Recurrent acute hemorrhagic edema of infancy (AHEI) during puberty

Abstract

A 13-year-old girl presented with a 12-month history of intermittent edema and erythema of the extremities, which had been occurring at eight-week intervals. Clinical examination revealed subcutaneous edema with polycyclic violaceous hemorrhagic plaques (reminiscent of targetoid lesions) on her forearms, hands, and feet (Figures 1, 2 ). In addition, there was right palpebral edema and swelling of the cheek, which gave way to non-blanchable erythema within one day ( Figure 3 ). Each fl are had been preceded by an upper respiratory infection 1-2 days earlier. A throat swab had shown normal fl ora. During one episode, a urinary tract infection

Nomenclature of cutaneous vasculitides – German translation of the dermatologic addendum to the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides

“…Not yet mentioned in the D-CHCC is acute hemorrhagic edema of childhood, a small-vessel vasculitis affecting postcapillary venules mostly in infants. The condition seems to be different from cutaneous IgA vasculitis with respect to the location of affected vessels (not only in the upper dermis, but also in the septa of the subcutis) and its sites of predilection (no preference of lower legs) [7].…”

Nomenklatur der kutanen Vaskulitiden – deutschsprachige Definitionen des Dermatologischen Anhanges zur Chapel Hill Consensus Conference

“…Nicht aufgenommen wurde das akute hämorrhagische Ödem der Kindheit , bei dem es sich um eine Vaskulitis der kleinen Gefäße, in Sonderheit der postkapillären Venolen, handelt, die sich bezüglich der Lokalisation der betroffenen Gefäße (offenbar nicht nur hauptsächlich in der oberen Dermis, sondern auch in den Septen der Subkutis) und der Prädilektionsstellen (nicht an den Unterschenkeln) von der kutanen IgA-Vaskulitis unterscheiden mag [ 7 ].…”