Henoch-Schonlein purpura


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purpura

 [per´pu-rah]
a hemorrhagic disease characterized by extravasation of blood into the tissues, under the skin, and through the mucous membranes, and producing spontaneous bruises, ecchymoses, and petechiae (small hemorrhagic spots) on the skin. (See plate in Dermatology Atlas.) When accompanied by a decrease in the circulating platelets, it is called thrombocytopenic purpura; when there is no decrease in the platelet count, it is called nonthrombocytopenic purpura. adj., adj purpu´ric.

There are two general types of thrombocytopenic purpura: primary or idiopathic, in which the cause is unknown, and secondary or symptomatic, which may be associated with exposure to drugs or other chemical agents, systemic diseases such as multiple myeloma and leukemia, diseases affecting the bone marrow or spleen, and infectious diseases such as rubella (German measles).
Symptoms. The outward manifestations and laboratory findings of primary and secondary thrombocytopenic purpura are similar. There is evidence of bleeding under the skin, with easy bruising and the development of petechiae. In the acute form there may be bleeding from any of the body orifices, such as hematuria, nosebleed, vaginal bleeding, and bleeding gums. The platelet count is below 100,000 per cubic millimeter of blood and may go as low as 10,000 per cubic millimeter (normal count is about 250,000 per cubic millimeter). The bleeding time is prolonged and clot retraction is poor. Coagulation time is normal.
Treatment. Differential diagnosis is necessary to determine the type of purpura present and to eliminate the cause if it can be determined. General measures include protection of the patient from trauma, elective surgery, and tooth extractions, any one of which may lead to severe or even fatal hemorrhage. In the thrombocytopenic form, corticosteroids may be administered when the purpura is moderately severe and of short duration. Splenectomy is indicated when other, more conservative measures fail and is successful in a majority of cases. In some instances, especially in children, there may be spontaneous and permanent recovery from idiopathic purpura. (See Atlas 2, Part Q.)
allergic purpura (anaphylactoid purpura) Henoch-Schönlein purpura.
annular telangiectatic purpura a rare form in which pinpoint reddened lesions coalesce to form a ring-shaped or continuously linked pattern, commonly beginning in the lower extremities and becoming generalized. Called also Majocchi's disease.
fibrinolytic purpura purpura associated with increased fibrinolytic activity of the blood.
purpura ful´minans a form of nonthrombocytopenic purpura seen mainly in children, usually after an infectious disease, marked by fever, shock, anemia, and sudden, rapidly spreading symmetrical skin hemorrhages of the lower limbs, often associated with extensive intravascular thromboses and gangrene.
purpura hemorrha´gica primary thrombocytopenic purpura.
Henoch's purpura a type of Henoch-Schönlein purpura in which abdominal symptoms predominate.
Henoch-Schönlein purpura a type of nonthrombocytopenic purpura, of unknown cause but thought to be due to a vasculitis; it is most often seen in children and is associated with clinical symptoms such as urticaria and erythema, arthritis and other joint diseases, gastrointestinal symptoms, and renal involvement. Called also Schönlein-Henoch purpura or syndrome and allergic or anaphylactoid purpura.
idiopathic thrombocytopenic purpura thrombocytopenic purpura not directly associated with any definable systemic disease, although it often follows a systemic infection; the cause is thought to be an IgG immunoglobulin that acts as an antibody against platelets, causing ecchymoses, petechiae, and other bleeding. There are both acute and chronic forms: the acute form has a sudden onset, is more common in children, and usually resolves spontaneously within a few months; the chronic form has a slower onset, is more common in adults, and may be recurrent.
nonthrombocytopenic purpura purpura without any decrease in the platelet count of the blood. In such cases the cause is either abnormal capillary fragility or a clotting factor deficiency.
Schönlein purpura a type of Henoch-Schönlein purpura in which articular systems predominate; called also Schönlein's disease.
Schönlein-Henoch purpura Henoch-Schönlein purpura.
purpura seni´lis dark purplish red ecchymoses occurring on the forearms and backs of the hands in the elderly.
thrombocytopenic purpura purpura associated with a decrease in the number of platelets in the blood; see purpura.
thrombotic thrombocytopenic purpura a form of thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, bizarre neurological manifestations, uremia (azotemia), fever, and thromboses in terminal arterioles and capillaries; some authorities consider it identical to the hemolytic uremic syndrome. Called also microangiopathic hemolytic anemia and Moschcowitz's disease.

