Prevalence of the MEFV gene mutations and their clinical correlations in Azeri Turkish patients with childhood Henoch-Schonlein purpura: The role of M680I and E148Q mutations

Abstract

Henoch-Shonlein purpura (HSP) is the most common systemic vasculitis of small vessels in childhood leading to involvement of the skin, gastrointestinal (GI) tract, joints, and kidneys. 1 The incidence of this disease is 12.9-15.0 per 100000 children. 2 Its peak incidence is between 2-8 years of age and is more common in males than females. 3 Clinical signs include nonthrombocytopenic purpura with mass distribution in lower extremities and buttocks, arthritis, especially in large joints, GI symptoms, and renal involvement. 3,4 The cause is unknown, but infectious agents, medications, food allergies and insect bites have been implicated in its occurrence. 5