HISTORY The disease is named after Eduard Heinrich Henoch (18201910), a German pediatrician, and his teacherJohann Lukas

Schnlein (17931864), who described it in the 1860s. The English physician William Heberden(17101801) and the dermatologist Robert Willan (17571812) had already described the disease in 1802 and 1808, respectively, but the name HeberdenWillan disease has fallen into disuse. William Osler was the first to recognise the underlying allergic mechanism of HSP. DEFINITION y

a systemic vasculitis (inflammation of the blood vessels)

immune response

Also called anaphylactoid purpura - resembling anaphylaxis, but not involving HSP is a systemic vasculitic syndrome with: Palpable purpura -from Latin: purpura, meaning "purple") is the appearance of red or purple discolorations on the skin that do not blanch on applying pressure. They are caused by bleeding underneath the skin 2 to 10 mm in diameter Arthritis -form of joint disorder that involvesinflammation of one or more joints. Abdominal pain (or stomach ache) can be one of the symptoms associated with transient disorders or serious disease; w/n & v Hematuria,

gastrointestinal hemorrhage

EPIDEMIOLOGY 90% of cases reported in children Peak in children aged 4-7 Male:Female (1.5:1) 50% follow a URTI Renal disease is more severe in adults Pathophysiology Possible causes: infections, autoimmunity, vaccinations, drugs caused by an abnormal response of the immune system. It is unclear why this occurs. Henoch-Schnlein purpura is thought to be an immunoglobulin A (IgA)mediated autoimmune phenomenon. An unknown antigenic stimulant has been postulated to cause a rise in IgA. The antigen-antibody complexes deposit locally throughout the body and activate pathways leading to necrotizing vasculitis. IgA- produced in mucosal linings than all other types of antibody combined; immunoglobulin found in mucous secretions, including tears, saliva,colostrum and

secretions from the genitourinary tract, gastrointestinal tract,prostate and respiratory epithelium. Medications Nonsteroidal anti-inflammatory drugs (NSAIDs) lessen joint pain and arthritis Ibuprofen (Ibuprin, Advil, Motrin) Flurbiprofen (Ansaid) Ketoprofen (Oruvail, Orudis, Actron) Naproxen (Anaprox, Naprelan, Naprosyn) Corticosteroid for significant abdominal pain or kidney disease Prednisolone (Cortisone) Prednisone (Deltasone, Sterapred, Orasone) y
y

Antibioticsto treat infection

Cyclophosphamide ( Cytoxan )to suppress the immune system when you have symptoms of severe kidney disease

Medical/Surgical Interventions Diagnosis y Blood tests -elevated creatinine and urea levels (in kidney involvement) -

(kidney

failure, azotemia)
-raised IgAlevels (in about 50%) Immunoglobulin A (IgA) is an antibody that plays a critical role in mucosal immunity. - raised CRP or erythrocyte sedimentation rate -non-specific measure of inflammation - platelet count may be raised (differentiates idiopathic thrombocytopenic purpura and thrombotic thrombocytopenic purpura) Skin biopsy - vasculitis - inflammatorydestruction of blood vessels. y y y Renal biopsy -Kidney involvement Urinalysis (hematuria, proteinuria) Stool sample

NCP
Impaired tissue integrity related to increase vascular permeability secondary to bacterial infection as evidenced by palpable Purpuric rashes , lower extremities edematous (especially feet), febrile Independent Monitor vital signs note for elevated temperature and increase RR Assist patient for TSB

 Encourage increase intake of fluids Emphasize good hand washing technique for all individual coming in contact with client Instruct SO to prevent skin to skin surface contact Instruct SO to provide freshly laundered bed linens. Keep skin free from pressure Elevate lower extremities if possible/appropriate avoidance of lotions or soap that may irritable the skin Collaborative Refer to nutritional support team

Science News Contaminated Cocaine Triggers Decaying, Dying Skin

ScienceDaily (June 23, 2011) If the obvious reasons for avoiding recreational drug use aren't off-putting enough, physicians have yet another detrimental consequence to add to the list -- crusty, purplish areas of dead skin that are extremely painful and can open the door to nasty infections.

