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Causes and symptoms
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).
allergic vasculitisA form of nonthrombocytopenic purpura due to a hypersensitivity vasculitis associated with urticaria, erythema, arthritis, gastrointestinal symptoms and renal involvement.
al·ler·gic pur·pu·ra(ă-lĕr'jik pŭr'pyŭr-ă)
Synonym(s): anaphylactoid purpura (1) .
|Mean LOS:||5 days|
|Description:||MEDICAL: Coagulation Disorders|
Allergic purpura is an allergic reaction that leads to acute or chronic inflammation of the vessels of the skin, joints, gastrointestinal (GI) tract, and genitourinary (GU) tract. It occurs as an acquired, abnormal immune response to a variety of agents that normally do not cause allergy, and it is manifested by bleeding into the tissues, organs, and joints, which leads to organ dysfunction, discomfort, and immobility. An acute attack of allergic purpura can last for several weeks, but usually episodes of the disease subside without treatment within 1 to 6 weeks. Patients with chronic allergic purpura can have a persistent and debilitating disease. The most severe complications are acute glomerulonephritis and renal failure. Hypertension often complicates the course, and if bleeding is excessive, the patient can develop a fluid volume deficit. On rare occasions, patients may be at risk for airway compromise from laryngeal edema.
Allergic purpura occurs in response to agents such as bacteria, drugs, food, or bee stings. The allergic reaction, probably an autoimmune response directed against the vessel walls, may be triggered by a bacterial infection. Most patients have experienced an upper respiratory infection, particularly a streptococcal infection, 1 to 3 weeks prior to the development of allergic purpura. Experts suggest that other causes, such as allergic reactions to drugs and vaccines, insect bites, and foods (wheat, eggs, chocolate, milk) may lead to the condition.
Over 20 genes have been associated with the development of allergy, and mutations of these may be passed on in families. Family studies indicate that if both parents suffer from allergies, the allergy risk in their offspring is 80%. Implicated genes include the IL-4, IL-4 receptor, INF-γ, β-adrenergic receptor, 5 lipoxygenase, PHF1, TARC, and leukotriene C4 synthetase genes. Some alleles of the MEFV and ACE genes along with various HLA alleles are also associated with allergic purpura.
Gender, ethnic/racial, and life span considerations
Allergic purpura can occur at any age, but it is most common in children between 3 and 10 years old; the condition is more common in males than in females. In North America, the disease occurs mostly from November to January. In one-half to two-thirds of children, an upper respiratory tract infection precedes the clinical onset by 1 to 3 weeks, and children are mildly ill with a fever. Whites are more affected than blacks/African Americans.
Global health considerations
No data are available.
Approximately one to two out of four patients with allergic purpura have GU symptoms such as dysuria and hematuria. Other symptoms include headaches; fever; peripheral edema; and skin lesions accompanied by pruritus, paresthesia, and angioedema (swelling of the skin, mucous membranes, or organs). Other patients describe severe GI symptoms (spasm, colic, constipation, bloody vomitus, bloody stools) and joint pain.
Inspect the patient’s skin for the typical skin lesions—patches of purple macular lesions of various sizes that result from vascular leakage into the skin and mucous membranes. These lesions most commonly occur on the hands and arms. Note that in children, the lesions more commonly start as urticarial areas that then expand into hemorrhagic lesions. Determine if the patient has any peripheral swelling, particularly in the hands and face. Perform gentle range of motion of the extremities to determine the presence and location of joint pain. Assess the color of the patient’s urine and stool, and note any bleeding.
The patient may experience a disturbance in body image because of the disfigurement caused by the rash and swelling. Determine the patient’s response to her or his appearance and identify whether the changes interfere with implementing various roles, such as parenting or work.
No single laboratory test identifies allergic purpura. Supporting tests include complete blood count, erythrocyte sedimentation rate, urinalysis, blood urea nitrogen, creatinine, and coagulation profile.
