cute 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

1. Acute Haemorrhagic Edema
of Infancy
By
Alan Mathew
TSMU

2. 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.


3. 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.

4. 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

5. 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)

6. 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.

7. Clinical Presentations

8. Rashes and Edema

9. 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

11. 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.

12. 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

13. 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.