Henoch-Schonlein purpura (HSP) or IgA vasculitis is a condition where small blood vessels of the skin, kidneys, intestines, and joints become inflamed. HSP in children is usually more common and often affects boys between two and six years of age (1).
The condition leads to characteristic purple-colored rashes, often on the buttocks and lower legs. HSP usually resolves in children without causing any long-term effects. However, if kidneys are involved, timely medical intervention is needed to avoid complications.
Keep reading to know the various symptoms, risk factors, causes, and treatments for Henoch-Schonlein purpura in children.
Signs And Symptoms Of Henoch-Schonlein Purpura
Henoch-Schonlein purpura causes inflammation of the blood vessels, which may cause bleeding in the abdomen, kidneys, and skin. The common signs and symptoms of Henoch-Schonlein purpura in children include (2) (3) (4) (5).
- Purpura (rash): HSP rashes are reddish-purple spots that appear on the feet, legs, and buttocks in children. These may look like spots of bruises on the skin. Some children can develop purpura on the face, arms, trunk, or pressure areas, such as the waistline or sock line. Subcutaneous edema (swelling), especially periorbital edema, may also be seen in younger children.
- Sore and swollen joints: HSP could cause arthritis in children. Pain and swelling on the knees and ankle joints are usually seen, and joint pain may occur one or two weeks before the onset of rashes. These symptoms often resolve when the disease passes and do not cause permanent damages.
- Gastrointestinal symptoms: Abdominal pain (belly pain) is one of the most common digestive tract symptoms in HSP. Vomiting, nausea, and bloody stools are seen in some children. These symptoms generally occur before the onset of purpura. Dark stools (melena) due to GI bleeding and acute pancreatitis are other common GI symptoms.
- Kidney symptoms: Kidney (renal) involvement, termed IgAV nephritis or HSP nephritis, is the most severe complication of HSP. Proteinuria (proteins in the urine) and hematuria (blood in the urine) are seen in HSP with kidney involvement. These symptoms may not be visible and are often identified via urine tests.
- Neurological symptoms: In some rare cases, the central nervous system may also be involved. Headache, seizures, vision problems, and intracranial hemorrhages are common symptoms seen in nervous system involvement.
Some children may have cardiac and pulmonary symptoms such as breathing difficulties due to the small vessels’ involvement in the heart or lungs. Swelling of the scrotum can also be seen in boys. These symptoms can also be seen in other diseases. Thus, seek medical care for the exact diagnosis and treatment.
Causes And Risk Factors For Henoch-Schonlein Purpura
The exact cause of Henoch-Schonlein purpura is not known (1). However, it is believed that the initial inflammation may be due to the immune system’s abnormal responses to certain triggers, and in some rare cases, allergic reactions. The following factors are known to trigger HSP in some children (3).
- Upper respiratory infections such as flu or cold
- Strep throat
- Exposure to cold weather
- Insect bites
Although they are exposed to these triggering factors, some children may not develop the disease. Boys are at a higher risk for developing HSP than girls, and Asian and white children are more likely to develop HSP than other ethnicities.
Complications Of Henoch-Schonlein Purpura
Henoch-Schonlein purpura may resolve within a month without any damages in most children, and there is a chance for recurrence in some children.
The possible complications of Henoch-Schonlein purpura include (6).
- Kidney damages or renal failure
- Bowel perforation
- Gastrointestinal bleeding
- Pleural effusion (water on the lungs)
- Testicular torsion
- Intracranial hemorrhage
- Pulmonary hemorrhage
When To See A Doctor?
If your child has HSP symptoms, seek pediatric care as soon as possible to ensure it does not cause permanent damages, such as kidney problems.
Although purpura is a characteristic symptom of HSP, the other symptoms may develop one or two weeks before these rashes. So, do not hesitate to consult the doctor for other symptoms.
Diagnosis Of Henoch-Scholein Purpura
Doctors may diagnose Henoch-Schonlein purpura by observing the skin rashes and inquiring about your child’s health history. The following tests are often ordered to confirm or rule in the diagnosis (7).
- Laboratory tests
Lab tests may help exclude the diagnosis of other conditions that cause similar symptoms. Blood tests are ordered to look for any abnormalities if the diagnosis is not precise. The presence of proteins and blood in urine may help determine kidney involvement.
- Imaging tests
Imaging tests, such as abdominal ultrasound, are often recommended to rule out abdominal pain. Complications such as bowel obstruction can also be identified via abdominal ultrasound.
Henoch-Schonlein purpura may cause IgA (immunoglobulin A) deposit in the affected organs. Biopsies of the affected part can confirm this. Skin biopsies are usually done in most children. However, renal (kidney) biopsies are ordered if there is suspicion of severe kidney involvement.
Although the diagnostic tests are not very specific, they are often required to make appropriate treatment decisions.
Treatment For Henoch-Schonlein Purpura
Treatment options may vary in each child depending on the severity, organs involved, complications, and health status. The treatment options may include (8).
- Home remedies
Henoch-Schonlein purpura may resolve in a month without long-term effects. So, doctors may recommend home care remedies for mild cases. These may include:
- Over-the-counter pain relievers
- Proper hydration
Home care measures are only recommended for mild cases. Do not use OTC medications for HSP in children before pediatric consultation.
There are no specific medications to cure Henoch-Schonlein purpura. Doctors may prescribe corticosteroids, such as prednisone, for symptomatic management to reduce the intensity and duration of joint and abdominal pain.
Prednisolone is not recommended for all cases since it does not prevent renal involvement and complications or alter the recurrence rate. You may discuss the risks and benefits of corticosteroid treatment with your pediatrician based on individual factors.
Surgery is required for complications such as bowel perforation, severe GI bleeding, and intussusception. Kidney transplantation is considered in children who have severe kidney disease and do not respond to medications. Tonsillectomy with pulse corticosteroid therapy is recommended for progressive IgAV nephritis.
How Long Does It Take To Recover From HSP?
Henoch-Schonlein purpura may go away within a month in most children. However, recurrence occurs in about one-third of children within four to six months after the initial onset (5).
Monthly urinalysis and blood pressure measurement are recommended for the first six months after diagnosis to identify renal involvement. Other tests to assess the renal function are ordered during follow-up visits if the urinalysis is positive. Never delay or stop follow-up care until recommended since early identification of organ involvement may prevent potential long-term complications.
2. Henoch-Schönlein Purpura; National Organization for Rare Disorders
3. Henoch-Schonlein purpura; St. Clair Hospital
4. Louise Oni and Sunil Sampath; Childhood IgA Vasculitis (Henoch Schonlein Purpura)—Advances and Knowledge Gaps; Frontiers in Pediatrics (2019).
5. Porsha Roache-Robinson and David T. Hotwagner; Henoch Schönlein Purpura; StatPearls Publishing (2020).
6. Henoch-Schönlein Purpura; American Academy of Family Physicians
7. Henoch-Schönlein purpura; The Royal Children’s Hospital Melbourne
8. Henoch-Schonlein Purpura; The Children’s Hospital of Philadelphia