The clinical implications of adult-onset henoch-schonelin purpura

Abstract

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. Pathologically, it can be considered a form of immune complex-mediated leukocytoclastic vasculitis (LCV) involving the skin and other organs. Though it primarily affects children (over 90% of cases), the occurrence in adults has been rarely reported. Management often involves the use of immunomodulatory or immune-suppressive regimens.

Palpable Purpura in a Vietnamese Teenage Girl

“…1 However, HSP can be seen at any age, though the occurrence in adults is rare and reported in 3.4 -14.3 cases per million. 3 The precise etiology of HSP is unknown. The proposed triggers are generally infectious; an upper respiratory infection often precedes HSP symptoms in 90% of cases.…”

“…IgA complex depositions accumulate primarily in the skin, intestinal mucosa, joints and kidneys-precisely the organ systems behind the classic clinical features of HSP. 3 The main clinical features seen with HSP are palpable purpura, abdominal pain, arthritis, and renal insufficiency. A retrospective analysis of 250 adult patients with HSP reported a purpuric rash in 96% of cases, arthritis in 61%, gastrointestinal disease in 47%, and renal disease in 32%.…”

“…2 Other proposed infectious agents include group A Streptococcus, MRSA, H. pylori, Parvovirus B 19, Hepatitis B, HIV, Stenotrophomonas maltophilia. 3 In adults, a wider variety of antigenic stimuli have also been suggested to predispose to HSP: vaccinations, insect bites, food allergies and drugs. Medication-induced HSP is particularly more common in adults and has been seen in association with ACE-inhibitors, ARBs, NSAIDs, and antibiotics.…”

“…A retrospective analysis of 250 adult patients with HSP reported a purpuric rash in 96% of cases, arthritis in 61%, gastrointestinal disease in 47%, and renal disease in 32%. 3 The classic skin lesions of HSP start as multiple small, painless, erythematous macular lesions that combine into palpable purpura. The rash is symmetrical and more prominent in dependent areas of the body such as the lower extremities.…”

Association of ACE, VEGF and CCL2 gene polymorphisms with Henoch–Schonlein purpura and evaluating the possible interaction effects of these loci in HSP patients

“…This systemic vasculitis is characterized by palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. 1,2 It has been shown that multiple different genes and their interactions with environmental factors are involved in the susceptibility to HSP. 3 Therefore, genetic background is an important factor in the pathogenesis of this immune-mediated inflammatory disease.…”