The condition is called purpura. Typical causes include a range of rare disorders, but it is also associated with the use of cocaine. Not just any cocaine, though: Physicians, researchers and health officials believe cocaine contaminated with a de-worming drug commonly used by veterinarians is the culprit. The drug, called levamisole, was found in 30 percent of confiscated cocaine in 2008 and 70 percent in 2009, according to the U.S. Drug Enforcement Administration. In the Journal of the American Academy of Dermatology, physicians highlight six new and very similar patient cases of purpura, mostly on and around the ears, following cocaine use. The cases -- four seen in Rochester, N.Y., and two in Los Angeles -- closely resemble two additional cases in San Francisco that were reported previously in the journal. In each case an extensive battery of blood tests ruled out the usual causes of purpura. The cases were reported by the University of Rochester Medical Center and the University of California, Los Angeles. Because testing for traces of levamisole in the blood is complex and unreliable, researchers cannot say for sure that it is the direct cause of purpura in these instances. But, due to the striking similarity of these cases, and the presence of another condition caused by levamisole called agranulocytosis -- low blood counts that up the risk of infection -- in the majority of the patients, doctors say there is strong reason to suspect the drug and to focus greater attention on what could become a widespread health concern. "We believe these cases of skin reactions and illnesses linked to contaminated cocaine are just the tip of the iceberg in a looming public health problem posed by levamisole," said the study authors. According to Mary Gail Mercurio, M.D., an author and associate professor in the Department of Dermatology at the University of Rochester Medical Center, "When we first started seeing these patients they all had a similar clinical picture, but they were really an enigma because they weren't falling into any other pattern we'd seen before. When a colleague at the National Institutes of Health mentioned levamisole contamination, we did toxicity screens and lo-and-behold, all the patients came up positive for cocaine. We had our diagnosis."

Drug enforcement officials have detected levamisole -- which was once used to treat colon cancer -- in cocaine since 2003, but have watched it increase rapidly in recent years. The Drug Enforcement Administration says that the drug, which is inexpensive, is used more and more as a diluting agent in order to stretch supplies. Study authors report that levamisole is known to increase dopamine, a neurotransmitter that helps control the brain's reward and pleasure centers, causing experts to believe it is also added to cocaine to further enhance or prolong the user's high. Researchers don't know how levamisole causes purpura, which occurs when vessels become plugged and blood can't flow to the skin, leading to skin death and the resulting purplish, crusty appearance. Cocaine alone constricts blood vessels, which is probably the first step, but how levamisole contributes is not yet understood, Mercurio said. Both smoking and snorting tainted cocaine can lead to purpura and both men and women can be affected. Treatment options include steroids to prevent inflammation, but stopping the exposure to cocaine is the best medicine: Mercurio and the other study authors observed that once patients stopped using cocaine, the purpura and low blood counts improved. "We've seen a lot of cases in Rochester alone, so it is important to alert the gatekeepers of medicine, the primary care physicians who are in the trenches every day, of this diagnosis," said Mercurio. "This is one of those entities that with familiarity and recognition can go a long way in helping physicians to quickly make a diagnosis and intervene without embarking on an elaborate workup where nothing will pan out." In addition to Mercurio, Catherine Chung, M.D., a resident in the Department of Dermatology at the Medical Center, also contributed to the research. Ghinwa K. Dumyati, M.D.,associate professor in the Department of Medicine, identified two of the four patients from Rochester, and these patients were recently discussed in the internal medicine literature. From the University of California Los Angeles, Paul C. Tumeh, M.D., Ron Birnbaum, M.D., Belinda H. Tan, M.D., Ph.D., Linda Sharp, M.D., Erin McCoy, M.D., and Noah Craft, M.D., Ph.D., also participated.

http://www.sciencedaily.com/releases/2011/06/110623151225.htm