Henoch-Schönlein purpura

Henoch-Schönlein purpura (pronounced "hen-awk, shurn-line pur-pu-ruh") is a relatively rare disorder that affects the small blood vessels just underneath the top layer of skin. The disease itself is inflammatory in nature and is thus classified as a form of vasculitis ("inflammation of blood vessels"). Because the disease most commonly affects children between the ages of 2 to 6 years old, it is important for parents to be aware of the symptoms, causes, diagnostic approaches, and treatment for the condition. 

Definition & Facts

Henoch-Schönlein purpura is designated as a "self-limited disease," which means that in many cases, and especially in the case of "common purpura," the disease will resolve itself whether treatment is administered or not.

However, this is not necessarily the case with more serious forms of the disease, which may require swift medical intervention and even surgery. There are several different forms of the disease, which can affect children as young as 2 all the way to adults.

It is thought that approximately 14 cases of Henoch-Schönlein purpura occur per 100,000 children in the United States each year. While sometimes causing alarming physical symptoms, the disease is not contagious.

Symptoms & Complaints

The rash is painless to the individual, but other accompanying symptoms (which are often described by patients as being similar to the onset of flu) are not. More boys than girls are affected by Henoch-Schönlein purpura. The most common symptoms other than the rash are as follows:

• Flu-like symptoms: appetite, headache, chills, fever, diarrhea, constipation, nausea.
• Joint swelling: ankles, knees, elbows, wrists, entire limbs, scrotum (in boys).
• Pain in the abdomen: includes cramping, vomiting, and rarely an emergency bowel fold condition (intussusception). 
• Black stool
• Blood in the urine: indication kidney function is affected - this is a medical emergency. 20 to 50 percent of child patients have issues with their kidneys
• Swelling (edema): rarely seen in the face and neck; sometimes accompanied by large hives.

Causes

Researchers are still working to determine the exact causes for Henoch-Schönlein purpura. However, some currently recognized possible links include the following:

• Bacterial infection (especially with Streptococcus, which is the cause of strep throat).
• Extreme food allergies (especially to eggs, chocolate, beans, milk).
• Administration of certain drugs (examples include diclofenac, nifedipine, cefuroxime, diltiazem).
• Previous history of insect bites.
• Preceding case of Rubella/measles (30 percent of cases of Henoch-Schönlein purpura show a link here) or upper tract respiratory infection (66 percent of cases of Henoch-Schönlein purpura show a positive link here). 
• Previous cases of hepatitis and chickenpox have also been cited as triggers.
• Exposure to cold weather is thought to trigger the disease for some.

It is thought that Henoch-Schönlein purpura could in itself be a symptom of an underlying autoimmune disease, where the body attacks itself for reasons as of yet unknown.

Diagnosis & Tests

Making an initial diagnosis of Henoch-Schönlein purpura relies on presentation of the main symptoms, which includes joint swelling, the red-purple rash and abdominal pain. However, other tests are typically required to confirm the diagnosis:

• Blood tests. These tests look for high levels of kidney waste, including creatinine and blood urea nitrogen.
• Clinical urine tests. If the urine shows high protein levels (proteinuria) or the presence of blood, this can confirm the diagnosis.
• Skin analysis tests. A skin biopsy may be taken as well to test the rash
• Ultrasound. An ultrasound imaging test can help the diagnosing physician see the condition of the abdominal area and check for any bowel obstruction.

It is more difficult to diagnose the condition when only one of the tests is done, since Henoch-Schönlein purpura in some of its forms can be confused with other similar (but less rare) vascular inflammatory diseases. It is also important to determine the precise type of Henoch-Schönlein purpura. Some types will affect the gastrointestinal tract and the kidneys while other types will affect the skin and joints.

Treatment & Therapy

Since Henoch-Schönlein purpura is considered to be a self-limited disease, it can often resolve itself in less than six months, although most cases will resolve within six weeks or less. For the mildest cases, rest, hydration and over-the-counter pain relievers can be all that is needed.

Because there is no known definitive treatment for the underlying disease itself, treatment usually focuses on alleviating the more uncomfortable symptoms, such as pain, cramping and swelling. Often prescribed drugs will include corticosteroids (prednisone, et al) to alleviate the sometimes severe gastrointestinal discomfort.

However, if there are complications that arise, these may need to be treated more aggressively and quickly. The two most common complications include damage to the kidneys and an obstructed bowel. In these two cases, surgery may be required.

Prevention & Prophylaxis

However, there are some known risk factors which can be controlled for. For example, knowing that the disease occurs more frequently in children, in males more than females, in white and Asian children more than in other races and in cooler seasons, parents can be aware and watchful for the early onset of symptoms.

In addition, minimizing any possible exposure to bacteria, drugs, diseases and conditions that are linked to the onset of Henoch-Schönlein purpura can also minimize the risk of an individual having this disease.