Henoch-Schönlein Purple: Characteristics

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

This disease is more common in childhood. Moreover, estimates indicate that it affects an average of 10 to 20 out of 100,000 children each year. Boys are also more likely to develop this condition.

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

Henoch-Schönlein purpura is also known as:

What is Henoch-Schönlein purple?

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

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

Cases have been reported in which the disease occurred after the use of certain medicines, insect bites, ingestion of certain types of food or the administration of vaccines. But these cases are not very common.

This disorder requires a clinical diagnosis based on its main features:

The rash

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

In rare cases, spots also appear on the face, ears, arms and back. Due to excess fluid, edema occurs on the scalp, hands, feet, face and scrotum at the onset of the disease. Blisters on the skin, filled with fluid or blood, are rare in children, but common in adults.

Inflammation and pain in the joints

There are joint symptoms in 40 to 75% of cases of Schönlein purpura. These include inflammation and pain (arthritis). The problems occur mainly in the knees and ankles.

Joint problems occur one to two weeks before the onset of skin symptoms, in about 15-25% of cases. This type of arthritis is temporary and usually goes away in a few days. In fact, it does not leave behind any deformation or sequelae.

Digestive difficulties in Henoch-Schönlein purple

Gastrointestinal problems occur in 50 to 75% of cases. In addition, the most common manifestation is abdominal pain, present in 85% of those affected. Only in 14% of cases it occurs before the cutaneous manifestations of the disease and often complicates the initial diagnosis.

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

Kidney problems

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

Basically, they include hematuria (blood in the urine) and proteinuria (protein in the urine). The condition can lead to various nephritic syndromes if the kidney problem progresses. These, in turn, can lead to kidney failure within 10 years in 50% of those affected.

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