Purpura Schönlein-Henoch

School ager

with bilateral scrotal pain and a skin rash. Transverse color doppler US of the scrotum shows enlargement and hypervascularity of the bilateral crescent shaped epididymides when compared to their respective round testicles, to which they are lateral in position.The diagnosis was bilateral epididymitis in a patient with Henoch-Schonlein Purpura

pediatricimaging.org • CC-by-nc-sa 4.0

Preschooler

with a purpuric skin rash and abdominal pain. AXR AP (left) and spot image of the terminal ileum (right) from a small bowel follow through show bowel wall fold thickening (thumbprinting) throughout the jejunum and ileum.The diagnosis was Henoch-Schonlein purpura.

pediatricimaging.org • CC-by-nc-sa 4.0

IgA

vasculitis • IgA vasculitis - Ganzer Fall bei Radiopaedia

Glick, Y. IgA vasculitis. Case study, Radiopaedia.org. (accessed on 24 Oct 2022) https://doi.org/10.53347/rID-91789 • CC by-nc-sa 3.0 (modified)

IgA

vasculitis • Henoch-Schonlein purpura - duodenojejunal enteritis - Ganzer Fall bei Radiopaedia

Bickle, I. Henoch-Schonlein purpura - duodenojejunal enteritis. Case study, Radiopaedia.org. (accessed on 24 Oct 2022) https://doi.org/10.53347/rID-47153 • CC by-nc-sa 3.0 (modified)

IgA

vasculitis • Henoch-Schonlein purpura - Ganzer Fall bei Radiopaedia

Hamidi, H. Henoch-Schonlein purpura. Case study, Radiopaedia.org. (accessed on 24 Oct 2022) https://doi.org/10.53347/rID-82338 • CC by-nc-sa 3.0 (modified)

IgA

vasculitis • Duodenal thickening secondary to IgA vasculitis - Ganzer Fall bei Radiopaedia

Burns M, Duodenal thickening secondary to IgA vasculitis. Case study, Radiopaedia.org (Accessed on 24 Nov 2023) https://doi.org/10.53347/rID-159517 • CC by-nc-sa 3.0 (modified)

Radiopaedia • CC-by-nc-sa 3.0 • de

IgA vasculitis (formerly known as Henoch-Schonlein purpura (HSP)) is a type of non-thrombocytopenic immune-mediated small vessel acute leukocytoclastic vasculitis.

In order to differentiate from other types of vasculitides, the four commonly adopted diagnostic criteria by the American College of Rheumatology are:

age of onset less than 20 years (although some case series show disease involvement in older individuals)
palpable skin purpura
gastrointestinal bleeding
intussusception, typically ileoileal
biopsy showing granulocytes around small arteriolar/venular walls

Epidemiology

It tends to occur in the pediatric population (peak incidence 3-10 years) . IgA vasculitis is the most common type of vasculitis in children .

Pathology

There is the deposition of IgA immune complexes in small vessels.

Distribution

Commonest organ systems which get involved are:

skin: 95-100%, usually the first organ to get involved
gastrointestinal tract: 50-75%
joints: ~80%
kidneys

Rare sites:

central nervous system

Radiographic features

Gastrointestinal tract

Plain radiograph

May show evidence of bowel wall thickening due to hemorrhage and/or evidence of bowel obstruction. Plain film features are however non-specific.

Fluoroscopy

Small bowel follow-through may show evidence of separation of bowel loops, "thumbprinting" and/or mucosal thickening.

CT

May show multifocal bowel thickening, lymphadenopathy, mesenteric edema and vascular engorgement. Although non-specific, CT findings may aid in the diagnosis of IgA vasculitis in a young patient with acute gastrointestinal illness along with other, e.g. cutaneous features of IgA vasculitis. Intussusception, typically ileoileal type, is also seen.

Ultrasound

Echogenic kidneys, target or doughnut sign in case of intussusception. IgA vasculitis has also been shown to cause massive scrotal edema and can be a source of severe pain in young boys .

Treatment and prognosis

The disease is self-limiting in most instances. Treatment is usually supportive including fluids and pain relief in the pediatric population. Steroids are used in adults due to the risk of serious renal involvement .