What is Henoch-Schonlein purpura (HSP)?
Henoch-Schonlein purpura (HSP) is a form of blood vessel inflammation or vasculitis. There are many different conditions that feature vasculitis. Each of the forms of vasculitis tends to involve certain characteristic blood vessels. HSP affects the small vessels called capillaries in the skin and frequently the kidneys. HSP results in a purplish skin rash (most prominent over the buttocks and behind the lower extremities) associated with joint inflammation (arthritis) and sometimes cramping pain in the abdomen. Henoch-Schonlein purpura is also referred to as anaphylactoid purpura.
What causes Henoch-Schonlein purpura?
HSP occurs most often in the spring season and frequently follows an infection of the throat or breathing passages. HSP seems to represent an unusual reaction of the body's immune system that is in response to this infection (either bacteria or virus).
- Aside from infection, drugs can also trigger the condition.
- HSP occurs most commonly in children, but people of all age groups can be affected, including adults.
The only known risk factors for HSP are exposure to certain viruses and drugs. The reaction to these agents that leads to HSP is not, however, predictable.
What are the symptoms of Henoch-Schonlein purpura?
- The rash of skin lesions appears in gravity-dependent areas, such as the legs.
- The joints most frequently affected with pain and swelling are the ankles and the knees.
- Patients with HSP can develop a fever.
- Inflammation of the blood vessels in the kidneys (nephritis) can cause blood and/or protein in the urine.
- Abdominal pain and gastrointestinal bleeding are more common in children than in adults.
- Serious kidney complications are infrequent but can occur.
Symptoms usually last approximately a month. Recurrences are not frequent but do occur.
Diagnosis of Henoch-Schonlein purpura
HSP is usually diagnosed based on the typical skin, joint, and kidney findings.
- Throat culture, urinalysis, and blood tests for inflammation and kidney function are used to suggest the diagnosis.
- A biopsy of the skin, and less commonly kidneys, can be used to demonstrate vasculitis.
- Special staining techniques (direct immunofluorescence) of the biopsy specimen can be used with microscopic examination to document antibody deposits of IgA in the blood vessels of involved tissue.
What is the treatment for Henoch-Schonlein purpura?
While HSP is generally a mild illness that resolves spontaneously, it can cause serious problems in the kidneys and bowels. The rash can be very prominent, especially on the lower extremities.
The treatment of HSP is directed toward the most significant area of involvement.
- Joint pain can be relieved by anti-inflammatory medications such as aspirin or ibuprofen (Motrin).
- Some patients can require cortisone medications, such as prednisone or prednisolone, especially those with significant abdominal pain or kidney disease.
- With more severe kidney disease, involvement called glomerulonephritis or nephritis, cyclophosphamide (Cytoxan), azathioprine (Imuran), or mycophenolate mofetil (Cellcept) have been used to suppress the immune system.
- Infection, if present, can require antibiotics.
What are complications of Henoch-Schonlein purpura?
HSP can have complications, which generally occur more frequently in children than in adults. These complications include
- severe abdominal pain and
- gastrointestinal bleeding.
- Adults can have extended kidney problems.
What is the prognosis for Henoch-Schonlein purpura?
The prognosis (outlook) for patients with HSP is generally excellent. Nearly all patients have no long-term problems.
- The kidney is the most serious organ involved when it is affected.
- Rarely, patients can have serious long-term kidney damage or an abnormal bowel folding called intussusception.
- Some patients have recurrences of symptoms, particularly skin rash, for months to a year after the onset of the illness.
Ruddy, Shaun, et al., eds. Kelley's Textbook of Rheumatology. Philadelphia: W.B. Saunders Co., 2000.
Top Henoch-Schonlein Purpura Related Articles
Abdominal PainAbdominal pain can have many causes that range from mild to severe. Some of these causes include bloating, gas, colitis, endometriosis, food poisoning, GERD, IBS (irritable bowel syndrome), ovarian cysts, abdominal adhesions, diverticulitis, Crohn's disease, ulcerative colitis, gallbladder disease, liver disease, and cancers. Signs and symptoms of the more serious causes include dehydration, bloody or black tarry stools, severe abdominal pain, pain with no urination or painful urination. Treatment for abdominal pain depends upon the cause.
aspirinAspirin (Aspirin, Arthritis Foundation Safety Coated Aspirin, Bayer Aspirin, Bayer Children's Aspirin, Ecotrin, and many others) is a NSAID used to treat fever, pain, and inflammation in the body that results from forms of arthritis, and soft tissue injuries. Aspirin is also used for decreasing the risk of heart attacks and strokes. Side effects, drug interactions, pregnancy information, and pregnancy safety information should be reviewed prior to taking any medication.
FeverAlthough a fever technically is any body temperature above the normal of 98.6 F (37 C), in practice, a person is usually not considered to have a significant fever until the temperature is above 100.4 F (38 C). Fever is part of the body's own disease-fighting arsenal; rising body temperatures apparently are capable of killing off many disease-producing organisms.
Arthritis (Joint Inflammation)Arthritis is inflammation of one or more joints. When joints are inflamed they can develop stiffness, warmth, swelling, redness and pain. There are over 100 types of arthritis, including osteoarthritis, rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, and gout.
Connective Tissue (CT) DiseaseConnective tissue disease is when the body's connective tissues come under attack, possibly becoming injured by inflammation. Inherited connective tissue diseases include Marfan syndrome and Ehlers-Danlos syndrome. Systemic lupus erythematosus, rheumatoid arthritis, scleroderma, polymositis, and dermatomyositis are examples of connective tissue diseases that have no known cause.
