7 Key Symptoms of Henoch-Schonlein Purpura (HSP Disease) You Should Know

Henoch-Schonlein purpura (HSP), also known as IgA vasculitis, is an inflammatory disorder that causes small blood vessels in the skin, joints, intestines, and kidneys to become inflamed and leak. This condition primarily affects children between the ages of 2 and 11, though adults can also develop it. Understanding the symptoms of HSP is crucial for early detection and proper medical management.

HSP disease occurs when the immune system produces an abnormal antibody called IgA, which accumulates in blood vessels and triggers inflammation. While the exact cause remains unclear, the condition often develops after a respiratory infection. The hallmark sign of henoch schonlein purpura is a distinctive purple or red rash, but the disease can affect multiple organ systems, producing a variety of symptoms.

Below, we explore the seven key symptoms of Henoch-Schonlein purpura that patients and caregivers should recognize.

1. Purpuric Rash (HSP Rash)

The most characteristic symptom of HSP disease is a distinctive skin rash known as palpable purpura. This HSP rash appears as raised, purple or red spots that don’t fade when pressed. The spots are caused by bleeding under the skin from inflamed blood vessels.

Key characteristics of the HSP rash include:

• Typically appears on the lower legs, buttocks, and sometimes arms
• Starts as small red or purple dots that may merge into larger patches
• Raised and palpable to the touch, unlike bruises which are flat
• Does not blanch (turn white) when pressure is applied
• May be itchy or uncomfortable but usually not painful
• Often appears in crops, with new spots developing over several days or weeks

The rash is present in virtually all cases of henoch schonlein purpura and is often the first symptom that prompts medical evaluation. In some cases, the rash may initially be mistaken for bruising or other skin conditions, but its characteristic appearance and location help distinguish it from other disorders.

2. Joint Pain and Swelling

Approximately 75% of patients with IgA vasculitis experience joint involvement, making it one of the most common symptoms after the rash. The joint problems in HSP can range from mild discomfort to severe pain that temporarily limits mobility.

Joint symptoms typically include:

• Pain and swelling most commonly affecting the knees and ankles
• Stiffness, particularly in the morning or after periods of rest
• Difficulty walking or bearing weight in children
• Temporary limitation in range of motion
• Symptoms that may come and go, often improving within a few days
• Wrists, elbows, and fingers may also be affected, though less commonly

The good news is that joint involvement in HSP disease is usually temporary and doesn’t cause permanent damage. Unlike some forms of arthritis, the inflammation resolves completely without leaving lasting joint deformities. However, the pain can be significant enough to interfere with daily activities during active disease.

3. Abdominal Pain

Gastrointestinal symptoms occur in approximately 50-75% of patients with henoch schonlein purpura. Abdominal pain results from inflammation and bleeding in the blood vessels of the intestinal walls. This symptom can sometimes be severe and may even appear before the characteristic rash, making diagnosis challenging.

Abdominal manifestations include:

• Cramping or colicky pain that comes in waves
• Pain typically located around the belly button (periumbilical area)
• Discomfort that may worsen after eating
• Nausea and vomiting
• Loss of appetite
• Bloating or feeling of fullness

In most cases, abdominal pain from HSP is self-limiting and resolves within a few days to weeks. However, severe abdominal symptoms require immediate medical attention, as complications such as intussusception (telescoping of the intestine) or intestinal perforation, while rare, can occur and require urgent intervention.

4. Blood in Stool

Due to inflammation of blood vessels in the gastrointestinal tract, many patients with IgA vasculitis experience gastrointestinal bleeding. This can manifest as visible or microscopic blood in the stool and is a symptom that should always prompt medical evaluation.

Signs of gastrointestinal bleeding include:

• Bright red blood mixed with or on the surface of stool
• Dark, tarry stools indicating bleeding higher in the digestive tract
• Blood-streaked mucus in bowel movements
• Positive fecal occult blood test (microscopic bleeding not visible to the eye)
• May be accompanied by abdominal cramping or pain

While small amounts of blood in the stool are relatively common in HSP disease and usually resolve without specific treatment, significant bleeding requires immediate medical attention. Parents and caregivers should monitor bowel movements closely and report any signs of bleeding to healthcare providers.

