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

Abstract

Although Henoch-Schönlein syndrome can occur at any age, it is overwhelmingly a disease of childhood. Indeed, Henoch-Schönlein 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-Schönlein 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-Schönlein 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.

Prevalence and Characteristics of Nonblanching, Palpable Skin Lesions With a Linear Pattern in Children With Henoch-Schönlein Syndrome

“…To date, fewer than 10 cases of nonblanching, palpable skin lesions with a linear pattern have been reported in both children and adults with Henoch-Schönlein syndrome. [2][3][4][5][6][7][8] The incongruity between what might be expected from the available literature and our experience is related to the fact that, for patients with Henoch-Schönlein syndrome, the physical skin examination focuses mainly on the distinctive palpable rash, which does not blanch with pressure and symmetrically affects the buttocks, legs, and feet. Unsurprisingly, therefore, it was not difficult for us to find images in the literature depicting the characteristic rash of Henoch-Schönlein purpura, [10][11][12] which was associated with evident linear lesions, although they were not specifically described as such in the image legends.…”

“…Ten years ago, one of us (M.G.B.) observed, for the first time, horizontal lesions on the waistline of a boy with Henoch-Schönlein syndrome . Between January 1, 2010, and December 31, 2015, all children with Henoch-Schönlein syndrome who presented to this pediatric hospitalist underwent a structured skin examination in addition to the usual clinical and laboratory evaluation (complete blood cell count, C-reactive protein, and urinalysis).…”

“…This tendency, often termed Köbnerization , is common in psoriasis, lichen planus, vitiligo, and many bullous dermatoses . Very rarely, such lesions have also been documented in patients with Henoch-Schönlein syndrome, an immunoglobulin A–dominant leukocytoclastic small vessel vasculitis . This report presents our prospective experience with linear nonblanching skin lesions in children with this vasculitis syndrome.…”

“…1 Very rarely, such lesions have also been documented in patients with Henoch-Schönlein syndrome, an immunoglobulin A-dominant leukocytoclastic small vessel vasculitis. [2][3][4][5][6][7][8] This report presents our prospective experience with linear nonblanching skin lesions in children with this vasculitis syndrome.…”

“…observed, for the first time, horizontal lesions on the waistline of a boy with Henoch-Schönlein syndrome. 3 Between January 1, 2010, and Decem-ber 31, 2015, all children with Henoch-Schönlein syndrome who presented to this pediatric hospitalist underwent a structured skin examination in addition to the usual clinical and laboratory evaluation (complete blood cell count, C-reactive protein, and urinalysis). Significant emphasis was put on the presence of nonblanching, palpable lesions with a linear pattern and a length of at least 3 cm (subsequently referred to as linear lesions) on the legs, groin, waistline, wrists, or forearms.…”