Henoch-Schonlein Pupura

Henoch-Schonlein Pupura is an infection of the small blood vessels. The disease is a type of vasculitis and usually affects the blood vessels in the skin, intestines and kidneys. The inflamed blood vessels bleed into the skin and cause a reddish-purple rash called ‘purpura’. Purpura is a raised rash which does not go away when the skin is stretched. Bleeding in the intestine or kidneys may also occur, as there may be blood in the child’s stool or urine. The disease is named after the first doctors to describe the disease, Drs. Henoch and Schonlein.

The disease is not very common; however it is the most common disease of the blood vessels among children. It is two times as likely to occur among boys as girls.

The cause of the disease is unknown. In the past, doctors thought that the disease was an allergic reaction, since it would break out after bug bites, exposure to cold, chemicals and drugs. In some countries it is known as rheumatic purpura, since there is muscular and joint involvement. Today researchers believe that the disease is caused by viruses or bacteria.

HSP is a systemic vasculitis and is characterized by deposition of immune complexes containing IgA. This reaction is an abnormal reaction of the immune system which affects the skin, joints, digestive system, kidneys and sometimes the central nervous system. The disease is not genetic, contagious or preventable.

Signs and Symptoms of Henoch-Schonlein Pupura
The main sign of the disease is a rash that happens to everyone who has the disease. The rash starts off as small red, raised lesions. Over time, the color changes to purple-brown. The disease usually appears on the lower limbs and buttocks. Most patients experience joint pain, swelling and limited mobility. This usually occurs in the knee or ankle. Swelling in the soft tissue of the hands, feet, forehead or testicles can occur early on during the illness, and usually among younger children. Some children experience stomach pain around the navel. If the kidneys are involved (in 20%-35% of cases), blood appears in the urine. Generally kidney involvement is mild. In severe cases, the kidney disease can last a few months or years. If there is severe kidney involvement, please consult a nephrologist.

The rash appears on all patients, but the extent of involvement of other organs varies from patient to patient. The disease is extremely rare among adults.

Diagnosis of Henoch-Schonlein Pupura
Diagnosis is done based on the clinical appearance of the purpura rash in the lower limbs, stomach pain and joint pain. There are no specific lab tests available for HSP. Urine samples should be done as a way to monitor the disease and determine any kidney involvement. Kidney biopsies are necessary in situations of severe kidney involvement.

Is Henoch-Schonlein Pupura treatable?
Most children don’t require any medication. Treatment, if necessary, helps treat pain and joint pain. Steroids are used in cases where other organs are involved.

Side Effects
Since most patients don’t require any medication, there are usually no side effects. In rare cases of kidney involvement where patients require immunosuppressants, there are possible side effects.

The disease usually lasts 4-8 weeks. Half of the children will experience a recurrence within six weeks, which is generally milder than the first outbreak. Most children completely recover from the disease. Monitoring is required in order to rule out kidney involvement. Most children will not suffer from long-term effects of the disease. Some children may develop kidney disease.

Children suffering from HSP should limit their physical activity when the disease flares up, but after they recover, your child can return to his normal routine.