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A previously healthy 12 month old girl presented with a one week history of coryzal symptoms and a 24 hour history of an unusual rash. Initially, her right ear had appeared bruised, but over 12 hours both ears became progressively more swollen, protruding, and discoloured (figs 1⇓ and 2⇓). The rash on her limbs and face was initially papular, then the lesions increased in number and size, becoming annular and purpuric (fig 3⇓). There was some swelling of her top lip and a right subconjunctival haemorrhage. There was no respiratory difficulty, abdominal discomfort, or joint pain. She was normotensive and urinalysis was negative. Full blood count and clotting screen were normal. There was no further progression of the rash, but 72 hours later she developed tense oedema of both hands and feet. The rash and oedema resolved completely within two weeks.
This presentation is typical of acute haemorrhagic oedema of infancy, which was first described in 1913,1 but has been rarely reported in the English language literature.2 It is characterised by the sudden appearance of sharply demarcated, annular, purpuric lesions, limited almost entirely to the face and limbs and often accompanied by oedema and low grade fever. It is frequently preceded by respiratory tract infection, drug intake, or vaccination, and typically affects children between 4 and 24 months of age.3 Systemic symptoms such as abdominal pain, gastrointestinal bleeding, arthritis, and nephritis are rarely reported. Spontaneous and complete resolution occurs within one to three weeks although recurrences are not uncommon. There is no specific therapy. Systemic corticosteroids do not alter the disease course. The histological findings in skin biopsy specimens are those of a leucocytoclastic vasculitis. IgA deposition is observed in about one third of specimens.
Acute haemorrhagic oedema of infancy may be considered a mild variant of Henoch-Schönlein purpura, but recognising it as a distinct clinical entity allows an appropriate prognosis to be made for this generally benign disease of infants.2,3