Primary nursing diagnosis
DiagnosisImpaired skin integrity related to damage and inflammation of vessels
OutcomesTissue integrity: Skin and mucous membranes; Wound healing; Body image
InterventionsSkin surveillance; Wound care; Body image enhancement
Planning and implementation
The treatment prescribed is based on the acuity and severity of the symptoms. Some patients are treated pharmacologically with corticosteroids to relieve edema and analgesics to manage joint and GI discomfort. Allergy testing to identify the provocative allergen is usually performed. If the allergen is a food or medication, the patient needs to avoid ingesting the allergen for the rest of his or her life. Patients who are placed on corticosteroids or immunosuppressive therapy need an environment that protects them as much as possible from secondary infection. If the patient is on corticosteroids, monitor her or him for signs of Cushing’s syndrome and the complications of corticosteroids, such as labile emotions, fluid retention, hyperglycemia, and osteoporosis.
|Medication or Drug Class||Dosage||Description||Rationale|
|Glucocorticoids||Varies by drug||Corticosteroid||Relieve edema and analgesics to manage joint and GI discomfort|
|Azathioprine||Varies by age and whether it is initial or maintenance dose||Immunosuppressant||Suppresses cell-mediated hypersensitivity|
If the patient has alterations in coagulation, create a safe environment to prevent bleeding from falls or other injuries. Protect open or irritated skin lesions from further tissue trauma and infection. Apply unguents and soothing creams, if appropriate, to manage discomfort. Assist the patient with colloidal baths and activities of daily living if joint pain and the lesions give the patient limited activity tolerance. Reassure the patient that the lesions are of short duration prior to healing. Explore possible sources of the allergy. Allow the patient time to discuss concerns about the disease. If the patient or significant other appears to be coping ineffectively, provide a referral to a clinical nurse specialist or counselor.
Evidence-Based Practice and Health Policy
Watson, L., Richardson, A. R., Holt, R. C., Jones, C., & Beresford, M. W. (2012). Henoch Schonlein Purpura―a 5-year review and proposed pathway. PLoS One, 7(1), e29512. Online publication. doi 10.1371/journal.pone.0029512
- Investigators conducted a cohort study to explore the presentation of allergic (Henoch Schonlein) purpura, in which 102 children were diagnosed (incidence was 6.21 cases per 100,000 children in this cohort).
- Among the children diagnosed with allergic purpura, 100% presented with a palpable rash, 45% presented with arthralgia, 19% presented with joint swelling, 9% presented with abdominal pain, and 5% presented with hyperthermia.
- Hypertension was present in 14% of patients, of which 28% had proteinuria and 7% had both proteinuria and hematuria. Additionally, 46% of the diagnosed children had initial renal inflammation.
- The presence of proteinuria at day 7 postdiagnosis had a positive predictive value of 32% (95% CI, 15% to 55%) and a negative predictive value of 97% (95% CI, 90% to 99%) in identifying children at risk for renal complications, including nephritis.
- Extent, location, and description of erythema; degree of discomfort; signs of wound infection; presence and description of edema
- Response to allergy testing and withdrawal of the provocative agent if identified
- Response to treatments: Medications, creams, and colloidal baths
- Emotional response to the condition; problems coping; body image disturbance
Discharge and home healthcare guidelines
prevention.Teach the patient about the disease and its cause. If the allergen is identified, assist the patient in eliminating the allergen if possible. Teach the patient to protect lesions from additional trauma by wearing long-sleeved blouses or shirts. Teach the patient to pay particular attention to edematous areas where skin breaks down easily if injured. Encourage the patient to prevent secondary infections by avoiding contact with others and by using good hand-washing techniques. Encourage the patient to report recurrent signs and symptoms, which are most likely to occur 6 weeks after the initial onset of symptoms.
medications.Provide the patient with information about the medications, including dosage, route, action, and side effects. Provide the patient with written information so that the patient can refer to it for questions at home.
al·ler·gic pur·pu·ra(ă-lĕr'jik pŭr'pyŭr-ă)
Synonym(s): anaphylactoid purpura (1) .