What Causes Henoch Schonlein Purpura?

In addition to gastrointestinal problems, Henoch-Schönlein purpura causes characteristic bruise-like marks on the skin.

Henoch-Schönlein purpura, now called IgA vasculitis or IgAV, is a vascular condition that usually causes a rash that looks like bruises. It may also affect the gastrointestinal tract, the kidneys, the joints, and, in rare cases, the lungs and the central nervous system. Causes are not well understood, but probably more than one factor is involved.

Henoch-Schönlein purpura (IgA vasculitis) typically has a prodrome, that is, symptoms that appear before the characteristic rash. Symptoms of the prodrome include:

• Headache
• Loss of appetite
• Fever

After the prodrome, symptoms of Henoch-Schönlein purpura (IgA vasculitis) develop, including:

• Rash (95-100% of cases – this is the main feature of the disease)
  • Rash looks like tiny raised bruises
  • Usually involves the lower legs and ankles in older children and adults
  • May involve the back, buttocks, arms, and upper thighs in younger children
  • Hives may also occur
  • Target lesions that resemble a bullseye may occur
• Abdominal pain
• Vomiting, including vomiting blood
• Ulcers
• Joint pain, tenderness, and swelling, especially involving the knees and ankles
• Subcutaneous edema (fluid retention under the skin)
• Swelling of the scrotum
• Blood in the stool
• Kidney problems
• Priapism (prolonged erection)
• Vasculitis involving the central nervous system
• Intracranial bleeding with confusion and convulsions; bilateral subperiosteal orbital hematomas (clots in tissues around the eyes)
• Cystic changes of the ovaries
• Acute pancreatitis (rare)
• Intussusception, where the intestine folds into itself and can cause intestinal blockage in and severe belly pain (in children)

For most patients, symptoms of Henoch-Schönlein purpura (IgA vasculitis) usually last about a month, sometimes two months, and go away without causing lasting problems. 

The cause of Henoch-Schönlein purpura (IgA vasculitis) is not completely understood but is believed to be caused by multiple factors, including:

• Genetics
• Environmental factors 
  • Certain drugs such as 
    • ampicillin, 
    • erythromycin, 
    • penicillin, 
    • quinidine, 
    • quinine, 
    • losartan, and 
    • cytarabine 
  • Foods
  • Horse serum
  • Cold exposure
  • Insect bites
• Infections 
  • Upper respiratory tract
  • Throat infections
  • Gastrointestinal infections
• Certain vaccines
• Typhoid and paratyphoid A and B
• Measles
• Yellow fever
• Cholera

Glomerulocystic kidney disease has also been noted

The diagnosis of Henoch-Schönlein purpura (IgA vasculitis) involves identification of palpable purpura (mandatory criteria) along with at least one of the following:

• Diffuse abdominal pain
• Biopsy sample showing predominant IgA deposition
• Arthritis or acute pain in any joint
• Kidney involvement (blood or protein in the urine)
• IgA deposition in a biopsy sample in patients with atypical distribution of purpura 

Lab tests may be indicated to exclude other conditions or to evaluate kidney function, such as:

• Antinuclear antibody (ANA) and rheumatoid factor (RF)
• Factors VIII and XIII
• Urinalysis
• Complete blood count (CBC)
• Platelet count
• Erythrocyte sedimentation rate (ESR)
• Stool guaiac test
• Blood urea nitrogen (BUN) and creatinine
• Amylase and lipase
• Electrolytes
• Plasma D-dimer (test for blood clots)
• Plasma thrombin-antithrombin (TAT) complex, prothrombin fragment (PF)-1, and PF-2
• Prothrombin time (PT) and activated partial thromboplastin time (aPTT)
• Serum IgA
• Antistreptolysin O (ASO)
• CH50
• C3 and C4
• Immunocomplexes of IgG and IgA

Imaging studies that may be used include:

• Ultrasound (abdominal, scrotal/testicular)
• X-rays (chest, abdomen, small intestine; barium enema study)
• Magnetic resonance imaging (MRI) for assessing neurologic findings
• Computed tomography (CT) of the head or abdomen

Other studies may include:

• Endoscopy
• Renal biopsy 

Treatment for Henoch-Schönlein purpura (IgA vasculitis) is usually supportive and aimed at relieving symptoms, and may include:

• Drinking plenty of fluids/staying hydrated
• Monitoring for abdominal and kidney complications
• Treating minor symptoms of arthritis, fluid retention (edema), fever, or feeling unwell (malaise)
• Eating a bland diet
• Stopping any drugs suspected of causing symptoms (Do not stop taking any medications without first talking to your doctor.)

Medications for Henoch-Schönlein purpura (IgA vasculitis) may include:

• Pain relievers 
  • Acetaminophen (Tylenol)
  • Ibuprofen (Advil, Motrin)
  • Naproxen (Aleve)
  • Ketoprofen (Orudis)
• Corticosteroids
  • Methylprednisolone (Solu-Medrol, Medrol, A-Methapred, Depo-Medrol)
  • Prednisone (Rayos)
  • Prednisolone (Millipred, Orapred, Orapred ODT, Prelone)
• Other drugs
  • Azathioprine
  • Cyclophosphamide
  • Cyclosporine (Gengraf, Neoral, Sandimmune)
  • Angiotensin converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs)
  • Dipyridamole
  • High-dose IV immunoglobulin G (IVIg)
  • Danazol
  • Fish oil

Plasmapheresis may be used to treat the progression of kidney disease. 

Surgery to treat Henoch-Schönlein purpura (IgA vasculitis) may be needed in specific circumstances and includes:

• Surgery for severe bowel ischemia
• Kidney transplantation for severe renal disease 
• Tonsillectomy with corticosteroid pulse therapy for progressive nephritis

Henoch-Schönlein purpura (IgA vasculitis) usually lasts about a month or two and goes away without causing lasting problems for most patients. 

Complications of Henoch-Schönlein purpura (IgA vasculitis) may include:

• Gastrointestinal problems
  • Intussusception, where the intestine folds into itself and can cause intestinal blockage in and severe belly pain (usually ileo-ileal)
  • Bowel infarction
  • Bowel perforation
  • Hydrops of the gallbladder
  • Pancreatitis
  • Massive GI bleeding
• Kidney (renal) problems
• Relapses