. EEM Major, on the other hand, occurs as a one-time disease – almost always drug-induced, due to cell-toxic side effects (cytotoxic reaction).
Symptoms, complaints, and signs
Erythema exsudativum multiforme often appears unexpectedly to the patient. A rash of spots, papules, wheals, or blisters suddenly forms on the skin, at skin level, somewhat raised, or eaten deeper into the skin. The lesions resemble cockades or shooting discs with a purple center and bluish outer ring. In the major form, the rash often spreads from the extremities to the trunk. Vesicles on the palate and gums or shooting disk lesions on the lips are also noticeable in the mouth. Itching may occur along with the rash. Anyone who has had an infection with herpes simplex virus, first observes a fiber blister on the lips, and then notices a severely itchy rash on the arms and legs should see the doctor. Stevens-Johnson syndrome is accompanied by fever, enlarged lymph nodes, liver and spleen. The mucous membranes are always affected, often preceded by catarrh-like symptoms. Variations in skin manifestations range from a few gunshot lesions to extensive scarlet-like exanthema.
Diagnosis and course
Diagnosis is usually made on the basis of the clinical picture. The history takes into account any infections or mycoses that have been overcome, as well as medication use and its temporal relationship. Without significant antecedents, a rash forms on the extremities or the entire body. The reddish papules initially measure 0.1 to 0.3 centimeters. Within 24 hours, they expand into bullet-disk-shaped cocardes, with central blisters in the major form. The exanthema may affect the backs of the hands, palms, and soles; appear on the neck, face, neck, or extensor sides of the arms; and manifest in clusters around the elbows or knees.Mild (mostly oral) mucosal involvement is present in about 50 percent of cases, on lips, buccal mucosa and tongue. Joint swelling and pain may occur. In the major form, the general condition is markedly impaired. Histologic examinations are not conclusive, the laboratory is usually unremarkable, and occasionally there are elevated inflammatory values, including eosinophilia.
Complications
Erythema exsudativum multiforme often develops after an infectious disease and manifests as an inflammatory rash that may involve the mucous membranes. The cause of the symptom is considered to be a drug reaction, a tumor attack, the outbreak of a herpes simplex virus, as well as an infection by bacteria capable of reproduction or streptococci. The affected person tends to develop blister-like wheals completely unexpectedly, forming a ring-shaped bluish skin reaction, the center of which stands out strongly in purple. The visible appearance is called, among other things, a shooting disc. As the disease progresses, foci of varying size and intense itching develop. Erythema exsudativum multiforme is divided into major and minor forms. Therefore, at the first signs, affected individuals should seek immediate medical attention, especially if a herpes simplex virus has previously caused an outbreak. If medical therapy is not initiated early, complications accumulate and take on chronic proportions. The symptom affects the entire body and manifests itself on the mucous membranes. In the further course, scars form due to scratching of the skin. Fever episodes as well as enlargements of the liver, spleen and lymph nodes are added and there is a smooth transition into the so-called Stevens-Johnson syndrome. Depending on the causative agent of erythema exsudativum multiforme, medical therapy prescribes various countermeasures in the form of anti-inflammatory glucocorticoids as well as skin lotions and mouth rinses to relieve symptoms.
When should you see a doctor?
Skin rash should always be medically examined and treated. If the affected areas spread or increase in intensity, a visit to the doctor is recommended. If the skin changes mean that general locomotion can no longer take place as usual, a doctor should be consulted. If there is poor posture of the body or pain of the muscles due to a crooked posture, a doctor is needed. Without correction, there is a risk of permanent damage to the skeletal system. If objects can no longer be held as usual due to the discomfort in the hands, or if the general performance limit drops, it is advisable to consult a doctor. If there are changes in the mucous membranes of the mouth or open wounds, a visit to the doctor is necessary. Germs can enter the organism and lead to new diseases. If fever sets in; dizziness or nausea, a doctor is needed. If swelling or skin discoloration occurs, a doctor should examine the affected areas and provide medical care. If the eyes and also the genitals are affected, the symptoms should be clarified by a doctor. If changes in vision set in or psychological problems occur, a doctor is needed. If blistering occurs on the skin for no apparent reason, this is considered unusual and should be investigated.
