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Acute hemorrhagic edema of infancy (AHEI)

      Introduction

      Acute hemorrhagic edema of infancy (AHEI) is a benign variant of leukocytoclastic vasculitis occurring in children up to 2 years of age. Although it was first described by Snow
      • Snow I.M.
      Purpura, urticaria, and angioneurotic edema of the hands and feet in a nursing baby.
      in the United States in 1913, it was not before the eighties
      • Saraclar Y.
      • Tinaztepe K.
      • Adalioglu G.
      • Tuncer A.
      Acute infantile hemorrhagic edema (AIHE) (medaillon-like purpura of nursing baby).
      and nineties
      • Dubin B.A.
      • Bronson D.M.
      • Eng A.M.
      Acute hemorrhagic edema of childhood an unusual variant of leucocytoclastic vasculitis.
      ,
      • Hirschel-Scholz S.
      • Hunziker N.
      Acute hemorrhagic edema of the infant (Finkelstein’s disease).
      ,
      • Legrain V.
      • Lejean S.
      • Taı̈eb A.
      • et al.
      Infantile acute hemorrhagic edema of the skin study of ten cases.
      of the twentieth century that more attention in English literature was given to the disease. Beforehand, cases were mainly reported in the French literature.
      • Gerbeaux J.
      • Hebert-Jouas J.
      Sur une observation d’oedème aigu hémorrhagique de la peau.
      ,
      • Nouaille J.
      • Gautier M.
      • Lucet P.
      Un cas de vascularite allergique àa type d’oedème aigu hémorrhagique de la peau avec manifestations renales.
      ,
      • Fontan A.
      • Battin J.J.
      • Alberty J.
      • et al.
      L’oedème cutané hémorrhagic du nourisson.
      ,
      • Laugier P.
      • Hunziker N.
      • Reiffers J.
      • et al.
      L’oedème aigu hémorrhagique du nourisson (purpura en cocarde avec oedème).
      Still, it is an important disease to recognize because of its very impressive clinical presentation combined with a benign outcome and prognosis.

      Clinical picture

      The prodromal period is, in 75% of the cases, characterized by viral (eg, mostly upper respiratory tract infections but also otitis media
      • Pride H.B.
      • Maroon M.
      • Tyler W.B.
      Ecchymoses and edema in a 4-month-old boy.
      ,
      • Cox N.H.
      Seidlmayer’s syndrome postinfectious cockade purpura of early childhood.
      or conjunctivitis,
      • Legrain V.
      • Lejean S.
      • Taı̈eb A.
      • et al.
      Infantile acute hemorrhagic edema of the skin study of ten cases.
      ,
      • Krause I.
      • Lazarov A.
      • Rachmel A.
      • et al.
      Acute haemorrhagic oedema of infancy, a benign variant of leucocytoclastic vasculitis.
      ) or bacterial infection (eg, streptococcal,
      • Sanchez del Rio J.
      • Marin M.C.
      • Martinez D.N.
      • et al.
      Edema agudo hemorragico del lactante.
      ,
      • Snoussi N.
      • Strobel M.
      • Heid E.
      • et al.
      Oedème aigu hhémorragique du nourisson démonstration d’une vascularite allergique dermique.
      staphylococcal
      • Laugier M.P.
      Oedème aigu hhémorragique (purpura en cocarde avec oedème).
      pharyngitis, pulmonary tuberculosis,
      • Seidlmayer H.
      Die frühinfantile, postinfektiöse Kokarden-Purpura.
      ,
      • Gonggryp L.A.
      • Todd G.
      Acute hemorrhagic edema of childhood (AHE).
      bronchopneumonia,
      • Saraclar Y.
      • Tinaztepe K.
      • Adalioglu G.
      • Tuncer A.
      Acute hemorrhagic edema of infancy (AHEI)—a variant of Henoch-Schönlein purpura or a distinct clinical entity?.
      or urinary tract infection
      • Gonggryp L.A.
      • Todd G.
      Acute hemorrhagic edema of childhood (AHE).
      ), vaccination (eg, measles, diphtheria-pertussistetanus,
      • Pride H.B.
      • Maroon M.
      • Tyler W.B.
      Ecchymoses and edema in a 4-month-old boy.
      or combined
      • Legrain V.
      • Lejean S.
      • Taı̈eb A.
      • et al.
      Infantile acute hemorrhagic edema of the skin study of ten cases.
      ), or drug intake (eg, penicillin,
      • Legrain V.
      • Lejean S.
      • Taı̈eb A.
      • et al.
      Infantile acute hemorrhagic edema of the skin study of ten cases.
      cephalosporins,
      • Legrain V.
      • Lejean S.
      • Taı̈eb A.
      • et al.
      Infantile acute hemorrhagic edema of the skin study of ten cases.
      ,
      • Pride H.B.
      • Maroon M.
      • Tyler W.B.
      Ecchymoses and edema in a 4-month-old boy.
      trimethoprimsulfamethoxazole,
      • Ince E.
      • Mumcu Y.
      • Suskan E.
      • et al.
      Infantile acute hemorrhagic edema a variant of leukocytoclastic vasculitis.
      paracetamole,
      • Vandeghinste N.
      • Naeyaert J.-M.
      • Geerts M.-L.
      • Kint A.
      Das akute hämorrhagische Ödem beim Säugling.
      cough syrup,
      • Dubin B.A.
      • Bronson D.M.
      • Eng A.M.
      Acute hemorrhagic edema of childhood an unusual variant of leucocytoclastic vasculitis.
      or a combination of these), suddenly followed by, at first, red macules or urticarial lesions later followed by impressive, more or less symmetrically distributed, large (1–5 cm) ecchymotic purpuric lesions, characteristically in a cockade pattern, and painful, nonpitting edema. In some instances, petechiae or reticulated purpura may be seen. The lesions may become necrotic especially on the ears. The face, eg, the ears, eyelids, and cheeks, the scrotal area, and the extremities are most commonly affected. The trunk and mucous membranes are usually spared. The skin eruption may be accompanied by low- to high-grade fever in an otherwise not very ill child, usually showing no concomitant symptoms. Rare cases of gastrointestinal vasculitis in one case followed by an intussusception
      • Larrègue M.
      • Lorette G.
      • Prigent F.
      • Canuel C.
      Oedème aigu hémorhagique du nourisson avec complication lethale digestive.
      with fatal consequences have been reported. Transitory renal problems, eg, microscopic hematurian or mild proteinuria, have been infrequently described with spontaneous recovery within days to 3 weeks
      • Legrain V.
      • Lejean S.
      • Taı̈eb A.
      • et al.
      Infantile acute hemorrhagic edema of the skin study of ten cases.
      ,
      • Snoussi N.
      • Strobel M.
      • Heid E.
      • et al.
      Oedème aigu hhémorragique du nourisson démonstration d’une vascularite allergique dermique.
      ,
      • Lambert D.
      • Laurent R.
      • Bouilly D.
      • et al.
      Oedème aigu hémorragique données immunologiques et ultrastructurales.
      The eruption spontaneously disappears without sequelae within 2–3 weeks, but relapses may occur during healing of the original lesions or occasionally later in the follow-up, even up to 15 months
      • Laugier M.P.
      Oedème aigu hhémorragique (purpura en cocarde avec oedème).
      after the first sign of the disease. Only necrotic areas may leave a scar.

