Henoch- Schonlein Purpura : Causes, Symptoms, Pathophysiology, Diagnosis, Treatment and Prognosis

Henoch-Schonlein purpura is a condition that causes inflammation of the small blood vessels in the skin, joints, bowels, and kidneys. A purplish-red rash (purpura) develops when blood from inflamed blood vessels bleeds into the skin.

The most obvious symptom of this type of vasculitis is a purplish rash, which frequently develops on the lower thighs and buttocks. Aching joints and stomach pain are additional Henoch-Schonlein purpura symptoms. Serious kidney damage is an uncommon occurrence.

Henoch-Schonlein purpura can affect anyone, however, it most commonly affects children under the age of 10. Usually, the issue resolves itself. Generally speaking, medical intervention is necessary if the condition affects the kidneys.

Causes of Henoch-Schonlein purpura

Small blood vessels in the body become inflamed as a result of Henoch-Schonlein purpura, which can lead to bleeding in the skin, abdomen, and kidneys.

There is no known cause for this early inflammation. It can be the result of an unintentional immunological response to particular triggers.

Nearly half of the patients with Henoch-Schonlein purpura had an upper respiratory infection, such as a cold, at its inception. Additional triggers include exposure to cold weather, hepatitis, chickenpox, strep throat, measles, certain medicines, nutrition, and insect bites.

Symptoms Of Henoch-Schonlein purpura

There are four main characteristics of Henoch-Schonlein purpura, and they are as follows:

• Rash (purpura). Bruises-like reddish-purple areas form on the legs, feet, and buttocks.On the arms, face, trunk, and pressure regions like the sock line and waistline, the rash may be extremely severe.
• Arthritis-related joint discomfort and edema. Patients with Henoch-Schonlein purpura usually complain of pain and edema near their joints, especially the knees and ankles. Sometimes the distinctive rash shows up a week or two before joint pain. These symptoms go vanish when the sickness is gone and inflict no long-term damage.
• Signs of the Gastrointestinal Tract. Children with Henoch-Schonlein purpura frequently have stomach pain, nausea, vomiting, and bloody feces. These warning signs and symptoms can occasionally appear before the rash.
• Henoch-Schonlein purpura may also have an effect on the kidneys. Most frequently, this shows up as blood or protein in the urine, which you might not even be aware of until a urine test is done. This typically goes away after the illness is ended, but in rare cases, patients develop chronic renal disease.

Pathphysiology of Henoch-Schonlein purpura

IgA has a key part in the pathophysiology of Henoch-Schönlein purpura, despite the fact that this is not entirely understood. IgA-antibody immune complexes deposited in the tiny vessels (often capillaries) of the skin, joints, kidneys, and gastrointestinal tract are brought on by antigenic exposure from an illness or treatment. 

Prostaglandins and other inflammatory mediators enter the system as a result of this. Complement C3 receptor cells may bind to immune complexes and accumulate in vessel walls, causing an inflammatory response that is over the top. 

Gastrointestinal haemorrhage could result from immune complexes that have been deposited in the gut wall. IgA-mediated immune complexes that affect the kidneys can cause mild proliferative glomerulonephritis or extremely severe crescentic glomerulonephritis. Palpable purpura and petechiae are brought on by immune complex deposition in the skin.

Diagnosis Of Henoch-Schonlein purpura

Your doctor can diagnose Henoch-Schonlein purpura if you exhibit the characteristic rash, joint discomfort, and digestive system symptoms. If one of these warning signs or symptoms does not exist, your doctor may suggest one or more of the tests listed below.

experimenting in a lab- Henoch-Schonlein purpura cannot be diagnosed with certainty by a single laboratory test, although various tests can assist exclude other diseases and raise the possibility that Henoch-Schonlein is the cause of the patient's symptoms. They might include

• Test using blood. Your blood may be examined if a diagnosis cannot be made purely based on your symptoms and signs.
• Analyses of urine. Tests on your urine may be done to search for symptoms to see if your kidneys are still functioning normally.
• Biposies. Immunoglobulin A (IgA) deposits are frequently found in the affected organs of Henoch-Schonlein purpura patients. Your doctor may take a very little sample of skin so a lab technician can analyse it. In cases of severe renal involvement, your doctor might suggest a kidney biopsy to assist guide your treatment decisions.
• Scanning tests. Your doctor can suggest an ultrasound to rule out alternate causes of stomach pain and look for possible complications like a bowel obstruction.

Treatment Of Henoch-Schonlein purpura

Henoch-Schonlein purpura frequently goes away on its own within a month with no long-term detrimental effects. Rest, a lot of water, and over-the-counter medicines can all help with symptoms.

Medications

The duration and intensity of joint and gastrointestinal discomfort may be lessened with the aid of prednisone and other corticosteroids. Because these medications can have serious side effects, talk to your doctor about the benefits and dangers of using them.

If a section of the bowel has ruptured or folded back on itself, surgery may be necessary.

Prognosis Of Henoch-Schonlein purpura

Henoch-Schönlein purpura normally has a good prognosis in patients without renal involvement and is a self-limited condition. Most patients finish their recovery in four weeks. Within 4 to 6 months of the first onset, Henoch-Schönlein Purpura returns in about one-third of patients.

The degree of renal involvement determines the long-term morbidity of Henoch-Schönlein purpura. End-stage renal disease (ESRD) and the need for a kidney transplant occur in about 1% of people with Henoch-Schönlein purpura.

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