5. Kidney Involvement (Hematuria and Proteinuria)

Kidney involvement is one of the most important complications of henoch schonlein purpura, occurring in approximately 20-60% of patients. While kidney problems may not cause obvious symptoms initially, they require careful monitoring as they can occasionally lead to long-term complications.

Signs of kidney involvement include:

• Blood in the urine (hematuria) – may be visible or only detectable by laboratory testing
• Protein in the urine (proteinuria)
• Swelling around the eyes, especially in the morning
• Swelling of the feet and ankles (edema)
• High blood pressure (may be asymptomatic)
• Reduced urine output in severe cases
• Foamy or bubbly urine due to excess protein

Most patients with kidney involvement in HSP have mild abnormalities that resolve completely. However, a small percentage may develop more significant kidney disease. Regular monitoring with urine tests and blood pressure checks is essential for all HSP patients, even after other symptoms have resolved, as kidney problems can sometimes develop weeks or months after the initial illness.

6. Fever and Fatigue

Many patients with HSP disease experience general constitutional symptoms, particularly at the onset of illness. These non-specific symptoms reflect the body’s inflammatory response and immune system activation.

General symptoms include:

• Low-grade fever, typically below 101°F (38.3°C)
• General feeling of being unwell (malaise)
• Fatigue and tiredness beyond what’s normal
• Lack of energy and reduced activity level
• Irritability, especially in young children
• Headaches in some cases
• Muscle aches or general body discomfort

These symptoms are often mild and may be overlooked or attributed to the viral infection that frequently precedes HSP. However, when combined with other characteristic signs like the purpuric rash or joint pain, they help complete the clinical picture of IgA vasculitis. The fatigue and general malaise typically improve as the inflammation subsides.

7. Scrotal Swelling in Boys

In males with henoch schonlein purpura, inflammation can affect the blood vessels of the scrotum and testicles, leading to swelling and pain in the genital area. This symptom occurs in approximately 10-35% of boys with HSP and can be quite alarming for patients and families.

Characteristics of scrotal involvement:

• Swelling of the scrotum, which may be unilateral or bilateral
• Pain or tenderness in the testicular area
• Redness or discoloration of the scrotal skin
• May be mistaken for testicular torsion, a surgical emergency
• Usually resolves without specific treatment
• Rarely causes long-term complications

While scrotal swelling in HSP typically resolves on its own without causing permanent damage, it’s important to seek medical evaluation to rule out other serious conditions such as testicular torsion, which requires emergency surgery. Healthcare providers may perform an ultrasound to differentiate between HSP-related inflammation and other causes of acute scrotal swelling.

Main Causes of Henoch-Schonlein Purpura

While the exact cause of HSP disease remains unknown, researchers have identified several factors that appear to trigger the condition. Understanding these potential causes can help patients and families recognize risk factors, though it’s important to note that HSP cannot always be prevented.

The main triggers and associated factors include:

• Infections: Upper respiratory tract infections are the most common trigger, particularly those caused by Streptococcus bacteria. Other infections linked to HSP include viral infections (such as varicella, parvovirus, and hepatitis), bacterial gastroenteritis, and mycoplasma.
• Immune System Response: HSP is believed to be an abnormal immune response where the body produces too much IgA antibody. These antibodies form complexes that deposit in small blood vessels, triggering inflammation.
• Medications: Certain medications have been associated with HSP, though this is less common. Antibiotics, anti-inflammatory drugs, and some other medications may rarely trigger the condition.
• Vaccinations: Very rarely, vaccinations have been temporally associated with HSP onset, though no causal relationship has been definitively established.
• Genetic Factors: While HSP is not inherited in a clear pattern, genetic susceptibility may play a role. Certain genetic markers may increase vulnerability to developing the condition.
• Environmental Factors: Exposure to certain chemicals, insect bites, or cold weather has occasionally been reported before HSP onset, though these associations are not well established.
• Age and Demographics: Children between ages 2-11 are most commonly affected, with peak incidence around age 4-6. The condition is slightly more common in boys than girls and occurs more frequently in winter and spring months.

In many cases, no specific trigger can be identified, and HSP appears to develop spontaneously. The condition is not contagious and cannot be spread from person to person.

