Description

Acute hemorrhagic edema of infancy (AHEI) is a rare disorder that follows a benign course.


 

Synonyms: Finkelstein's disease, Seidlmayer's disease, postinfectious cockade purpura

 

Clinical features:

(1) age of the patient is usually from 4 to 24 months

(2) recent antigenic stimulus (see below)

(3) skin lesions (see below)

(4) low-grade fever

(5) variable peripheral non-pitting edema which may be tender

(5) otherwise asymptomatic and nontoxic

(6) recovery in 6 to 21 days

(7) occasionally may involve recurrent episodes

 

Antigenic stimulus may include:

(1) viral infection (echovirus, enterovirus, coxsackie, rhinovirus, etc)

(2) mycoplasma infection

(3) bacterial infection

(4) vaccination

(5) drug therapy

 

Skin lesions:

(1) tend to involve the face and extremities while sparing the trunk

(2) may be erythematous macules, papules or plaques, urticaria, purpura, targetoid lesions

 

Laboratory findings:

(1) variable leukocytosis, usually mild if present

(2) variable elevation ESR and C-reactive protein

(3) normal serum IgA

 

A biopsy shows leukocytoclastic vasculitis. Immunofluorescence usually shows C1q deposition but is negative for IgA deposits.

 

Differential diagnosis:

(1) Henoch Schonlein purpura

(2) erythema multiforme

(3) fixed drug eruption

(4) meningococcemia

(5) Sweet's syndrome

(6) urticarial vasculitis

 


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