Acute haemorrhagic oedema of infancy

Acute haemorrhagic oedema of infancy (hemorrhagic edema with American spelling) was originally described by Snow in the USA in 1913. Finkelstein described it in Europe in 1938 and it has been recognised in the European literature under various terms since: Finkelstein Disease, Seidlmayer syndrome, infantile post-infectious iris-like purpura and oedema, purpura en cocarde avec oedema.

What is acute haemorrhagic oedema of infancy?

Acute haemorrhagic oedema of infancy is a rare type of cutaneous vasculitis with a characteristic presentation.

It consists of a clinical triad of:

• Large bruise-like lesions (purpura)
• Swelling (oedema)
• Fever

Skin biopsy reveals a leukocytoclastic vasculitis (this means there are broken-up white cells involved with inflamed small blood vessels).

There is uncertainty whether acute haemorrhagic oedema of infancy is a mild variant of Henoch-Schoenlein Purpura (HSP) that occurs in infancy, or a distinct clinical entity. Clinically it is similar to but milder than HSP, but occurs in a more restricted age range, and has different skin lesions. Histopathologic findings are identical to HSP. However, the pattern of antibody staining on direct immunofluorescence of a skin biopsy is different to HSP. In HSP, IgA deposition occurs, but IgA is found in only one third of patients with haemorrhagic oedema.

Who is affected?

Acute haemorrhagic oedema generally develops in children between the ages of 4 months and 2 years of age.

What causes it?

The cause is unknown. It is an immune mediated process, possibly an immune complex disorder. Immune complexes are made up of aggregates of antibodies and the particles that these antibodies are directed against.

Many possible triggers for this immune mediated disease have been reported.

What are the clinical features?

Acute haemorrhagic oedema of infancy is frequently preceded by a prodromal illness such as a viral upper respiratory tract infection. The striking characteristic features appear as described in the table below; however the child remains in good general health.

Finkelstein disease or acute haemorrhagic oedema of infancy

How is it diagnosed?

Acute haemorrhagic oedema of infancy is usually diagnosed on clinical grounds alone. Other causes of purpura may first need to be excluded, as well as rashes that have a similar cockade pattern, like erythema multiforme, urticaria and Kawasaki disease. Inflicted injury should also be considered.

How is it treated?

No treatment is required as it resolves spontaneously. Systemic steroids do not alter the disease course.

Acute haemorrhagic oedema of infancy usually resolves spontaneously over 1-3 weeks with complete recovery. Recurrence may occur but is uncommon, and usually occurs early.