Recurrent hemifacial oedema

Description

A 3-year-old boy, with no relevant family/personal history, presented at the emergency department due to recurrent left hemifacial erythematous oedema and feverish peak. The mother reported two similar episodes in the previous months, interpreted as periorbital cellulitis, with resolution with systemic antibiotic and oral corticosteroid. She denied fever, weight loss or other constitutional symptoms.

At examination presented skin paleness; soft, non-tender swelling and erythema of the left periorbital region (Figures 1); shotty, non-tender, well defined, smooth generalised lymphadenopathy (cervical, submaxillary, axillary, inguinal); firm and non-tender splenomegaly palpable 2 cm below the costal margin. Ophthalmological examination and funduscopy were normal.

Figure 1.

Oedema and erythema of the left periorbital region.

Laboratory tests revealed a normocytic and normochromic anaemia (haemoglobin of 8.4 g/dL), leucocytosis (28100 WBC/μL) with 77% of lymphocytes, 137.000/μL platelets, normal liver function enzymes and negative serology for Epstein-Barr virus. Peripheral blood smear revealed 44%...

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