acute hemorrhagic edema of infancy

The typical clinical picture is edema on the cheeks, auricles, and extremities along with purpuric skin lesions.{{cite journal | last=Karremann | first=Michael | last2=Jordan | first2=Alexander J. | last3=Bell | first3=Nellie | last4=Witsch | first4=Michael | last5=Dürken | first5=Matthias | title=Acute Hemorrhagic Edema of Infancy: Report of 4 Cases and Review of the Current Literature | journal=Clinical Pediatrics | publisher=SAGE Publications | volume=48 | issue=3 | date=September 3, 2008 | issn=0009-9228 | doi=10.1177/0009922808323113 | pages=323–326}} It has a violent onset, a brief and benign course, and recovers spontaneously after 1 to 3 weeks.{{cite journal | last=SARACLAR | first=Y | last2=TINAZTEPE | first2=K | last3=ADALIOLU | first3=G | last4=TUNCER | first4=A | title=Acute hemorrhagic edema of infancy (AHEI)—A variant of Henoch–Schönlein purpura or a distinct clinical entity? | journal=Journal of Allergy and Clinical Immunology | publisher=Elsevier BV | volume=86 | issue=4 | year=1990 | issn=0091-6749 | doi=10.1016/s0091-6749(05)80202-7 | pages=473–483| doi-access=free }} Mild fever has been reported in the majority of patients.{{cite journal | last=Smitt | first=J.Henk Sillevis | last2=Vermeer | first2=Maarten H | last3=Faber | first3=William R | title=Acute hemorrhagic edema of infancy (AHEI) | journal=Clinics in Dermatology | publisher=Elsevier BV | volume=20 | issue=1 | year=2002 | issn=0738-081X | doi=10.1016/s0738-081x(01)00232-2 | pages=2–3}}

AHEI typically begins with palpable hemorrhagic skin lesions and petechiae, which can progress to medallion-like lesions 1 to 6 cm in diameter. The rashes are usually sharply edged, and the centers of the iris-like lesions are rarely normal skin color. The extremities, including the ears, chin, eyelids, malar region, and scrotal area, are particularly affected. The trunk is usually unaffected.{{cite journal | last=Da Silva Manzoni | first=Ana Paula Dornelles | last2=Viecili | first2=Josiane Burmann | last3=De Andrade | first3=Cristiane Benvenuto | last4=Kruse | first4=Ricardo Lapa | last5=Bakos | first5=Lucio | last6=Cestari | first6=Tania Ferreira | title=Acute hemorrhagic edema of infancy: a case report | journal=International Journal of Dermatology | publisher=Wiley | volume=43 | issue=1 | date=December 24, 2003 | issn=0011-9059 | doi=10.1111/j.1365-4632.2004.01820.x | pages=48–51}} It has been described as a bullous variation with tense hemorrhagic blisters.{{cite journal | last=Lai-Cheong | first=J. E. | last2=Banerjee | first2=P. | last3=Hill | first3=V. | last4=Kenny | first4=P. | last5=Ross | first5=J. | title=Bullous acute haemorrhagic oedema of skin in infancy | journal=Clinical and Experimental Dermatology | publisher=Oxford University Press (OUP) | volume=32 | issue=4 | year=2007 | issn=0307-6938 | doi=10.1111/j.1365-2230.2007.02355.x | pages=467–468}} Lesions, particularly those on the ears, can become necrotic and leave a scar.{{cite journal | last=Legrain | first=Valérie | last2=Lejean | first2=Sylvie | last3=Taïeb | first3=Alain | last4=Guillard | first4=Jean-Michel | last5=Battin | first5=Jacques | last6=Maleville | first6=Jean | title=Infantile acute hemorrhagic edema of the skin: Study of ten cases | journal=Journal of the American Academy of Dermatology | publisher=Elsevier BV | volume=24 | issue=1 | year=1991 | issn=0190-9622 | doi=10.1016/0190-9622(91)70002-j | pages=17–22}}

Edema primarily affects the extremities, especially the backs of the hands and feet. It is frequently asymmetric and begins distally. It can spread to the forearms and legs, but it can also appear on the face, eyelids, earlobes, and even the scrotum. Edema can be painful. AHEI can sometimes appear without fever or edema but without purpura.

• Cutaneous small-vessel vasculitis
• Henoch–Schönlein purpura

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Category:Vascular-related cutaneous conditions