Acute Infantile Hemorrhagic Edema and Henoch–Schönlein Purpura Overlap in a Child

Edema hemorrágico agudo da infância

“…9 Perivascular IgA deposits are not usually found in direct immunofluorescence, but there are reports that they may be positive in one third of the cases. 10,11 Therefore, the clinical characteristics, age and progression of the disease assist in making the differential diagnosis, and the absence of IgA deposits is not required for diagnosis.…”

“…1,3 Reports of atypical cases with characteristic findings of both diseases have been considered overlapping of IAHE and HSP. 1,10 In normal neonates, IgA levels are very low at birth and remain low during childhood, increasing progressively until adolescence. 12 For some authors, the absence of perivascular IgA in the IAHE could result from immaturity of the immune system in producing IgA.…”

“…Thus, the IAHE would not be a distinct condition, but a HSP variant that occurs in younger children, and the differences between the two diseases would be the result of a developing immune system. 10 There is no specific treatment for the IAHE. 1,5 The use of systemic corticoids and anti-histamines does not change the course of the disease.…”

Acute Hemorrhagic Edema of Infancy

“…Actually, newly added reports of AHEI in English literature between 2008 and 2010 (Alp et al, 2009;Babic et al, 2008;Cicero, 2008;Ferraira et al, 2010;Fiore et al, 2008;Halıcıoglu et al, 2010;Jain & Patel, 2008;Javidi et al, 2008;Kumar et al, 2008;Karremann et al, 2009;Obeid et al, 2008;Slutsky et al, 2010;Sites et al, 2008) with the review by AlSufyani (AlSufyani, 2009) make the mean age approximately 16.56 months instead of 13.75 months reported by Alsufyani (Alsufyani, 2009). Indeed, there have been some reports of cases occurring beyond the age of 3 years; however, these cases have the features that overlap with those of Henoch-Schönlein purpura (Legrain et al, 1991;Shah et al, 2002).…”

“…In the review of Fiore and colleagues, general appearance was reported as severely reduced in 1.0%, mildly reduced in 7.0% and very satisfactory in remaining 92% (Fiore et al, 2008). Additionally, atypical presentations such as painful edema (Dubin et al, 1990;Gonggryp & Todd, 1998;Krause et al, 1996;Lantner & Simon, 1996;Millard et al, 1999;Morrison & Saulsbury, 1999;Shah et al, 2002), itching (AlSufyani, 2009;Caksen et al, 2002), irritability (Crowe & Jonas, 1998;Kuroda et al, 2002;Lantner & Simon, 1996) and decreased appetite (Lantner & Simon, 1996) may occur. Fever is typically low grade, but may range from mild to severe and also afebrile cases have been published in the literature (AlSufyani, 2009;Amitai et al, 1993;Dubin et al, 1990;Fiore et al, 2008;Gonggryp & Todd, 1998;Karreman et al, 2009;Krause et al, 1996;Saraclar et al, 1990;Scaramuzza et al, 1997;Serna et al, 1994).…”