Acute hemorrhagic infantile edema D69.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 12.12.2021

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Synonym(s)

Acute childhood haemorrhagic edema; Acute hemorrhagic edema of childhood; Acute infantile hemorrhagic edema; Cockade purpura; Hemorrhagic edema of childhood; iris-like pupura; purpura cocard purpura; Seidlmayer cocard purple

History
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Snow, 1913; Finkelstein, 1938; Seidlmayer, 1940

Definition
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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.

Etiopathogenesis
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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.

Manifestation
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In infants and toddlers before the age of 2 years.

Localization
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Face, extremity extensor sides, buttocks.

Clinical features
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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
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Routine diagnostics are not very helpful. Urinalysis and haematology are usually without pathological findings.

Histology
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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.

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

Differential diagnosis
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Therapy
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Externally symptomatic, healing usually within 1-3 weeks, no tendency to recur.

Progression/forecast
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Favorable, no recurrence tendency.

Case report(s)
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  • 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.

Literature
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  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

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Last updated on: 12.12.2021