BACKGROUND: Acute hemorrhagic edema of infancy (AHEI), or Seidlmayer's disease, is a type of leukocytoclastic vasculitis proper of infants and children. It is characterized by a local increase in temperature, erythematous edema and purpuric lesions involving mainly the face and extremities. There usually is no visceral involvement. The disease is self-limited, bearing a benign clinical course. Infection, drugs and immunization have been considered as precipitating factors. The main differential diagnosis is Henoch-Schönlein purpura (HSP). METHODS: We describe a classic example of acute hemorrhagic edema of infancy, and comment on the clinical features, pathology, immunopathology and proposed therapy. We characterize the differences between AHEI and HSP. RESULTS: A course of corticosteroids was given to avoid quick progression of disease. CONCLUSION: Acute hemorrhagic edema of infancy is a rare disease, and the most striking classic feature of the disease is the contrast between the acuteness of the cutaneous lesions and the good general condition of the patient. Considering its clinical features, pathology and immunopathology, AHEI can be justifiably characterized as a unique disorder, distinct from HSP.
BACKGROUND: Acute hemorrhagic edema of infancy (AHEI), or Seidlmayer's disease, is a type of leukocytoclastic vasculitis proper of infants and children. It is characterized by a local increase in temperature, erythematous edema and purpuric lesions involving mainly the face and extremities. There usually is no visceral involvement. The disease is self-limited, bearing a benign clinical course. Infection, drugs and immunization have been considered as precipitating factors. The main differential diagnosis is Henoch-Schönlein purpura (HSP). METHODS: We describe a classic example of acute hemorrhagic edema of infancy, and comment on the clinical features, pathology, immunopathology and proposed therapy. We characterize the differences between AHEI and HSP. RESULTS: A course of corticosteroids was given to avoid quick progression of disease. CONCLUSION: Acute hemorrhagic edema of infancy is a rare disease, and the most striking classic feature of the disease is the contrast between the acuteness of the cutaneous lesions and the good general condition of the patient. Considering its clinical features, pathology and immunopathology, AHEI can be justifiably characterized as a unique disorder, distinct from HSP.