Henoch–Schönlein purpura
| Henoch-Schönlein purpura | |
|---|---|
 |
|
| Typical purpura on lower legs and | |
| Classification and external resources | |
| Specialty | Dermatology |
| ICD-10 |
D69.0 (ILDS D69.010) |
| ICD-9-CM | 287.0 |
| DiseasesDB | 5705 |
| MedlinePlus | 000425 |
| eMedicine | derm/177 emerg/767 emerg/845 ped/3020 |
| MeSH | D011695 |
Henoch–Schönlein purpura (HSP), also known as IgA vasculitis,anaphylactoid purpura,purpura rheumatica, and Schönlein–Henoch purpura, is a disease of the skin, mucous membranes, and sometimes other organs that most commonly affects children. In the skin, the disease causes palpable purpura (small hemorrhages), often with joint pain and abdominal pain. With kidney involvement, there may be a loss of small amounts of blood and protein in the urine (hematuria and proteinuria), but this usually goes unnoticed; in a small proportion of cases, the kidney involvement proceeds to chronic kidney disease. HSP is often preceded by an infection, such as a throat infection.
HSP is a systemic vasculitis (inflammation of blood vessels) and is characterized by deposition of immune complexes containing the antibody immunoglobulin A (IgA); the exact cause for this phenomenon is unknown. It usually resolves within several weeks and requires no treatment apart from symptom control, but may relapse in a third of cases and cause irreversible kidney damage in about one in a hundred cases.
Purpura, arthritis and abdominal pain are known as the "classic triad" of Henoch–Schönlein purpura. Purpura occur in all cases, joint pains and arthritis in 80%, and abdominal pain in 62%. Some include gastrointestinal hemorrhage as a fourth criterion; this occurs in 33% of cases, sometimes, but not necessarily always, due to intussusception. The purpura typically appear on the legs and buttocks, but may also be seen on the arms, face and trunk. The abdominal pain is colicky in character, and may be accompanied by nausea, vomiting, constipation or diarrhea. There may be blood or mucus in the stools. The joints involved tend to be the ankles, knees, and elbows, but arthritis in the hands and feet is possible; the arthritis is nonerosive and hence causes no permanent deformity. Forty percent have evidence of kidney involvement, mainly in the form of hematuria (blood in the urine), but only a quarter will have this in sufficient quantities to be noticeable without laboratory tests. Problems in other organs, such as the central nervous system (brain and spinal cord) and lungs may occur, but is much less common than in the skin, bowel and kidneys.
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