Characteristics of Henoch-Schönlein Purpura Disorder

04 March, 2021
Henoch-Schönlein's purpura disorder can happen at any age but almost always occurs in childhood. The prognosis is good in most cases and recovery occurs spontaneously without further consequences.

Henoch-Schönlein purpura is a vascular autoimmune disease whose cause is still unknown. Johann Lukas Schönlein described it first in 1860 in collaboration with his teacher Eduard Heinrich Henoch. However, its etiology is still unclear.

This disease is significantly more common in childhood. Moreover, estimates indicate it affects an average of ten to 20 children per 100,000 every year. Also, boys are more likely to develop this condition.

This disease occurs when the immune system doesn’t function properly. A protein called immunoglobulin A (IgA), which functions as an antibody, begins to build up in the blood vessels and triggers certain symptoms.

Henoch Schönlein’s purpura disorder is also known as:

  • Schönlein’s purpura
  • IgA vasculitis
  • Anaphylactoid purpura
  • Purpura rheumatica
  • Schönlein–Henoch purpura

What’s Henoch-Schönlein purpura?

An illustration of the circulatory system.

Henoch-Schönlein purpura is a disorder in which small blood vessels inflame and irritate. This condition is known as vasculitis and mainly affects the skin but can also occur in the intestines and even the kidneys at times.

In many cases, this disease is preceded by a bacterial or viral infection of the upper respiratory tract. Be it a sinus, throat, or lung infection.

There have been reports of some cases in which the disease appears after the use of certain medications, insect bites, and ingestion of certain types of food or vaccines. However, these cases aren’t too common.

This disorder requires a clinical diagnosis on the basis of its main characteristics:

  • Skin rash
  • Inflammation and pain in the joints
  • Digestive difficulties
  • Kidney problems

The skin rash

There are skin symptoms in 80 to 100% of cases of Henoch-Schönlein purpura. The most characteristic is a collection of purplish-red spots that are similar to bruises that usually appear on the buttocks, legs, and feet.

In some rare cases, the spots also appear on the face, ears, arms, and back. It’s common to have edema or swelling from excess fluid on the scalp, hands, feet, face, and scrotum at the onset. Blisters on the skin, filled with fluid or blood, are rare in children, but frequent in adults.

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Inflammation and pain in the joints

 

Several pictures of a person with a bruised skin.

There are joint symptoms in 40 to 75 % of cases of Schönlein’s purpura disease. They basically include inflammation and pain (arthritis). This occurs mainly in the knees and ankles.

The joint problems appear one to two weeks before the onset of skin symptoms, in about 15 to 25% of the cases. This kind of arthritis is temporary and usually disappears in a few days. In fact, it doesn’t leave any deformity or sequelae.

Digestive difficulties in Henoch-Schönlein purpura

Gastrointestinal problems occur in 50 to 75% of cases. Furthermore, the most frequent manifestation is abdominal pain, present in up to 85% of those affected. Only in 14% of cases does it appear before the skin manifestations of the disease and often complicates the initial diagnosis.

There’s blood in the stools in half of the cases. Other symptoms such as nausea and vomiting are also common. In a small percentage of cases, there’s also gastrointestinal bleeding, intussusception, ulcers, perforations, and acute pancreatitis.

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Kidney problems

Renal manifestations appear in 20 to 50% of the cases and occur during the first month in 75 to 80% of patients and in the following three months in 97 to 100% of the cases. Renal problems determine the severity of the disease and its long-term prognosis.

Basically, they comprise hematuria (blood in the urine) and proteinuria (protein in the urine) but rarely. Finally, the condition can lead to different nephritic syndromes if the kidney problem progresses. These, in turn, can lead to renal failure within 10 years in 50% of those affected.

Thanks for reading.

  • Lovillo, M. C., & Cruz, M. L. (2013). Púrpura de Schönlein-Henoch, enfermedad de Kawasaki y otras vasculitis. Pediatr Integral, 17(1), 34-46.