(Purpura anaphylactoid, Rheumatoid purpura)

The most common vasculitis in childhooh affecting mostly boys between the age of 4 and 8 years. Its annual incidence varies between 8 and 20/100.000. Due to the formation of immune IGA complexes deposits in the vessels and the renal mesangium. The cause is unknown but it is probably an immune response to an environmental antigen (respiratory infection, food, medicine).

It is manifested by:

-         purpuric rash  ("like a geography map ") often urticarial and edematous at the level of the legs, buttocks and arms, without thrombopenia

-         transient arthritis mostly affecting the lower limbs

-         gastrointestinal involvement: gastroenteritis, abdominal pain, rectorrhagia or intussusception (typically ileoileal), subocclusion (hematomas of the intestinal wall) or intestinal perforation

-         renal impairment: hematuria and/or proteinuria following focal or segmental glomerulonephritis (50%). Clinical expression is variable: hematuria, nephrotic syndrome, acute or chronic renal insufficiency (5-10%).

There is sometimes mild brain involvement with convulsions and coma.

The disease progresses by successive outbreaks over several weeks.

There is no anomaly of hemostasis and the platelet count is normal.

Treatment: corticosteroid therapy is started if the skin, the digestive tract or  an important joint is affected but it is inefficient in case of renal impairment. In case of severe renal impairment, immunosuppressive treatment is attempted.

Anesthetic implications: 

-         monitor renal function

-         difficult peripheral venous access (edema, multiple punctures)

-         in case of intussusception, it is useless to attempt to reduce it with a barium or air enema

References : 

-        Giniès J-L.
Purpura rhumatoïde,
In Gastroentérologie pédiatrique, coordonnateurs F Gottrand & D Turck, p 145-50,  Eds: Doin 2016

Updated: October 2016