Prevention of Henoch-Schonlein Purpura

Unfortunately, there is no known way to prevent henoch schonlein purpura, as the exact cause of the condition remains unclear and it often develops unpredictably. However, certain measures may help reduce the risk of triggers or support overall immune health.

General health measures include:

• Infection Prevention: Since respiratory infections are the most common trigger, practicing good hygiene can help reduce infection risk. This includes regular handwashing, avoiding close contact with sick individuals, and keeping vaccinations up to date.
• Prompt Treatment of Infections: Seeking appropriate medical care for strep throat and other bacterial infections may potentially reduce the risk of subsequent HSP, though this relationship is not definitively proven.
• Medication Awareness: If you or your child has had HSP before, inform healthcare providers about this history when new medications are prescribed, as certain drugs have rarely been associated with HSP.
• Supporting Immune Health: Maintaining overall health through adequate nutrition, sufficient sleep, regular physical activity, and stress management supports proper immune function.

For individuals who have had HSP, the risk of recurrence is relatively low (approximately 10-15%). There are no specific measures proven to prevent recurrence, but maintaining regular follow-up with healthcare providers, especially for kidney monitoring, is important.

Parents should be reassured that HSP is not caused by anything they did or didn’t do, and in most cases, could not have been prevented.

Frequently Asked Questions

How long does HSP disease last?

Most cases of henoch schonlein purpura resolve within 4-6 weeks, though symptoms can fluctuate during this time. Some patients experience a relapse of symptoms, which occurs in about one-third of cases, usually within the first few months. The rash and joint pain typically resolve first, while kidney monitoring should continue for at least six months.

Is Henoch-Schonlein purpura contagious?

No, HSP is not contagious and cannot be spread from person to person. While it often follows an infection that may be contagious, the HSP itself is an immune system response and not an infectious disease. Children with HSP can return to school once they feel well enough, even if the rash is still visible.

Can adults get HSP disease?

Yes, although henoch schonlein purpura is much more common in children, adults can develop the condition. Adult cases tend to be more severe, with a higher likelihood of kidney involvement and other complications. Adults with HSP may require more intensive monitoring and follow-up care.

What is the difference between HSP and IgA vasculitis?

There is no difference – they are two names for the same condition. IgA vasculitis is the newer, more medically accurate term that describes the disease mechanism (inflammation of blood vessels caused by IgA antibodies). HSP or Henoch-Schonlein purpura is the traditional name. Medical literature now increasingly uses “IgA vasculitis,” but both terms refer to the identical disease.

Will the HSP rash leave scars?

In most cases, the purpuric rash of HSP resolves completely without leaving scars. However, the affected areas may have temporary discoloration or darkening of the skin that can persist for weeks to months before fading completely. True permanent scarring is very rare unless there has been skin breakdown or secondary infection.

When should I seek emergency medical care for HSP?

Seek immediate medical attention if you or your child experiences severe abdominal pain, persistent vomiting, blood in vomit or stool, significant decrease in urine output, severe headache, changes in consciousness or behavior, difficulty breathing, or signs of severe allergic reaction. These could indicate complications that require urgent evaluation and treatment.

Can HSP affect the kidneys permanently?

Most patients with kidney involvement in HSP recover completely without long-term kidney damage. However, approximately 1-2% of children and a higher percentage of adults may develop chronic kidney disease. This is why regular monitoring with urine tests and blood pressure checks is essential for all HSP patients, continuing for at least 6-12 months after other symptoms resolve.

Do dietary changes help with HSP symptoms?

There is no specific diet proven to treat or prevent HSP. However, if significant gastrointestinal symptoms are present, your doctor may recommend temporarily avoiding certain foods or following a bland diet until symptoms improve. Some patients find that avoiding foods that increase intestinal irritation (such as spicy foods, caffeine, or high-fiber foods) helps reduce abdominal discomfort during the acute phase.

References:

• Mayo Clinic – Henoch-Schonlein Purpura
• National Center for Biotechnology Information – Henoch-Schönlein Purpura
• Johns Hopkins Medicine – Henoch-Schonlein Purpura
• NHS – Henoch-Schönlein purpura (HSP)
• Boston Children’s Hospital – Henoch-Schönlein Purpura
• National Kidney Foundation – Henoch-Schönlein Purpura