[New therapeutic strategies for the different subtypes of urticaria]. / Neue Therapieoptionen der Urtikaria und ihrer Subtypen.

Urticaria is one of the most common skin diseases. It is divided into several categories including acute and chronic urticaria as well as physical urticaria and special subtypes such as cholinergic and autoimmune urticaria. The causes of urticaria are multiple. In more than 80%, urticaria is triggered by an inflammatory focus, a subclinical infection or autoimmune actions. In addition, non-specific pharmacological or toxin-mediated release of inflammatory mediators from basophils or mast cells can also trigger urticaria. These new aspects in the pathophysiology of urticaria suggest new and promising therapeutic strategies. The first line treatment of urticaria still consists of non-sedating H1-antihistamines. Since urticaria has a profound impact on quality of life, effective treatment is very important. We consider new therapeutic options based on our long-term experience in treating patients with urticaria.

[Chronic urticaria in childhood]. / Chronische Urtikaria im Kindesalter.

Chronic urticaria in children occurs more often than it might be supposed, but its management is not part of the usual daily routine. While acute urticaria in children can be treated symptomatically without further investigations, chronic urticaria requires a search for trigger factors. Autoimmune urticaria can occasionally be documented in children. The treatment of chronic urticaria depends on the suspected trigger, always keeping in mind that medications may need to be adjusted to meet pediatric requirements.

[Hexadactyly of hand and feet in a patient with basal cell nevus syndrome]. / Hexadaktylie an Hand und Fuss bei einem Patienten mit Basalzellnävussyndrom.

A 35-year-old male patient with hexadactyly on the right hand and both feet presented with multiple basal cell carcinomas of the skin. The results of further investigations led to the diagnosis of basal cell nevus syndrome (BCNS). The basal cell nevus syndrome (Gorlin-Goltz syndrome) characterized by multiple basal cell carcinomas, odontogenic cysts, skeletal abnormalities and associated neoplasms belongs to the group of genodermatoses. Reviewing the current literature we discuss aspects of the pathogenesis of the basal cell nevus syndrome as well as diagnosis criteria and treatment options.

[Hereditary C1 esterase inhibitor deficiency type I. Divergence of clinical symptoms and laboratory chemical findings]. / Hereditärer C1-Esterase-Inhibitor-Mangel Typ I. Divergenz klinischer Symptomatik und laborchemischer Befunde.

A 35- and a 29-year-old woman presented with longstanding recurrent angioedema refractory to therapy with steroids and antihistaminic drugs. Laboratory data revealed in both cases a functional and immunohistochemical deficiency of C1-esterase-inhibitor protein (C1-INH). Further investigations of their families showed some individuals with the same pathological findings without any clinical symptoms. Thus we identified striking differences in degree of severity of clinical features in hereditary C1-INH-deficiency type I. These case-reports emphasize the importance of knowing further diagnostic steps in angioedema refractory to therapy with steroids and antihistamines drugs. Furthermore we give a picture of the different clinical courses of C1-INH-deficiency type I and underline that there is no correlation between clinical symptoms and laboratory findings.