Acute hemorrhagic infantile edema D69.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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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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Mostly an infection with mycoplasma pneumoniae is detectable, therefore the clinical picture can be interpreted as an infectious allergic reaction to mycoplasma infection. Rarely rotaviruses are detected as triggering factors.

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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Beginning of symptoms with mostly only low febrile temperatures and largely undisturbed general condition. Skin symptoms consist of facial or acral edema affecting the backs of the hands, followed by a petechial, sometimes painful exanthema and further, 1-3 cm large, circular, partly shooting-disc-like configured (hence the name "cockade purpura"), deep to blue-red, urticarial plaques; possibly extensive spreading. Rare blistering or lesional necroses. In about 8.5% of children with the disease internal organs (kidney, GI, joints) are involved.

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

Histology
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Detection of leukocytoclastic vasculitis of small vessels with deposits of IgA in the vessel walls in about 1/3 of patients. S.a. Leucocytoclastic 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. 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
  4. Legrain V et al (1991) infantile acute hemorrhagic edema of the skin: Study of ten cases. J Am Acad Dermatol 24: 17-22
  5. Paradisi M et al (2001) Infantile acute hemorrhagic edema of the skin. Cutis 68: 127-129
  6. Ruhrmann G (1977) Cocard purpura. Hemorrhagic variant of erythema exsudativum multiforme as a consequence of mycoplasma pneumoniae infection? Pediatric practice 19: 37-40
  7. Seidlmayer H (1940) The early infantile, post-infectious cockade purpura. Z Pediatrics 61: 217
  8. Vandeghinste N et al (1992) The acute hemorrhagic edema in infants. Dermatologist 43: 786-88

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Last updated on: 29.10.2020