Acute Haemorrhagic Edema of Infancy

By

Alan Mathew

TSMU

Definition Acute hemorrhagic edema of infancy is a distinctive,

benign, self-limiting disease with cutaneous small vessel leukocyto- clastic vasculitis ,which generally develops in children less than 2 years of age.

Other Terms of AHEI

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.

clinical triad of AHEI

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

Epidemiology Mortality/Morbidity

Acute hemorrhagic edema of infancy (AHEI) is usually benign and without sequelae, with spontaneous recovery occurring within 1-3 weeks. Rare reports have described complications such as arthritis, nephritis,abdominal pain, gastrointestinal tract bleeding, intussusception, scrotal pain, and testicular torsion. AHEI may recur, but this is uncommon. One case report describes an AHEI patient whose eruption resolved with unusual scarring.

Sex

Acute hemorrhagic edema of infancy (AHEI) is more common among male infants than among female infants; the male-to-female ratio is approximately 4.6:1.

Age

Age of onset for acute hemorrhagic edema of infancy (AHEI) usually is 2-60 months (median, 11 mo; mean, 13.75 month)

ETIOLOGY & PATHOGENESIS

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.

Clinical Presentations

Rashes and Edema

Affected Areas

It mainly affects the face and extremities, sparing the trunk and mucous membranes. Some areas may even become necrotic. Visceral involvement is rare, whereas the kidneys and joints are spared

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.

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.

 there are no other diagnostic laboratory examinations, the history and physical examination provide clues to the successful recognition of the disease.

Differential Diagnoses Acute Febrile Neutrophilic Dermatosis Dermatologic Manifestations of Hematologic Disease Drug Eruptions Erythema Multiforme Henoch-Schönlein Purpura (Anaphylactoid Purpura) Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis) Meningococcemia Oral Manifestations of Drug Reactions Purpura fulminans Septicemia Urticaria, Acute

Treatment

No effective therapy exists for acute hemorrhagic edema of infancy (AHEI).

Treatment with oral corticosteroids has been reported, but this is unnecessary due to its spontaneous recovery.

The use of steroids and antihistamines remains controversial, and they do not appear to alter the disease course.

Treatment is symptomatic; discontinue antibiotics after obtaining negative culture results.