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Erythema Multiforme: Case Report and Discussion

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Erythema Multiforme: Case Report and Discussion J Am Board Fam Pract: first published as 10.3122/15572625-11-1-63 on 1 January 1998. Downloaded from BRIEF REPORTS Erythema Multiforme: Case Report and Discussion Lisa M. Kroonen, MD Erythema multiforme may be placed on a disease tympanic membrane was clear and the right was continuum that includes Stevens-Johnson syn­ dull and erythematous. His sclerae and conjuncti­ drome and toxic epidermal necrolysis. It was first vae were without any lesions. His nose and throat described in 1866 by Hebra as a skin disease with were clear, and there were no lip or oral mucosal symmetrically distributed red papules, evolving lesions. Heart, lung, and abdominal examination within several days to form annular or iris-like results were all unremarkable. His skin had multi­ shapes or blisters. The term multiforme described ple lesions on the extensor surfaces of all extremi­ this evolution of primary lesions into different ties, the chest, the back, and the face. Some of the forms. In 1922 Stevens and Johnson described a lesions were solid raised erythematous areas, and febrile illness with stomatitis, purulent conjunc­ others were target-like lesions with central pallor, tivitis, and skin lesions similar to those of ery­ raised erythematous borders, and surrounding thema multiforme, which has become known as layers of lighter erythema. Stevens-Johnson syndrome or erythema multi­ Complete blood cell count was within normal forme major. l,2 Toxic epidermal necrolysis, with limits, and no other laboratory data were ob­ severe exfoliation of the skin and mucous mem­ tained. The child's condition was diagnosed as branes and a high rate of mortality, is considered erythema multiforme. His mother was told to stop by many sources to be at the most severe extreme the amoxicillin and was given a prescription for of this disease spectrum. cefixime. He was also given a prescription for an oral prednisone solution 1 mglkg. Instructions Case Report were given for the child to return if any oral or oc­ An IS-month-old boy was brought to the emer­ ular lesions were noted, if oral intake ceased, or if gency department with worsening generalized the rash developed any signs of superinfection. body rash. There was no complaint of fever, lack The child was seen 1 week later with good resolu­ of oral intake, itchiness, or ocular involvement. tion of symptoms. Lesions were sparse with mild http://www.jabfm.org/ One week earlier he had been seen for presumed resolving erythema and hyperpigmentation. Dur­ otitis media and given a prescription for amoxi­ ing an examination 1 month later, no skin lesions cillin. Five to 6 days after starting the medication, were observed. he began to develop an erythematous rash that be­ gan on his arms and legs. Two days later the rash Discussion was evident on his palms and soles, as well as trun­ The causes of erythema multiforme and its disease on 6 October 2021 by guest. Protected copyright. cal area. Initially the rash was specifically de­ spectrum are numerous. Herpes simplex virus, scribed as beginning as small areas of redness, Mycoplasma pneumoniae, and drug reactions are the which within days became raised and enlarged and most common. Herpes simplex virus accounts for then developed surrounding lighter layers and more than one half the cases of erythema multi­ central areas of paleness. forme. Both type 1 and 2 infections are associated, Upon examination the child was mildly fussy, and repeated episodes of secondary erythema but consolable. His temperature was 98.8°F, and multiforme associated with recurrent herpes sim­ his blood pressure and pulse were stable. His left plex virus are common. Mycoplasma infection is the most commonly recognized bacterial infection associated with erythema multiforme and can Submitted, revised, 23 September 1997. cause the disease 1 to 3 weeks after the respiratory From the Family Medicine Residency Program, Kaiser illness. Drug-associated erythema multiforme is Fontana, Fontana, Calif. Address reprint requests to Lisa M. Kroonen, MD, Department of Family Medicine. 9985 Sierra common with the use of antibiotics, especially Ave, Fontana, CA 92335. penicillins and sulfonamides. It can be seen 1 to 3 Erythema Multiforme 63 J Am Board Fam Pract: first published as 10.3122/15572625-11-1-63 on 1 January 1998. Downloaded from weeks after drug therapy is started.3 guish this more serious form of disease because it Other less common causes of erythema multi­ can affect the lips, pharynx, larynx, esophagus, forme include vaccinia, bacteria, fungi, drugs, irra­ bronchial tree, kidney, and eyes, therefore leading diation from radiographic studies, malignancy to serious sequelae including dehydration, visceral (carcinomas and lymphomas), and certain colla­ organ damage from mucosal ulcerations, inflam­ gen-vascular diseases (lupus erythematosus, der­ matory renal lesions that could cause an acute matomyositis, and periarteritis nodosa).2,4 