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".

Rotaviruses 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 non-diagnostic, 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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