Treatment and therapy
EEM is an acute self-limited condition and the skin lesions heal on their own within two weeks. Treatment is limited to symptomatic therapy. Good experience has been made with external treatment of the skin lesions with Lotio alba, a shaking mixture of aqueous zinc oxide. Joint swellings are controlled with cool compresses or pads or a local antihistamine. Glucocorticoid-containing creams such as 0.1 percent triamcinolone cream or 0.05 to 1 percent betamethasone emulsion help relieve bothersome itching. For lesions of the oral mucosa, mouth rinses with chamomile extract provide relief. In the more severe major form, systemic glucocorticoids such as prednisone may be prescribed for intensive treatment of the affected mucous membranes. Antibiotic administration to prevent secondary infections may also be advisable. For more severe itching, oral antihistamines such as desloratadine or cetirizine can be used.
Outlook and prognosis
Erythema exsudativum multiforme has a favorable prognosis. The changes in skin appearance occur acutely, triggering the impression of a serious disorder.Ultimately, however, the symptoms recede completely within a few weeks. Most patients are symptom-free after less than a fortnight. In addition, they do not require medical assistance to alleviate the symptoms, as the organism regenerates itself independently. If no complications develop due to itching or due to the visual blemish, the healing process is of short duration and can be managed without medical care. The overall prognosis is less favorable as soon as other diseases are present. In many cases, erythema exsudativum multiforme is a concomitant of another disease. Although the skin lesions resolve without any external influence or the administration of medication, there are often complex diseases that need to be treated. The prognosis of these diseases is often unfavorable or protracted. The weaker the patient’s immune system is due to the underlying disease present, the more likely are delays in the healing process of erythema exsudativum multiforme. In addition, the risk of complications is increased. In the case of open wounds, pathogens can enter the organism and trigger sepsis. This poses a potential threat to life. Fever episodes are also possible. They temporarily weaken the patient additionally.
Prevention
If a triggering substance has been identified without doubt, it is sufficient for the patient to avoid it in the future to prevent recurrence of EEM. In patients with frequent recurrences due to herpes simplex virus, suppressive antiviral therapy may help – either short-term or as a permanent therapy, for example with aciclovir.
Follow-up
In most cases of erythema exsudativum multiforme, no special or direct measures or options for aftercare are available to the affected person. In this disease, the affected person is primarily dependent on rapid and early treatment and diagnosis so that there is no further worsening of the symptoms. As a rule, the disease cannot heal on its own, so a visit to a doctor is always necessary. In the case of erythema exsudativum multiforme, the affected person should in any case rest and take care of his body. In this regard, efforts or other stressful and physical activities should be refrained from, so that no further complications occur. The affected person should also take the medication prescribed by the doctor regularly and in the correct dosage. The doctor should be consulted if there are any questions or other uncertainties. Regular examinations of the inflammation by a doctor are also important in this regard, in order to always monitor and control the current status. If the treatment of erythema exsudativum multiforme is successful, there is usually no reduction in life expectancy. In this case, no further follow-up measures are necessary.
What you can do yourself
EEM is an acute condition that must first be evaluated by a physician. Treatment can be supported by those affected by various measures. If it is suspected that a drug is responsible for erythema exsudativum multiforme, the responsible drug must be discontinued. Affected persons should talk to the responsible physician about this. The skin lesions must always be treated with medical drugs. Sometimes the antihistamines used can be supplemented with cooling compresses. Especially in the case of joint swelling, cooling helps, whether in the form of wraps, cooling sprays or compresses. However, the use of such agents must be discussed with a physician beforehand in order to avoid adverse events. In the case of the severe major form, medical treatment is required in any case. Those affected can support the therapy by taking care of the mucous membranes. Dietary measures, for example, are suitable for this purpose: A diet free of spicy, acidic or overly hot foods and abstaining from stimulants such as coffee or alcohol. If the symptoms persist despite all these measures or even increase in intensity, a doctor must be consulted. It is possible that the erythema exsudativum multiforme is based on a serious condition that must be diagnosed and treated.
