HENOCH-SCHONLEIN PURPURA
Jaimie Bassett
Rasmussen College
Author Note This paper is being submitted on October 31, 2014, for Michael Mileski’s G150/PHA1500 Section 02 Structure and Function of the Human Body - 2014 Fall Quarter.
Henoch-Schonlein Purpura Henoch-Schönlein purpura is the most common form of systemic vasculitis in the pediatric setting with 90% of cases occurring in childhood between the ages of 3 and 15 years. Although diagnosis in the primary care setting may be difficult, it is vital in order to avoid significant complications. (Penny, Fleming, Kazmierczak, Thomas, 2010) There is no single diagnostic test to confirm Henoch-Schönlein purpura; diagnosis depends on recognition of clinical manifestations that vary in onset occurrences. (Lim, Cheng, Wong, 2009) Henoch-Schönlein purpura, or HSP, effects many areas of the body. It is characterized by a systemic leukocytoclastic angiitis (shows as palpable purpura.), mainly affecting the small blood vessels of the skin, joints, gastrointestinal tract and kidneys. Other organs are occasionally involved, such as the brain, lungs and scrotum. (Penny, Fleming, Kazmierczak, Thomas, 2010) The etiology of HSP is still unknown. Even though the etiology of HSP remains obscure, a variety of antigenic stimuli, including infections, drugs (e.g. cytarabine, ampicillin, erythromycin, clarithromycin, quinine, enalapril and lisinopril) toxins, systemic diseases and cancer have been incriminated. Drug-induced HSP typically resolves rapidly after discontinuation of the involved drug. (El-Husseini, Ahmed, Fabulo, 2013). Histologically, HSP exhibits an immune mediated leukocytoclastic vasculitis, with deposits of immunoglobulin A (IgA) and its immune complexes within the walls of involved vessels and organs. Patients have elevated serum levels of IgA, IgA immune complexes, IgA anticardiolipin antibodies and transforming growth factor-ß, as well as altered IgA glycosylation. (Lim, Cheng, Wong, 2009) Part of the symptoms of HSP is skin manifestations. The rash of HSP begins as erythematous (abnormal redness of skin), urticarial (itchy raised areas), and macular wheals (change in skin color) eventually becoming palpable purpura. The rash often manifests in a symmetrical pattern at pressure dependant areas, such as the lower extremities in adults and the buttocks in toddlers. In children the face, trunk, and upper extremities may be more affected. (Lim, Cheng, Wong, 2009) Another symptom of HSP is Arthralgia, otherwise known as joint pain, which occurs in 84% of HSP patients and often coexists with other symptoms. The large joints of the lower extremities are most commonly affected. Transient oligoarticular arthritis (arthritis that involves less than five joints in the first six months of disease) and peri-articular swelling (swelling around a joint) may cause pain, tenderness and restricted movement. (Lim, Cheng, Wong, 2009) This can make it very difficult and painful for the patient to walk or even stand without assistance. Renal manifestations are also a part of HSP. Renal involvement is in about 20.54% of HSP patients, ranging from isolated haematuria (blood in urine) and/or proteinuria, in which the urine contains an abnormal amount of protein, (without any abnormality in renal function and blood pressure) to acute nephropathy with renal impairment. Renal manifestations generally occur over a period of 28 days after the initial presentation of HSP. There are possible long term complications with HSP too of renal impairment. (Lim, Cheng, Wong, 2009) HSP patients also suffer from gastrointestinal
