Schönlein-Henoch purpura

What are vasculitis?

Vasculitis is a set of many diverse diseases that are characterized by the existence of an inflammation of the blood vessels of different calibers with very varied causes, many of them being of autoimmune origin, that is, caused by the patient's own immune system.

How is it produced?

Schönlein-Henoch purpura is predominantly infantile and juvenile, under 20 years of age. It is more common in males and occurs more frequently in spring. This vasculitis usually occurs after an upper respiratory infection, especially by bacteria of the Streptococcus type.

It is a vasculitis produced by hypersensitivity, that is, due to the fact that the patient's body is exposed to an antigen - in this case it is usually the antigen - against which his immune system creates antibodies, which bind to antigens to attack them, forming what is called antigen-antibody complex. Once the infection has passed, these complexes are not adequately eliminated and are deposited in the smaller vessels, producing an inflammatory reaction in them that gives rise to vasculitis.

The capillaries and venules of the dermis are mainly affected, as well as the filtration vessels of the kidney, in which the aforementioned complexes and type A (IgA) immunoglobulins are found.

Symptoms

Schönlein-Henoch purpura is classified within systemic autoimmune diseases because they affect different systems, apparatus or organs simultaneously.

The characteristic feature that occurs in 100% of patients is the appearance of a palpable purple, that is, skin lesions with relief, that can be felt, purple or violaceous, mainly at the level of the lower extremities and buttocks, although they also appear less frequently on the face, ears or mucous membranes (for example, oral), as well as on the extensor surfaces of the upper extremities.

They are injuries symmetrical and rough to the touch that do not disappear when pressed and that can sometimes be painful. When they heal, they usually leave a hyperpigmented lesion, that is, a region of the skin that is more raised in color.

80% of patients report inflammation and joint pain, especially in large joints near the areas affected by cutaneous purpura, such as ankles, knees or elbows. Two out of every three patients present gastrointestinal symptoms, mainly abdominal cramping pain, primarily after eating food and due to the edema that occurs in the abdominal wall. nausea, vomiting or bloody diarrhea can sometimes occur, as well as upper or lower gastrointestinal bleeding or even peritoneal irritation.

At the renal level, due to involvement of the small vessels that make up the complex filtering system of the kidneys, hematuria or proteinuria may occur, that is, the presence of blood or protein in the urine, but this only occurs in 40% of cases. the patients. Rarely does kidney involvement eventually lead to chronic kidney failure. On more rare occasions, pulmonary, cardiac or central nervous system involvement may occur.

Diagnosis

The diagnosis will be based on the clinical suspicion due to the presence of fever, purple lesions on the skin, abdominal pain and joint manifestations, especially in children or young people with a history of having recently suffered from an upper respiratory infection. At the level of blood test you can see an elevation of leukocytes, as well as IgA. Renal function should also be assessed using urea and creatinine values. It is also advisable to carry out a urine test to determine the presence of blood or protein in the urine.

The diagnosis of certainty would be given by the biopsy of a venule with the lesions typical of Schönlein-Henoch purpura, but this procedure is performed very rarely, only if there are doubts about the diagnosis.

Treatment

Schönlein-Henoch purpura is usually self-limited, that is to say, it cures itself without the need for any treatment. Symptoms last between 4 and 8 weeks and only 1% end up giving one in the long run. In cases of significant joint involvement or severe abdominal pain that does not subside with analgesia, corticosteroids or even immunosuppressants such as cyclophosphamide can be administered if necessary.

Precautionary measures

There are no preventive measures against Henoch-Schönlein purpura since it is a autoimmune disease. In case of suspicion of the same one should go immediately to the pediatrician or general medicine doctor.

• It is characterized by an inflammation of the blood vessels.
• The characteristic feature is the appearance of raised skin lesions, which can be felt, purple or violet in color, mainly on the legs and buttocks.
• It is a disease that lasts from four to eight weeks and that heals on its own without the need for treatment.