The Most common systemic vasculitic disease of childhood.

Age: varies from 6 months to adulthood, peak incidence is 2-8 years. Complexes of immunoglobulin A (IgA) and complement component 3(C3) are
deposited on arterioles, capillaries, and venules.

Almost in all cases

Classic rash: palpable purpura

Begins as: maculopapular rash petechiae & purpura. Symmetric, over extensor surfaces & buttocks Lesions occur in cropsand last 3-10days.

Occurs in: 80% of cases. Ankles and knees are the most commonly affected joints. Prominent periarticular swelling which sometimes tender but without erythema and warmth. Effusion (if present) are serous (not hemorrhagic). Symptoms are transient (resolves in a few days) with no permanent damage to the joints.

Of the 40% of patients who develop kidney involvement, almost all have evidence (visible or on urinalysis) of blood in the urine. More than half also have proteinuria. Most disease is mild, but nephrotic syndrome, HTN, acute renal failure can occur

Occurs in 62% of cases. The abdominal pain is colicky in character, and may be accompanied by nausea, vomiting, constipation or diarrhea. Complicated by: gastrointestinal hemorrhage, bowel ischemia and necrosis, intussusception and bowel perforation.

Occurs in 1.3-13.6% of reported series. The leadpoint is usually a small bowel hematoma secondary to intestinal hemorrhage and mucosal edema. Most common types: are ileoileal (51%) and ileo-colic (39%). In contrast to idiopathic intussusception where 8090% are ileo-colic. Contrast enema reduction is often ineffective in cases of intussusceptions in HSP as they are often confined to the small bowel

Clinical presentation is less distinctive, especially when the child already has abdominal symptoms & signs. Definitive treatment by enema is hazardous in view of the underlying intestinal vasculitis & may not be accessible by the enema as it is usually located in the small intestine.

After surgery, intussusception may occur at a different site.

A 5-year-old girl was admitted to our institute with a two days history of swelling of the feet, purpuric rashes on the buttocks and the extensor aspects of the forearm and intermittent abdominal pain. Urinalysis revealed microscopic hematuria.

A presumptive diagnosis of HenochSchnlein purpura was made.

On the next day, she suffered of acute severe abdominal pain, tenderness all over the abdomen and palpable periumbilical mass. NGT showed bilious drainage.

A plain X-ray abdomen together with abdominal U/S confirmed the diagnosis of intussusceptions. Hydrostatic reduction was not attempted in view of the patients clinical condition as well as the underlying vasculitis.

At laparotomy , an ileoileal intussusception was identified and reduced manually with some difficulty.
Though the bowel was congested looking and viable, a small gangrenous ileal zone was found and resection with primary anastomosis was mandatory.

The post-operative period: showed uncomplicated recovery till she passed stool and tolerated oral feeding before been discharged on the 5th postoperative day.