Henoch-Schönlein Purpura in Children and Adults

Henoch-Schönlein Purpura is a medical condition in which there is inflammation and bleeding in the small blood vessels of the skin, joints, intestines, and kidneys. The classic feature of Henoch-Schönlein Purpura in Children and Adults is a purple colored rash which forms usually in the lower legs and the hips. This condition can also cause pain in the abdomen and joints. In rare cases, this condition causes kidney dysfunction. This condition can occur at any age, but it is mostly found in children between the ages of 2 and 5. It is a self limiting condition and resolves on its own without any specific treatment. Medical treatment is only required if this condition starts to affect the kidneys.

Henoch-Schönlein Purpura is caused due to inflammation of the small blood vessels of the body resulting in bleeding from the skin, joints, abdominal areas, and kidneys. It is not entirely clear as to why this inflammation occurs although some studies suggest that it may be caused due to some problem with the immune system of the body. Studies show that majority of the cases of Henoch-Schönlein Purpura in Children and Adults occurred after they had an upper respiratory infection like cold which triggered the condition. Other conditions like chickenpox, strep throat, measles and hepatitis have also been shown to cause this condition. Some of the other triggers for Henoch-Schönlein Purpura in Children and Adults are certain classes of medications, food, insect bites, or exposure to extremely cold temperatures.

Some of the risk factors which predispose a Child or an Adult to Henoch-Schönlein Purpura are:

• Age: This disease is mostly found in children between the age of 2 and 5 hence they are the ones who are most at risk for developing this condition
• Sex: This condition is known to occur more in boys than girls
• Race: This condition is known to occur more in whites than in African-American children
• Season: Henoch-Schönlein Purpura tends to occur mostly in the autumn, winter and spring and is very rare in the summer season.

Normally there are four main classic presentations of Henoch-Schönlein Purpura, but it is not always that an affected Child or an Adult has all the four signs of Henoch-Schönlein Purpura. These features are:

Rash: Presence of a purple colored rash looking somewhat like a bruise are the most classic sign of Henoch-Schönlein Purpura. This rash can be seen mostly in the hips, legs, and feet but it can also be seen in the arms, face and trunk of the child or an adult. This rash is usually worse in areas where there is increased pressure of the body like the waist.

Swollen Joints: Apart from this rash, the affected child or an adult will also have swollen and sore joints usually of the knees and ankles. This pain usually follows the development of the purple colored rash. The pain and swelling slowly subsides as and when the disease clears up and causes no permanent damage.

Gastrointestinal Symptoms: Quite a few of children with Henoch-Schönlein Purpura tend to suffer from gastrointestinal symptoms like pain in the abdomen, nausea, vomiting or blood in the stools. These symptoms tend to occur before the appearance of the rash.

Renal Problems: Henoch-Schönlein Purpura can at times affect the function of the kidneys in children or adult. In majority of the cases, this will be evident as proteinuria or hematuria and it may not even be diagnosed until a blood test is done. Normally, this clears up as and when the condition clears but at times the kidney dysfunction may persist for a bit longer.

In the presence of the classic purple colored rash, it becomes easier for a physician to diagnose Henoch-Schönlein Purpura along with presence of joint pain, and other gastrointestinal symptoms. In case Henoch-Schönlein Purpura is suspected and not all of the classic signs and symptoms are present then the physician may order certain test to confirm the diagnosis of Henoch-Schönlein Purpura. These tests are:

Laboratory Investigations: Even though there is specific test to confirm the diagnosis of Henoch-Schönlein Purpura, but certain laboratory tests listed below can help rule out other conditions that may be causing the symptoms:

Blood Draws: Children or Adult with Henoch-Schönlein Purpura will have an abnormal level of a specific antibody in their blood which can be identified in the blood studies.

Urine Tests: Urinalysis can be done to detect the presence of proteinuria or hematuria.

Biopsies: In case if the rash present is not classic and other tests fail to confirm Henoch-Schönlein Purpura then in that case the physician may take a skin biopsy to look for inflammation. The physician may also take a kidney biopsy if there is a suspicion of a kidney involvement and based on the results the physician will then formulate a detailed treatment plan for the child or an adult with Henoch-Schönlein Purpura.

Radiographic Studies: The treating physician will also recommend an ultrasound to rule out other causes of pain in the abdomen like some type of intestinal or bowel obstruction.

Henoch-Schönlein Purpura in Children or Adult is a self limiting condition and improves on its own without requiring any specific treatment and does not cause any permanent damage to any of the vital organs of the body. The physician may recommend adequate bedrest, taking plenty of fluids, and may prescribe pain medications like ibuprofen or Advil for pain and inflammation control.

Steroid medications are sometimes given for control of gastrointestinal symptoms that are caused due to Henoch-Schönlein Purpura, but the efficacy of it in this condition is debatable and also steroids have significant side effect profile, thus before starting this medication it is advisable to discuss the risk benefit ratio with the treating physician.

Surgery for Henoch-Schönlein Purpura: A surgical repair may be contemplated in case if there is folding of the bowel on itself.

Henoch-Schönlein Purpura is a condition which is more common in children than adults. In children it is more of a self limiting disease and recovery period is within four to six weeks without any specific treatment, whereas in adults this takes more of a serious turn with involvement of the kidneys even though this disease is rare in adults. This condition tends to recur in children but tends to have no long term complications.

In adults there is a greater possibility of renal involvement and studies have shown more cases of renal failure, thus making the prognosis in adults significantly guarded. Since the prognosis is quite guarded, based on studies, adults usually require treatment with high dose steroids with significantly successful outcome without any renal complications.

As stated, Henoch-Schönlein Purpura is quite rare in adults, but if it is diagnosed it is imperative to treat the condition as early as possible as the prognosis starts to decline the more the treatment is delayed.

It is advisable to make sure that children with Henoch-Schönlein Purpura are made as comfortable as possible till the condition resolves itself. Adequate bedrest, drinking adequate amounts of fluids, and use of pain medications for pain relief is vital for the symptom control in Henoch-Schönlein Purpura.