glomerulonephritis-like syndrome, and ocular Erythema multiforme is more common in complications that can cause blindness.s,6 Most male patients and in those aged less than 20 years, mucocutaneous lesions tend to heal completely in and it might be related to seasons because there is 2 to 6 weeks, and fatal forms are exceptional (1 higher incidence in some months of provoking percent of cases in one series).5,7 agents, such as mycoplasma and herpes simplex Toxic epidermal necrolysis is the most serious virus infection.5 form on this clinical spectrum, the hallmark of which is Nikolsky's sign (epidermal loss with lat-' Diagnostic Evaluation eral shearing force). Again there tends to be a Because there are serious sequelae involved in the more serious prodromal syndrome and then a most severe manifestations of this disease spec­ burning or painful eruption, located symmetri­ trum, it is important to recognize the clinical signs cally on the face and upper torso. This eruption and symptoms of various forms of the disease so rapidly extends to the entire body, although it pre­ that a prompt diagnosis can be made and a proper dominates on the trunk and proximallimbs.6,8 treatment regimen can be started. Such extensive skin involvement can lead to The characteristic rash of erythema multi­ "acute skin failure," which can result in massive forme is often preceded by nonspecific prodromal fluid loss, a hypercatabolic state, and bacterial col­ symptoms, suggesting an infection of the upper onization of skin resulting in systemic sepsis.9 Mu­ respiratory tract. The hallmark target or iris le­ cous membrane involvement and ocular lesions sions occur 7 to 10 days later, symmetrically on can lead to serious above-mentioned sequelae. Up the extensor surface of limbs, the dorsum of hands to 30 percent of cases can be fata1. 6 and feet, and the palms and soles. Lesions begin as areas of circumscribed erythema with clearly de­ Treatment fined margins, which then become raised. They The progression of these diseases is difficult to grow eccentrically, and after 24 to 48 hours be­ predict, and treatment varies depending on clinical http://www.jabfm.org/ come several centimeters in diameter. Days later severity. Initial measures are termination of possi­ they form a peripheral ring of lighter color and ble inciting medications, fluid replacement, symp­ the center then flattens and becomes a cyanotic tomatic relief of itching and pain, and antibiotics red color, thus assuming a target or iris-like ap­ for mouth and skin lesions with secondary infec­ pearance. Lesion centers can also form blisters tion. \Vhether to use systemic corticosteroids has with peripheral microvessicles (in herpes-related remained the subject of controversy. They can de­ infections). Five to 7 days later the lesions retract crease symptoms and retard the spread oflesions,2 on 6 October 2021 by guest. Protected copyright. and heal completely with only transitory hyper­ but they do not accelerate healing and might in~ pigmentation. Subjective symptoms are limited duce serious complications such as gastrointestinal only to a burning sensation. Prognosis is very bleeding and infection.1O One measure that has re­ good with complete healing in all cases and a re­ duced the recurrence rate of herpes-related ery­ currence rate of22 to 37 percent.s thema multiforme is oral acyclovir.2 Because early In Stevens-johnson's syndrome the prodromal intervention is crucial to preventing sequelae, syndrome is more serious and can include fever, prompt o,Phthalmic and dermatologic consultation asthenia, nausea and vomiting, pharyngitis, and should be sought in severe cases. arthralgia. There is rapid involvement of mucosa, which can inhibit sound production, swallowing, Conclusion and oral intake. Skin lesions are characterized by Erythema multiforme, although usually a self-lim­ lack of symmetry and can involve mucous mem­ ited disease, can be confused with the more severe branes, lips, and genitalia. It is important to distin- disease states on the continuum. Because it can be 64 }ABFP Jan.-Feb.1998 Vol. 11 No.1 J Am Board Fam Pract: first published as 10.3122/15572625-11-1-63 on 1 January 1998. Downloaded from caused by many agents, a detailed history of the 5. Fabbri P, Panconesi E. Erythema multiforme ("mi­ prodromal symptoms and extent of involvement is nus" and "maius") and drug intake. Clin Dermatol necessary to ascertain the severity of illness. Fur­ 1993;11:479-89. 6. Wilkins], Morrison L, White CR]r. Oculocutaneous thermore, a thorough physical examination, pay­ manifestations of the erythema multiformelStevens­ ing attention specifically to distribution of lesions Johnson syndrome/toxic epidermal necrolysis spec­ and associated symptoms, is essential to determine trum. Dermatol Clin 1992;10:571-82 . what treatment regimen should be applied. Treat­ 7. SchopfE, Stuhmer A, Rzany B, Victor N, ZentgrafR, ment measures are generally supportive, although Kapp]E Toxic epidermal necrolysis and Stevens­ the use of corticosteroids remains controversial. Johnson syndrome.
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