Cortisone InjectionCortisone injections are used to treat small areas of inflammation or widespread inflammation throughout the body. There is minimal pain from these injections, and relief from the pain of inflammation occurs rapidly.
Henoch-Schonlein Purpura PictureHenoch-Schonlein purpura. Henoch-Schonlein purpura (HSP) is a disease that involves inflammation which causes blood vessels in the skin, intestines, kidneys, and joints to start leaking. The main symptom is a rash with numerous small bruises, which have a raised appearance, over the legs or buttocks. Other symptoms can include joint and abdominal pain as well as kidney impairment. HSP most commonly occurs in children and in boys more than girls. Adults who develop it generally experience more severe cases.
Hirschsprung Disease (Symptoms, Causes, and Treatments)
Hirschsprung disease is an inherited condition that is present at birth (congenital) in which the nerves of parts of the large intestine are missing. The primary symptom is constipation. The diagnosis of Hirschsprung disease is made by examining the newborn or child, genetic testing, and other test results. Treatment for Hirschsprung disease is surgery, either pull-through procedure for newborns or ostomy for children. Most newborns and toddlers feel much better after surgery.
Other information about Hirschsprung disease.
Hirschsprung disease is a genetic, or inherited, condition. Other symptoms in newborns and toddlers are:
- Diarrhea, often with blood.
- Green or brown vomit
- Abdominal distension
- Nausea and vomiting
- Weight loss
- Failure to thrive in infancy
- Intestinal obstruction
- Slow growth
- Intellectual disability
The only treatment for Hirschsprung disease is surgery. Doctors and surgeons treat newborns with a pull-through procedure in which the surgeon removes the part of the large intestine that is missing nerves and connects it to the healthy part of the anus. Toddlers and children require ostomy surgery, in which part of the intestine is brought through the abdominal wall so that feces can leave the body without passing through the anus. The opening in the abdominal wall is called a stoma, and a removable external pouch is attached to it.
Complications can occur with either type of surgery, and may include:
- Narrowing of the anus
- Delayed toilet training
- Stool leaking from the anus
Hirschsprung disease can be a medical emergency that requires surgery. If your newborn or child has these symptoms listed, contact your OB/GYN or Pediatrician urgently.
NIH; National Institute of Diabetes and Digestive Diseases. "Hirschsprung Disease." Updated: Sep 2015.
Genetic Home Reference. "Hirschsprung disease." Updated: Jun 27, 2017.
NCBI. "Hirschsprung Disease Overview." Updated: Oct 1, 2015.
NIH; National Center for Advancing Translational Sciences; GARD. "Hirschsprung's disease." Updated: Jun 01, 2017.
ibuprofenIbuprofen is a nonsteroidal anti-inflammatory drug (NSAID) used to reduce mild to moderate pain, inflammation, and fever. Ibuprofen works by blocking an enzyme that makes prostaglandin (a hormone-like substance that participates in a variety of body functions), which results in lower levels of prostaglandins in the body. Lower levels of prostaglandins reduce pain, inflammation, and fever. Ibuprofen is prescribed to treat diseases and conditions that cause mild to moderate pain, fever, and inflammation.
mycophenolate mofetil (CellCept)Mycophenolate mofetil (CellCept) is a drug prescribed for the prophylaxis of organ rejection in people receiving kidney, heart, or liver transplants. Common side effects include diarrhea, vomiting, pain, pain in the stomach area, high blood pressure, and swelling of the lower legs, ankles, or feet.
Prednisone is a drug that belongs to the corticosteroid drug class, and is an anti-inflammatory and immune system suppressant. It's used to treat a variety of diseases and conditions, for example: inflammatory bowel disease (Crohn's disease and ulcerative colitis), lupus, asthma, cancers, and several types of arthritis.
Common side effects are weight gain, headache, fluid retention, and muscle weakness. Other effects and adverse events include glaucoma, cataracts, obesity, facial hair growth, moon face, and growth retardation in children. This medicine also causes psychiatric problems, for example: depression, insomnia, mood swings, personality changes, and psychotic behavior. Serious side effects include reactions to diabetes drugs, infections, and necrosis of the hips and joints.
Skin RashThe word "rash" means an outbreak of red bumps on the body. The way people use this term, "a rash" can refer to many different skin conditions. The most common of these are scaly patches of skin and red, itchy bumps or patches all over the place.
Rheumatology and RheumatologistRheumatology is the study of rheumatic diseases and conditions. Rheumatologists are internal medicine physicians who treat these illnesses, in particular arthritis.
Skin BiopsyDuring a skin biopsy, a piece of skin is removed under a local anesthesia and examined using a microscope. The different types of skin biopsy include shave biopsy, punch biopsy, and excisional biopsy. Skin biopsies are performed to diagnose skin growths, skin conditions, and skin cancers.
Urinalysis (Urine Test)Urinalysis (urine test, drug test) is a test performed on a patient's urine sample to diagnose conditions and diseases such as urinary tract infection, kidney infection, kidney stones, and inflammation of the kidneys, or screen for progression of conditions such as diabetes and high blood pressure.
VasculitisVasculitis (arteritis, angiitis) is a general term for a group of uncommon diseases which feature inflammation of the blood vessels. Each form of vasculitis has its own characteristic pattern of symptoms. The diagnosis of vasculitis is definitively established after a biopsy of involved tissue demonstrates the pattern of blood vessel inflammation. Treatment is directed toward decreasing the inflammation of the arteries and improving the function of affected organs.