(anaphylactoid purpura, rheumatoid purpura, IgA vasculitis)

Annual incidence in children is between 1/4,880 and 1/6,660, and around 1/1,000,000 in adults. The ratio of males to females is 1.5:1. This vasculitis mainly affects boys between 4 and 8 years of age.

It is caused by the formation of immune IGA complexes deposits in the small 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, generally symmetrical 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 %).

Other manifestations are rare, but may include: headache, convulsions, coma, paresis, orchi-epididymitis, urethritis, pancreatitis, myositis, episcleritis, pulmonary hemorrhage and myocarditis.

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 involvement, an immunosuppressive treatment is attempted.

Anesthetic implications: 

-         monitor renal function; adapt fluid and electrolyte intake

-         difficult peripheral venous approach (edema, multiple punctures)

-         in the event of intussusception, there is no point in attempting a barium or gas enema to reduce it: risk of perforation

-         IV corticosteroid therapy

-         although platelet levels are usually normal, the risk of bleeding is a contraindication to a perimedullary block

References : 

-        Sedeek KA, Liu J.
The management of neuraxial anesthesia in Henoch–Schonlein purpura (HSP) patient.
Pediatr Anesth 2009 ; 19 : 811-2. 

-        Patel RV, Sheth J, Kumar H, Patwardhan N.
Laparoscopic reduction of ileoileal intussusception in Henoch-Schönlein purpura. 
BMJ Case Rep 2013 doi:10.1136/bcr-2013-200344

-        Hasija N, Taxak S, Bhardwaj M, Vashist K.
Anesthetic management of a patient with Henoch-Schonlein purpura for drainage of cervical lymphadenitis: A case report.
Saudi J Anaesth 2014;8:282-3.

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

Updated: May 2025