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Skin Eruptions in Acute Hemorrhagic Edema of Young Children: Systematic Review of the Literature

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eMediNexus    09 October 2021

Acute hemorrhagic edema of young children is also known as cockade purpura with edema or Finkelstein-Seidlmayer disease. It is a skin-limited small-vessel leukocytoclastic vasculitis, which typically follows a simple, mostly viral, febrile illness. 

Infants between 4 weeks to 2 years of age are affected. The lesions spontaneously remit within 3 weeks and sometimes relapse. 

Vascular immunoglobulin A deposits may not be present in the majority of cases, however hemorrhagic edema is frequently seen as the infantile variant of immunoglobulin A vasculitis. A palpable rash mainly affecting the lower extremities and the buttocks is a frequent presentation of hemorrhagic edema.

Evidence suggests that extensive and symmetrically distributed annular or nummular eruptions and edema develop in over 48 h or less, in the affected children. 

Since there is sparse data regarding this vasculitis, a study investigated the type, distribution, and evolution of the rash in this illness and showed-

  • Presence of key features of rash in 383 children.
  • 98% of cases showed Annular eruptions, particularly targetoid (polycyclic or arciform eruptions were also present in many).
  • 93% of cases showed nummular eruptions.
  • A large majority of the cases demonstrated purpuric eruptions and ecchymoses.
  • Lesser cases showed the presence of Macules and wheals.
  • Edema was present in all cases
  • Painful, indurate and non-pitting were the key features of edema.
  • Annular or nummular eruptions mainly affected legs, followed by feet, face, arms, ears, trunk, and genitals.
  • Edema too showed similar distribution, except for feet.
  • The eruption initiated as a wheal or a macule that evolved into a nummular or an annular eruption in most cases.
  • Nummular eruptions later turned to annular ones.

These data on the type, distribution, and evolution of skin eruptions in hemorrhagic edema of young children will aid physicians to make the clinical diagnosis of this apparently alarming but very benign vasculitis rapidly and noninvasively.

Source: Skin Eruptions in Acute Hemorrhagic Edema of Young Children: Systematic Review of the Literature. Dermatology, 2021. doi: 10.1159/000519009

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