|
The clinical implications of adult-onset henoch-schonelin purpuraAbstract: Henoch-Schonlein Purpura (HSP) is a small vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement [1]. Pathologically, it can be considered a form of leukocytoclastic vasculitis that can involve not only the skin but other tissues as well. Though it primarily affects children (over 90% of cases), the occurrence in adults has been rarely reported (3.4 to 14.3 cases per million). This low incidence could be due to either under-diagnosis or misdiagnosis.Typically the disorder is commoner in males and may follow an infectious illness [2]. In the cases reported in children, the majority (of over 75%) of cases presented with an eruption, while up to 66% presented with abdominal pain and close to 50% the cases demonstrated renal involvement [2]. In children, the disorder is often self-limiting, while a more complicated course has been described in adults, including a high incidence of renal insufficiency developing in almost 50% of those patients who showed renal involvement. It appears that patients over 20 years old with bloody stools, relapsing disease and persistent eruption are more likely to progress onto complications [3]. Besides renal disease, cardiac, pulmonary, ocular, gastrointestinal and neurological complications have been described in this disorder. In that sense, this is truly a multisystem disease and may result in considerable morbidity and mortality in some patients. A variety of disorders have been associated with HSP including infection with Helicobacter pylori, hepatitis B and certain malignancies. The following review describes the course, complications and management of adult-onset HSP.A 56 year old man with prior history of hypertension and adult onset diabetes mellitus presented with a skin eruption over the lower extremities of several weeks duration (appearance of the eruption is shown in Figures 1A and 1B). T
|