He·noch-Schön·lein pur·pu·ra

(he'nok shĕrn'līn),
an eruption of nonthrombocytopenic, palpable purpuric lesions due to dermal leukocytoclastic vasculitis with IgA in vessel walls associated with joint pain and swelling, colic, and passage of bloody stools, and occurring characteristically in young children; glomerulonephritis may occur during an initial episode or develop later.

Henoch-Schönlein purpura

[hen′ôkh shœn′līn]
Etymology: Eduard H. Henoch, German physician, 1820-1910; Johannes L. Schönlein, German physician, 1793-1864
a self-limited hypersensitivity vasculitis, chiefly of children, characterized by purpuric skin lesions that appear predominantly on the lower abdomen, buttocks, and legs and are usually associated with pain in the knees and ankles. Other joint involvement, GI bleeding, and hematuria are also common findings. The disease lasts up to 6 weeks and has no sequelae if renal involvement is not severe. Immunosuppressive drugs, such as corticosteroids, may help relieve the nephropathy. Also called anaphylactoid purpura, Henoch-Schönlein syndrome, Schönlein-Henoch purpura.

Henoch-Schönlein purpura

Anaphylactoid purpura, vascular purpura Internal medicine An acquired form of small vessel vasculitis with IgA-dominant immune deposits affecting small vessels–arteries, capillaries, venules, typically of the skin, gut, and glomeruli, associated with arthralgia and/or arthritis; HSP is most common in younger Pts Clinical Red maculopapules on legs and buttocks, glomerulonephritis, abdominal pain ± infarction, diarrhea, fever, arthritis Lab IgA deposits in basement membrane of skin and glomerulus Treatment NSAIDs, aspirin, steroids–eg, prednisone, especially in Pts with major abdominal pain or kidney disease Prognosis Usually self limited. See Small vessel vasculitis, Systemic vasculitis.

He·noch-Schön·lein pur·pu·ra

(hen'awk shĕrn'līn pŭr'pyŭr-ă)
An eruption of nonthrombocytopenic purpuric lesions due to dermal leukocytoclastic vasculitis with IgA in vessel walls associated with joint pain and swelling, colic, and passage of bloody stools, and occurring characteristically in young children; glomerulonephritis may occur during an initial episode or develop later.
Synonym(s): anaphylactoid purpura (2) , Henoch purpura, Schönlein purpura.

Henoch-Schönlein purpura

(hen'ok-shan'lin)
[Eduard H. Henoch, Ger. pediatrician, 1820–1910; Johann Lukas Schönlein, Ger. physician, 1793–1864]
Enlarge picture
HENOCH-SCH~Ouml`NLEIN PURPURA: Characteristic rash on the buttocks
A form of small vessel vasculitis of unknown cause that affects children, esp. between the ages of 3 and 5, more often than adults. It is marked by a purpuric rash on the buttocks and legs, and, in some patients, abdominal pain or gastrointestinal bleeding, polyarticular joint disease, and renal involvement (e.g., glomerulonephritis). The illness usually lasts about 2 weeks before resolving spontaneously. In some instances (more commonly in adults than in children) renal failure can complicate the illness. Synonym: anaphylactoid purpura See: illustration

Treatment

Joint symptoms respond to rest and administration of nonsteroidal anti-inflammatory drugs. Corticosteroid drugs, such as prednisone, are used to treat patients with severe gastrointestinal or renal involvement. Hemodialysis is used to support patients who develop chronic renal failure.

