[Schönlein-Henoch syndrome]

Abstract

Schönlein-Henoch's syndrome is particularly seen between the ages of two and eight years and is twice as frequent in boys as in girls. In up to 80%, symptoms in the form of skin rash, arthritis and abdominalia are observed. The pathognomonic symptom is the skin lesion which most often is confined to the region below the waist and is the first symptom in more than 50%. Incorrect diagnosis particularly of abdominal symptoms may result in unnecessary laparotomy. The etiology and pathogenesis are not yet completely elucidated but it is believed that SHS is caused by an IgA-mediated immune response to a variety of foreign antigens. The treatment and the prophylaxis, particularly concerning the nephropathy, of SHS is still very deficient today. Depending on the nature and the degree of difficulty the treatment is: salicylates, NSAID, prednisone, cytotoxica, plasmapheresis, substitution with factor 13-concentrate and, in terminal renal failure, allotransplantation renis. The course and the prognosis is usually good in cases without nephropathy. In mild cases, the disease lasts a few days, in more serious cases 4-6 weeks with subsequent exacerbations and remissions. Patients who have had SHS-nephritis ought to be followed for at least five years with an annual examination of the blood pressure and urine test for protein and blood. SHS ought to be considered as differential diagnosis in acute cases of abdominal pains, arthralgia, hematuria and CNS-symptoms even if the characteristic skin symptoms are missing.