Report of Eight Infants with Acute Infantile Hemorrhagic Edema and Review of the Literature

Abstract

Acute infantile hemorrhagic edema (AIHE) is a cutaneous leukocytoclastic vasculitis, clinically characterized by the symptom triad of fever, large purpuric skin lesions, and edema. The clinical picture has a violent onset, a short benign course, and spontaneous complete recovery. In this article, we present eight patients who were admitted with rashes on the skin and edema on the eyelids and extremities, and were diagnosed with AIHE according to their clinical and histopathological features (immunohistological study was also performed in three of them). Our purpose was to emphasize that, aside from Henoch-Schönlein purpura, meningococcemia, septicemia, and purpura fulminans, AIHE benign disorder should also be considered in the differential diagnosis to determine the clinical course and treatment protocol in patients with purpuric rashes.

Acute Hemorrhagic Edema of Infancy

“…This probably supports the role of infections and immune complexes in the pathogenesis of AHEI. Other infections described as prodromes that have been accused in the pathogenesis are upper respiratory tract infections (Alp et al, 2009;Babic et al, 2008;Blasini et al, 2007;Caksen et al, 2002;Dubin et al, 1990;Fiore et al, 2008;Gelmetti et al, 1985;Halıcıoglu et al, 2010;Ince et al, 1995;Karremann et al, 2009;Kumar et al, 2008;Lai-Cheong JE et al, 2007;McDougall et al, 2004;Michael, 2006;Millard et al, 1999;Paradisi et al, 2001;Poyrazoglu et al, 2003;Saraclar et al, 1990), pharyngitis (Gattorno et al, 1997;Gonggryp & Todd, 1998), tonsillitis (Legrain et al, 1991;Karremann et al, 2009), otitis (Caksen et al, 2002;Crowe et al, 1998;Karremann et al, 2009;Krause et al, 1996;Lantner et al, 1996;Macea et al, 2003), conjunctivitis (Legrain et al, 1991), neck abscess, bronchopneumonia (Alp et al, 2009;Saraclar et al, 1990), bronchitis (Karremann et al, 2009), pneumococcal bacteremia (Morrison et al, 1999), pulmoner tuberculosis (Gonggryp & Todd, 1998), cellulitis (Can et al, 2006), urinary tract infections (Saraclar et al, 1990) and gastroenteritis (Caksen et al, 2002;…”

“…Classically histological examination shows features of an intense dermal leucocytoclastic vasculitis involving small blood vessels in the dermis with (Acun et al, 2006;AlSufyani, 2009;Blasini et al, 2007;Braun-Falco & Dietrich Abeck, 2002;Caksen et al, 2002;Calıskan et al, 1995;Can et al, 2006;Chatproedprai & Wananukul, 2007;Colantonio et al, 1997;Crowe & Jonas, 1998;Cunningham et al, 1996Cunningham et al, , 1999Bozaykut et al, 2002;da Silva Manzoni et al, 2004;Di Lernia et al, 2004;Dubin et al, 1990;Fiore et al, 2008;Fujimura et al, 2001;Garty et al, 2006;Gelmetti et al, 1985;Goraya & Kaur, 2002;Gonggryp & Todd, 1998;Gattorno et al, 1997;Javidi et al, 2008;Krause et al, 1996;Kuroda et al, 2002;Lakshmi & Srinivas, 2003;Lantner et al, 1996;Lee et al, 2006;Long & Helm, 1998;Macea et al, 2003;Michael, 2006;Millard et al, 1999;Morrison & Saulsbury, 1999;Paradisi et al, 2001;Poyrazoglu et al, 2003;Pride et al, 1995;Roh et al, 2004;Tomac et al, 1996;…”

“…This is probably because, the most papers on the subject being written different languages in the world and AHEI is called Henoch-Schönlein purpura in the United States. Although most of the prints were in case reports (Babic et al, 2008;Can et al, 2006;Di Lernia et al, 2004;Garty et al, 2002;Michael, 2006;Millard et al, 1999;Macea et al, 2003;Obeid et al, 2008;Serna et al, 1994, Silveira & Braganca, 2006, case series were also published (Alp et al, 2009;Caksen et al, 2002;Fiore et al, 2008;Gelmetti et al, 1985;Gonggryp & Todd, 1998;Ince et al, 1995;Karremann et al, 2009;Krause et al, 1996;Legrain et al, 1991;Poyrazoglu et al, 2003;Saraclar et al, 1990;Sites et al, 2008). Today, AHEI is considered as a separate entity, but not a variant of Henoch-Schönlein purpura, with clinical findings and prognosis.…”

Edema agudo hemorrágico da infância: relato de três casos

“…1 It was initially described in the United States by Snow, in 1913, under the title of "Purpura, urticaria and angioneurotic edema of the hands and feet in a nursing baby". 2 Since then, some cases have been also reported in Europe, as Finkelstein's Disease and Seidlmayer Syndrome. It is rarely reported in American literature, probably because in the United States AHEI is called Henoch-Schönlein Purpura (HSP) in infants.…”

“…2 Pediatrician Infectologist at the Children's Hospital (Hospital Infantil Nossa Senhora da Glória -HINSG) -Vitória (ES), Brazil. 3 Pathologist at the Children's Hospital (Hospital Infantil Nossa Senhora da Glória -HINSG) -Vitória (ES), Brazil.…”