A 12-month-old boy with high fever, erythematous lesions and haemorrhagic oedema

Abstract

A 12-month-old male infant was referred to the hospital with a 3-day history of high fever and erythematous lesions on his face, ears and lower extremities. The lesions appeared initially on the ears and face. The next day, erythematous plaques had spread over his lower extremities. Two weeks previously he had had an upper respiratory tract infection. On admission, the patient was alert with a temperature of 39.2 C. Physical examination revealed oedema of the left leg and large palpable erythematous purpura on the face, ears, and lower extremities. A medallion-like purpura was present on his right leg (Fig.1). The remaining physical examination was normal. Laboratory investigations showed the following: haemoglobin 8.2 g/dl, WBC 11800/mm, platelets 525000/mm, ESR 64 mm/h, C-reactive protein level 2.5 mg/dl, serum creatinine 0.5 mg/dl, BUN 23 mg/dl, total protein 6.4 g/dl, albumin 3.8 g/dl, AST 21 U/l and ALT 24 U/l. Urinalysis, autoimmune profile, complement and clotting screen were normal. Full bacterial and viral screens were unremarkable. Over the next 6 days the palpable erythematous lesions and oedema resolved completely. A diagnosis of acute infantile haemorrhagic oedema (AIHE) was made based upon the presence of fever, large palpable erythematous purpura on the face, auricles and extremities and oedema of the left leg, without organ involvement, normal urinalysis and spontaneous resolution. AIHE is a benign form of leukocytoclastic vasculitis occurring in infants under 2 years of age. Typically, urticarial lesions or small maculopapular rashes appear on the face, ears and extremities, followed by medallionlike purpura. Facial oedema may be the initial sign [2, 3, 4,5]. The most striking feature is the contrast between acuteness and dramatic appearance of the cutaneous lesions with the general good health of the child [1]. The entity has been classified as Henoch-Schonlein purpura (HSP) by some authors due to marked similarities although the age of onset of AIHE is younger as compared to a peak age of 4 to 7 years in HSP. In addition, systemic complications (i.e. arthritis, gastrointestinal and renal involvement) that are common in HSP, are extremely rare in AIHE [3, 4,6]. AIHE that is usually seen in infancy should be distinguished from meningococcaemia, septicaemia, purpura fulminans, Kawasaki disease, erythema multiforme and urticaria in order to avoid unnecessary invasive investigations and therapy [1]. A short course with complete recovery is the rule. Spontaneous and complete resolution occurs within 5 to 14 days, without any complications [1,3]. Our patient also recovered without complications and no recurrence has been observed. B. Acar (&) AE Z. B. Ozcakar AE S. Yuksel AE T. Pekacar M. Ekim AE F. Yalcinkaya Department of Paediatric Nephrology, Ankara University Faculty of Medicine, Ankara, Turkey E-mail: drbanuacar@superonline.com Tel.: +90-312-2210790 Fax: +90-312-3620581