Acute hemorrhagic edema of infancy - is it really a mild, benign disease?

Background: Acute hemorrhagic edema of infancy (AHEI) is a rare vasculitis, which presents dramatically as palpable purpuric skin lesions on the limb, face and auricles, with swelling of these parts and low-grade fever, in children up to 2 years of age. To date, approximately 400 cases have been described in the literature. The etiology remains mostly unknown. With or without treatment, AHEI goes to spontaneous recovery within 1-3 weeks, usually without any complications. To our knowledge, compartment syndrome as complication of AHEI has only been reported in one case. We present an unusual case of AHEI with serious complications due to compartment syndrome of the right-hand fingers.

Case: A 16-month-old male child presented with fever and sudden appearance and rapid spread of palpable, painless, non-itching ecchymotic hematomas on the thigh, cheeks, earlobes, forearms, dorsum of hands and feet, with mild edema of these regions. Complete systemic examination and all vital parameters were normal for age. There was no history of bleeding disorders in the family. Except low hemoglobin on complete blood count and increased D-dimer values, all other laboratory investigations were in the normal range. Changes on the right forearm and hand expanded on almost the entire dorsal side and all surfaces of the fingers, with pronounced swelling and formation of bullous lesions, which were spreading and cracking. Skin biopsy confirmed nonspecific small-vessel vasculitis. That required the use of Methylprednisolone, low-molecularweight heparin, antibiotics and debridement of necrotic eschar, with necrectomy of the affected fingers.

Conclusions: Early recognition of AHEI is important to avoid unnecessary investigation and therapy. On the other hand, our reported case warns that unexpected complications may occur.