Acute hemorrhagic edema of infancy, also known as hemorrhagic edema of childhood, acute hemorrhagic edema of infancy, Finkelstein's disease, Seidlmayer syndrome, Seidlmayer's cockade purpura, infantile post-infectious iris-like purpura and edema, purpura en cocarde avec oedema, or post-infectious cockade purpura, is considered to be an independent disease in Europe and a heteromorphosis of IgA vasculitis in most American.


Causes

Infection, medications, or vaccination may be the trigger for this disease. Patients often have a recent upper respiratory tract infection and antibiotic treatment. The disease is leukocytoclastic vasculitis caused by immune complexes.


Signs and Symptoms

Acute onset is generally present. Edema without tenderness occurs initially on the face or extremities, often asymmetrically. Subsequently, edematous petechiae, ecchymoses, and large, annular, nummular, or target purpura occur on the head, face, ears, and distal extremities, especially on the basis of edema, and may progress into bullae or necrotic lesions. Skin lesions occur initially on the distal extremities, head, and face, developing toward the proximal areas, sometimes involving the scrotum. Patients may have low fever, but often have no septicemia. Articular, gastrointestinal, and renal involvement is rare. Skin lesions subside spontaneously in 1 - 3 weeks, leaving no sequelae, generally without recurrence.

Figure 1 acute hemorrhagic edema of infancy

Figure 2 acute hemorrhagic edema of infancy

Figure 3 acute hemorrhagic edema of infancy

In most patients, ESR can be slightly elevated, and circulating immune complexes can be increased in few patients.


Histopathology

The skin lesion is leukocytoclastic vasculitis of small blood vessels, has many similarities with IgA vasculitis. However, the incidence of IgA deposition around the blood vessels is only 10% - 35%.


Diagnosis

According to the typical skin lesions, the disease can be diagnosed.


Treatment

There is no special treatment. Systemic glucocorticoids and antihistamines do not change the duration of the disease, and patients with infection can be treated with antibiotics.

Most patients recover in 1 - 3 weeks even without treatment.