      Histopathology and immunofluorescence

      Histopathological analysis demonstrates a leukocytoclastic vasculitis of the dermal vessels with fibrinoid necrosis, extravasation of red blood cells, and leukocytoclasia. Direct immunofluorescence examination shows vascular deposits of C1q, C3, and fibrinogen in all cases.
      • Snow I.M.
      Purpura, urticaria, and angioneurotic edema of the hands and feet in a nursing baby.
      Immunoglobulin (Ig) deposits in the vessels were found in the following percentages: IgA in 30%; IgG in 20%; IgM in 80%; and IgE in 30%.
      • Snow I.M.
      Purpura, urticaria, and angioneurotic edema of the hands and feet in a nursing baby.

      Laboratory investigations

      Apart from a mild leukocytosis, lymphocytosis, thrombocytosis, or rarely an eosinophilia, no consistent laboratory findings have been reported.

      Differential diagnosis

      The differential diagnosis of acute haemorrhagic edema of infancy includes Henoch-Schönlein purpura (HSP), meningococcaemia, erythema multiforme, urticaria with haemorrhagic elements, Kawasaki disease, skin lesions in septicemia, or a drug eruption. The following diagnostic criteria for AHEI were suggested:
      • Krause I.
      • Lazarov A.
      • Rachmel A.
      • et al.
      Acute haemorrhagic oedema of infancy, a benign variant of leucocytoclastic vasculitis.
      • An age of < 2 years.
      • Purpuric or ecchymotic targetlike lesion, with edema of the face, auricles, and extremities, with or without mucosal involvement.
      • Lack of systemic disease or visceral involvement.
      • Spontaneous recovery within a few days or weeks.
      Using these characteristics, most diseases thought of in the differential diagnosis are easily ruled out. The most difficult distinction is that between AHEI and HSP. To date, it is not clear whether these two are different diseases or whether HSP behaves more benignly in infancy.

      Treatment and follow-up

      The benign course justifies investigative and therapeutic reserve, but a firm and reassuring attitude towards the parents is needed.
      • Hirschel-Scholz S.
      • Hunziker N.
      Acute hemorrhagic edema of the infant (Finkelstein’s disease).
      Although some reported patients were treated with oral corticosteroids or antihistamines, these interventions are not necessary, because the spontaneous recovery occurs within a relative short time period. Of course, therapeutic interventions for the prodromal symptoms may be needed.

      References

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        • Canuel C.
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