Schönlein-Henoch Syndrom des Kindesalters – Gewöhnliche und ungewöhnliche Beschwerden, Komplikationen, Finkelstein-Seidlmayer Variante, und Behandlung

Autor: Mara Bernasconi, Daniel Blumberg, Mattia Rizzi, Barbara S. Bucher, Luca Garzoni, Mario G. Bianchetti, Elisabetta Fiore
Rok vydání: 2008
Předmět:
Zdroj: Therapeutische Umschau. 65:269-277
ISSN: 1664-2864
0040-5930
DOI: 10.1024/0040-5930.65.5.269
Popis: Although Henoch-Schonlein syndrome can occur at any age, it is overwhelmingly a disease of childhood. Indeed, Henoch-Schonlein syndrome is the most common vasculitis that affects children. The clinical features of this vasculitis are well documented, and the diagnosis is generally not difficult. This article briefly reviews both common and uncommon clinical aspects of the condition and information concerning therapy. A further focus of this review is recent information concerning abnormalities of immunoglobulin IgA1 glycosylation and the role of aberrantly glycosylated immunoglobulins in the development of Henoch-Schonlein syndrome. The final focus of the article is acute hemorrhagic edema, a benign vasculitis limited to the skin, which is characterized by circinate, medallion-like purpura, and ecchymoses and occurs in children younger than 4 years of age. The nosologic position of acute hemorrhagic edema, which has also been called Finkelstein-Seidlmayer syndrome, as a variant of Henoch-Schonlein syndrome is the subject of considerable debate, but most authors agree that there are sufficient clinical and prognostic differences to consider it a separate entity.
Databáze: OpenAIRE