Acute Hemorrhagic Edema of Infancy ; D69.0

Rare clinical picture associated with cocardiform or annular, hemorrhagic plaques in early childhood. A hemorrhagic variant of erythema exsudativum multiforme or a variant of Schönlein-Henoch purpura have been discussed in the past. There is an increasing tendency to regard this disease not as a hemorrhagic variant of purpura rheumatica, but as an independent entity, even if overlaps between the two clinical pictures can be observed between the ages of 2-4 years.

An infection with Mycoplasma pneumoniae is often detectable; the clinical picture can therefore be interpreted as an "infectious allergic reaction".

Rota or Coxsackie viruses (Ferrarini A et al. 2018) are less frequently detected as triggering factors.

Ferreira O et al. (2011) describe the clinical picture after H1N1 immunization.

For infants and toddlers aged 4 to 24 months.

Onset of symptoms usually with only mild febrile temperatures and largely undisturbed general condition. Skin symptoms consist of facial or acral oedema involving the backs of the hands, followed by a petechial, sometimes painful exanthema and further, 1-3 cm large, circular, partly disc-like configured (hence the name "cocardenpurpura"), deep to blue-red, urticarial plaques; possibly widespread. Rarely blistering or lesional necrosis. Involvement of internal organs (kidney, GI, joints) is seen in about 8.5% of affected children.

Laboratory findings are non-specific and not diagnostically relevant, but may show mild leukocytosis, lymphocytosis, thrombocytosis or elevated inflammatory markers. Coagulation tests, liver function tests and kidney function tests are normal.

Evidence of leukocytoclastic vasculitis of small vessels; deposits of IgA in the vessel walls are detected in around 25% of patients (Shah P et al. 2021).

S.a.u. Leukocytoclastic vasculitis.

In about 25% of patients, IgA deposits are found in the vessel walls.

Schönlein-Henoch purpura: systemic involvement is rare in AHEI (<10% of children with AHEI), which distinguishes AHEI from Henoch-Schönlein purpura (HSP), which usually presents with nausea, vomiting, abdominal pain and renal involvement (Shah P et al. (2021). HSP is an IgA-mediated disease, biopsies of HSP lesions show IgA deposits, which are found in only 25% of cases of childhood acute hemorrhagic edema (Fiore E et al. (2008). HSP occurs at an older age, has systemic findings and shows predominantly purpura on the buttocks and lower extremities compared to AHEI (Shah P et al. 2021).

Drug exanthema

Erythema exsudativum multiforme

Urticaria, acute.

Acute hemorrhagic edema of infancy is a benign condition that resolves on its own after 1 to 3 weeks. The likelihood of symptoms recurring in these patients is only 5 to 10 %. The clinical picture is similar to other systemic diseases such as Henoch-Schönlein purpura (HSP), meningococcemia, Kawasaki disease, erythema multiforme and acquired coagulopathy, so it is important for primary care physicians to make this diagnosis.

11-month-old girl with no history of infection.

Clinical picture: Suddenly appearing, less symptomatic, symmetrical, hemorrhagic plaques on both cheeks. On the lower legs there were 2.0-4.0 cm large, deep red, partly annular, partly flat plaques. The ears were conspicuously edematous, swollen and reddened. There was a slight fever of up to 38 °C and mild diarrhea. The child's AZ was not significantly impaired.

Diagnostics: Stool examination: evidence of rotavirus infection, leukocytes 9500/μl, ESR: 30/60; CRP slightly elevated, Haemoccult pos. alpha 2-globulin elevated, other laboratory findings unremarkable.

Course: 2 weeks after the onset of the disease, there was a spontaneous decrease in symptoms.

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