Duodenojejunitis: is it idiopathic or is it Henoch-Schönlein purpura without the purpura?

Henoch-Schönlein purpura is a small-vessel vasculitic disease that most often affects the skin. Gastrointestinal manifestations have been well described, including duodenojejunal inflammation (DJI).

Four children with DJI and clinical features of HSP are described, in whom the rash was either not present or appeared atypically late in the illness.

The characteristic rash did not develop in three children, and it developed much later in one. The patients (three boys and one girl) were aged between 7 and 9 years (mean, 7.5 years). Growth characteristics were normal. In all patients, pain occurred acutely with colicky abdominal pain in the spring or fall of the year, and all stools were positive for occult blood. No infectious cause was identified. Upper gastrointestinal endoscopic examinations demonstrated significant visual and histologic duodenitis in a pattern consistent with previous reports in children with known HSP. Factor XIII activity was absent. Immunoglobulin A levels were increased in three of four children. All children made a prompt recovery with the administration of intravenous glucocorticoids. In one child, the characteristic rash of HSP developed 18 weeks after the initial examination.

Duodenojejunal inflammation may be the primary manifestation of HSP, even in the absence of the characteristic rash.