Henoch,

Eduard Heinrich, German pediatrician, 1820-1910.
Henoch chorea - a disorder in which sudden spasmodic coordinated movements of certain muscles or groups of physiologically related muscles occur at irregular intervals. Synonym(s): spasmodic tic
Henoch purpura - Synonym(s): Henoch-Schönlein purpura
Henoch-Schönlein purpura - an eruption of nonthrombocytopenic purpuric lesions due to dermal leukocytoclastic vasculitis. Synonym(s): acute vascular purpura; anaphylactoid purpura; Henoch purpura; Henoch-Schönlein purpura; Henoch-Schönlein syndrome; purpura nervosa; purpura rheumatica; Schönlein disease; Schönlein purpura
Henoch-Schönlein syndrome - Synonym(s): Henoch-Schönlein purpura
Schönlein-Henoch syndrome - Synonym(s): Henoch-Schönlein purpura

Schönlein,

Johann Lukas, German physician, 1793-1864.
Henoch-Schönlein purpura - see under Henoch
Henoch-Schönlein syndrome - Synonym(s): Henoch-Schönlein purpura
Schönlein disease - Synonym(s): Henoch-Schönlein purpura
Schönlein purpura - Synonym(s): Henoch-Schönlein purpura
Schönlein-Henoch syndrome - Synonym(s): Henoch-Schönlein purpura

He·noch-Schön·lein pur·pu·ra

(hen'awk shĕrn'līn pŭr'pyŭr-ă)
Eruption of nonthrombocytopenic, palpable, purpuric lesions due to dermal leukocytoclastic vasculitis with IgA in vessel walls associated with joint pain and swelling, colic, and bloody stools; occurring characteristically in young children.
Synonym(s): anaphylactoid purpura (2) .
References in periodicals archive ?
Prevalence and significance of mutations in the familial Mediterranean fever gene in Henoch-Schonlein purpura.
Cerebral vasculitis in Henoch-Schonlein purpura : a case report with sequential magnetic resonance imaging.
The American College of Rheumatology 1990 criteria for the classification of Henoch-Schonlein purpura.
Previously, during January 2002-June 2006, a total of 173 serum samples had been obtained from children (age range 1 month-15 years) with hepatitis (hepatitis B, 20 samples; hepatitis C, 11 samples; unknown hepatitis, 31 samples), Kawasaki disease (12 samples), and Henoch-Schonlein purpura (18 samples) and from healthy children (same age range, 81 samples) (9).
Review of systems revealed no gastrointestinal, joint or skin symptoms to suggest Henoch-Schonlein purpura.
Vasculitis involving the small vasculature includes Henoch-Schonlein purpura, essential cryoglobulinemia, hypersensitivity vasculitis, vasculitis secondary to an autoimmune connective tissue disease, vasculitis secondary to viral infection, and vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA), including Wegener's granulomatosis, (3, 4) Churg-Strauss granulomatosis, (2, 5, 6) microscopic polyangiitis (MPA), (7, 8) and the renal limited form of MPA sometimes known as pauci-immune glomerulonephritis or idiopathic crescentic glomerulonephritis.
CASE FACTS: Thomas Phibbs, a 16-year-old adolescent, was diagnosed with Henoch-Schonlein Purpura with acute renal failure and other medical conditions.
Andrew, from Wombourne, Wolverhampton, was diagnosed with Henoch-Schonlein purpura - an inflammation of the blood vessels which affected his kidneys - in July 2002.
Dermatological diseases in which Koebner's phenomenon occurs Contact dermatitis Molluscum contagiosum Darier's disease Pellagra Dermatographism Perforating disorders Eruptive xanthoma Pityriasis rubra pilaris Erythema multiforme Porokeratosis Henoch-Schonlein purpura Psoriasis * Kyrle's disease Reactive perforating collagenosis Lichen nitidus Rhus dermatitis * Lichen planus * Verrucae * Lichen sclerosus et atrophicus Vitiligo * Most frequently seen Table 2.
Increased IgA-producing cells in the blood of patients with active Henoch-Schonlein purpura.