Incomplete Kawasaki disease represents a diagnostic challenge for pediatricians. In the absence of classical presentation, the laboratoristic evaluation of systemic inflammation can help in placing the correct diagnosis to promptly start adequate therapy. Erythema multiforme is an acute, self-limiting condition considered to be a hypersensitivity reaction commonly associated with various infections or medications. This aspecific skin condition has been rarely described as a sign of Kawasaki disease. We report on the case of a 4 years old boy presenting high-grade fever associated with erythema multiforme and evidence of systemic inflammation who showed a good response to prompt treatment with intravenous immunoglobulins.
A 4 years old boy was admitted to our Hospital for a one day history of remittent fever (up to 40.0C), accompanied by irritability and annular, slightly itchy rash, started on his hands and feet and progressively extended to the flexor and extensor surfaces of the extremities, with relative sparing of the trunk (Figure 1). The child appeared extremely suffering. Physical examination showed bilateral lymphadenopathy (
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Treatment with intravenous immunoglobulins (2 gr/Kg) and high-dose aspirin was promptly started. After immunoglobulins administration the child's clinical conditions improved with defervescence and reduction in systemic inflammation indexes. After 24 hours the child presented again transient fever up to 39.5C, that responded to paracetamol with final defervescence. Cutaneous lesions progressively faded (Figure 2C). Aspirin dose was reduced to low-dose (5 mg/Kg per day), after the child has been afebrile for 48 hours. As expected, on 10th day blood analysis showed thrombocytosis. On 12th day from the onset of the fever electrocardiogram and cardiac ultrasound were normal, so the child was discharged. Two weeks after the discharge erythema multiforme was completely resolved, no desquamation was observed and flogosis indexes returned into normal range. Low-dose aspirin was maintained until patient shows no evidence of coronary changes by 8 weeks after the onset of illness. Cardiologic follow-up in the next 6 months was normal.
The case we reported is particularly interesting for the unusual presentation of incomplete Kawasaki disease. Indeed, erythema multiforme was reported as a cutaneous manifestation of classic Kawasaki disease in only 2 young children, a 22-month-old girl in 1979 [9] and a 16-month-old boy in 2010 [10]. In addition, other 3 patients were described affected by Kawasaki disease associated to annular lesions [14]. To our knowledge, this is the first report of erythema multiforme as first sign of incomplete Kawasaki disease.
The skin eruption of Kawasaki disease has been described as an erythematous rash usually appearing within 5 days of the onset of fever. The most common is a non specific, diffuse maculopapular eruption. Occasionally some other skin pictures such as urticarial exanthem, scarlatiniform rash, erythroderma, erythema-multiforme-like rash or, rarely, fine micropustular eruption have been described. The rash usually is extensive, with involvement of the trunk and extremities and accentuation in the perineal region, where early desquamation may occur [1]. In our case the rash erupted the first day of fever as annular, slightly itchy cutaneous manifestations that evolved to multiple target-like erythematous lesions compatible with erythema multiforme in 4th day of fever. Erythema multiforme was more appreciable at the extremities, including palms and soles, with relative unusual sparing of the trunk. We did not observed changes in the extremities, neither changes of the lips and oral cavity.
In the case we described there was no development of coronary abnormalities, but electrocardiography showed completely reversible abnormalities in ventricular repolarization. This finding is compatible with Kawasaki disease, where ECG may show arrhythmia, prolonged PR interval, or non specific ST and T wave changes [1]. Finally, we demonstrated previous streptococcus pyogenes infection and we hypothesized it as a trigger for Kawasaki disease development, as previously described [14, 18]. An infectious trigger of Kawasaki disease has been suspected by the epidemiologic features, such as age of affected children, seasonality of cases, and occurrence of community outbreaks and epidemics [1]; however, no known infectious agent has been consistently found [19]. As streptococci are infectious agents associated even with erythema multiforme [20], therefore it is possible to speculate that, in our case, streptococcus pyogenes infection may have been a common trigger underlying both conditions, Kawasaki disease being the end result of an extensive immune activation in a predisposed host.
The paper indicates erythema multiforme as a possible early cutaneous manifestation of Kawasaki disease, particularly in the case of the incomplete form. This acquisition is strongly important in the pediatric practice to avoid delaying in diagnosis and to promptly start adequate treatment.
Erythema multiforme (EM) is a cutaneous and mucosal hypersensitivity reaction with characteristic lesions in target triggered by certain antigenic stimuli. It represents an acute condition, sometimes recurrent, of the skin and mucosal membranes manifested by papular, bullous, and necrotic lesions. Its causes are variable and numerous, and its evolution is generally favorable.This activity reviews the cause, pathophysiology, and presentation of erythema multiforme and highlights the role of the interprofessional team in its management.
Objectives:Recall the cause of erythema multiforme.Describe the presentation of erythema multiforme.Summarize the treatment options for erythema multiforme.Outline the importance of improving care coordination among interprofessional team members to improve outcomes for patients affected by erythema multiforme.Access free multiple choice questions on this topic.
An 8-year-old Japanese girl presented with sequential skin manifestations, including erythema nodosum, erythema multiforme and Henoch-Schönlein purpura. Although a chest radiograph showed no significant lung abnormalities, serological examinations revealed that these skin manifestations were associated with M. pneumoniae infection.
A wide variety of skin manifestations are associated with Mycoplasma pneumoniae infection[1]. It has been reported that the variations in cutaneous manifestations of M. pneumoniae infections can be attributed to the immaturity of the adaptive immunity of a host[2]. However, the precise mechanisms by which M. pneumoniae infection is able to produce a variety of cutaneous manifestations are poorly understood. In this report, we describe the case of a patient with sequentially appearing skin manifestations, including erythema nodosum, erythema multiforme and Henoch-Schönlein purpura, associated with M. pneumoniae infection.
A previously healthy 8-year-old Japanese girl was referred to our hospital with a 2-day history of symmetrical, multiple, round, light pink, tender erythematous nodules on her lower legs and arthralgia of her ankles 13 days before admission (Figure1). Their sizes were approximately 2cm. The diagnosis of erythema nodosum was made. The lesions gradually improved and disappeared 7 days before admission; however, the patient then developed multiple maculopapular and targetoid lesions over her lower legs 6 days before admission (Figure2A). No involvement of the mucous membranes was found. The diagnosis of erythema multiforme was made. The physical examination also revealed tonsillitis. Specific serum immunoglobulin M (IgM) antibodies for M. pneumoniae were positive, and clarithromycin therapy (300mg/day) was started. The lesions gradually improved and disappeared 3 days before admission (Figure1). The patient had a mild cough; arthralgia of both hands; painful, localized edema on her scalp bilaterally; and a purpuric rash over her hip and lower legs concurrently 3 days before admission (Figure2B). She also had abdominal pain and was admitted to our hospital. She was born in Japan and had not traveled out of Japan. She had no animal or insect bites. On admission, her body temperature was 36.8C, heart rate 106 beats/minute and blood pressure 96/50mmHg. She had no weight loss. She had a purpuric rash over her hip and lower legs. The remainder of the examination was normal. Laboratory examinations showed a slightly elevated C-reactive protein level of 0.5mg/dl (normal 2ff7e9595c
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