Acute hemorrhagic infantile edema D69.0

Rare clinical picture in early infancy associated with coccardiac hemorrhagic plaques. A hemorrhagic variant of the erythema exsudativum multiforme or a variant of the purpura Schönlein-Henoch are discussed. There is an increasing tendency to understand 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 at the age of 2-4 years.

In most cases, an infection with Mycoplasma pneumoniae is detectable; therefore, the clinical picture can be interpreted as an infectious allergic reaction to mycoplasma infection.

Less commonly, rotaviruses or coxsackie viruses (Ferrarini A et al. 2018) are shown to be causative factors.

Ferreira O et al (2011) describe the disease pattern following H1N1 immunization.

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.

Evidence of leukocytoclastic vasculitis of small vessels with deposits of IgA in the vessel walls in about 1/3 of patients.

See also leukocytoclastic vasculitis.

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

• 11-month-old girl with no history of infection.
• Clinic: Suddenly developing, less symptomatic, symmetrical hemorrhagic plaques on both cheeks. On the lower legs deep red plaques of 2.0-4.0 cm in size, partly anular, partly flat. The ears were strikingly edematous, swollen and reddened. There was low fever up to 38 °C and slight diarrhoea. The child's AZ was not significantly restricted.
• Diagnosis: Faecal examination: evidence of a rotavirus infection, leukocytes 9500/μl, BSG: 30/60; CRP slightly elevated, Haemoccult pos. alpha 2-globulin elevated, other laboratory inconspicuous.
• Course: 2 weeks after onset of the disease there was a spontaneous decrease of symptoms.

1. Caksen H et al (2002) Report of eight infants with acute infantile hemorrhagic edema and review of the literature. J Dermatol 29: 290-295
2. Di Lernia V et al (2004) Infantile acute hemorrhagic edema and rotavirus infection. Pediatric Dermatology 21: 548-550
3. Ferrarini A et al (2018) Acute hemorrhagic edema of infancy associated with coxsackie virus infection. Arch Pediatr 25: 244.

4. Ferreira O et al (2011) Acute hemorrhagic edema of childhood after H1N1 immunization. Cutan Ocul Toxicol 30:167-169.

5. Fiore E et al. (2008) Acute hemorrhagic edema of young children (cockade pupura and edema). A case series and systematic review. J Am Acad Dermatol 59: 684-695
6. Legrain V et al (1991) Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol 24: 17-22
7. Paradisi M et al (2001) Infantile acute hemorrhagic edema of the skin. Cutis 68: 127-129
8. Ruhrmann G (1977) Cocard purpura. Hemorrhagic variant of erythema exsudativum multiforme secondary to mycoplasma pneumoniae infection? Pediat Prax 19: 37-40
9. Seidlmayer H (1940) The early infantile, postinfectious cocard purpura. Z Pediatrics 61: 217
10. Vandeghinste N et al (1992) The acute hemorrhagic edema in the infant. Dermatologist 43: 786-88