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Acute Hemorrhagic Edema of Infancy: A Rare Cause of Purpuric Exanthema

Published:October 29, 2014DOI:https://doi.org/10.1016/j.jpeds.2014.09.048
      A 5-month-old girl presented to the emergency department with the acute onset of edema and large, erythematous purpuric plaques on the earlobes, which rapidly progressed to the cheeks and limbs. She had shown mild coryzal symptoms 3 days prior and low-grade fever (38°C). Her birth, developmental, and nutritional history were uncomplicated. There was no history of recent vaccination or drug intake. Physical examination revealed a well-appearing infant with multiple, coalescent, erythematous and purpuric plaques, some target-shaped, on the face and extremities, relatively sparing the trunk, with mild edema (Figure 1). The remaining physical examination was unremarkable. Urinalysis, liver and renal function testing, serum complement, immunoglobulins, clotting tests, and full blood count were normal, and C-reactive protein was negative. Skin biopsy (Figure 2; available at www.jpeds.com) and direct immunofluorescence were performed and confirmed the diagnosis of acute hemorrhagic edema of infancy. Viral serology showed evidence of acute infection by both echovirus and Coxsackie virus (IgM+, IgG−) and reverse transcription polymerase chain reaction of nasal secretions identified rhinovirus. The child received symptomatic treatment only and complete regression occurred at 4 weeks.
      Figure thumbnail gr1
      Figure 1Large, round, purpuric plaques on the face, upper and lower limbs, with sparing of the trunk.
      Acute hemorrhagic edema of infancy is a rare, benign leukocytoclastic vasculitis, the differential diagnosis of which includes erythema multiforme, hemorrhagic urticaria, drug-induced vasculitis, Kawasaki disease, infected eczema, meningococcemia, and child abuse.
      • Fiore E.
      • Rizzi M.
      • Simonetti G.D.
      • Garzoni L.
      • Bianchetti M.G.
      • Bettinelli A.
      Acute hemorrhagic edema of young children: a concise narrative review.
      The etiology is unknown, but there is frequent association with acute infection, recent vaccination, or drug intake.
      • Fiore E.
      • Rizzi M.
      • Simonetti G.D.
      • Garzoni L.
      • Bianchetti M.G.
      • Bettinelli A.
      Acute hemorrhagic edema of young children: a concise narrative review.
      • Fotis L.
      • Nikorelou S.
      • Lariou M.S.
      • Delis D.
      • Stamoyannou L.
      Acute hemorrhagic edema of infancy: a frightening but benign disease.
      • Jindal S.R.
      • Kura M.M.
      Acute hemorrhagic edema of infancy— a rare entity.
      Because there are no specific initial laboratory tests, history and physical examination provide essential clues for the successful recognition of the disease. Treatment is symptomatic and complete recovery usually occurs within 6-21 days.
      • Fiore E.
      • Rizzi M.
      • Simonetti G.D.
      • Garzoni L.
      • Bianchetti M.G.
      • Bettinelli A.
      Acute hemorrhagic edema of young children: a concise narrative review.
      • Poyrazoglu H.M.
      • Per H.
      • Gunduz Z.
      • Dusunsel R.
      • Arslan D.
      • Narin N.
      • Gümüş H.
      Acute hemorrhagic edema of infancy.

      Appendix.

      Figure thumbnail gr2
      Figure 2Mixed infiltration of the dermis by lymphocytes and neutrophils, with leukocytoclasis and eosinophils with perivascular, perianexial, and interstitial distribution. No fibrinoid necrosis is present.

      References

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        • Rizzi M.
        • Simonetti G.D.
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        Acute hemorrhagic edema of young children: a concise narrative review.
        Eur J Pediatr. 2011; 170: 1507-1511
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        Acute hemorrhagic edema of infancy: a frightening but benign disease.
        Clin Pediatr (Phila). 2012; 51: 391-393
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        • Kura M.M.
        Acute hemorrhagic edema of infancy— a rare entity.
        Ind Dermatol Online J. 2013; 4: 106-108
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        • Per H.
        • Gunduz Z.
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        • Gümüş H.
        Acute hemorrhagic edema of infancy.
        Pediatr Int. 2003; 45: 697-700

      Linked Article

      • Small for gestational age children have specific food preferences
        The Journal of PediatricsVol. 166Issue 6
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          Oliveira et al1 reported increased feeding difficulties in children born small for gestational age detected at 4-6 months of life, compared with children who were born appropriate for gestational age. Although their study did not evaluate food preferences, the authors state: “… it may be that low birth weight condition (as a proxy of in utero growth restriction) programs the appetite and satiety mechanisms, and ultimately influences the desire for specific foods”. Our group has shown that this is indeed the case.
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