Ichthyoses are a group of genetic disorders with defective cornification, clinically characterized by scaling of the skin. Additionally, distinctive cutaneous inflammation can often be observed. For most of the patients these diseases lead to a significant restriction in quality of life. The diagnostic criteria include clinical and histological findings, often confirmed by specialized tests. Because many of the ichthyoses are extremely rare, their accurate diagnosis is often carried out in specialized centers. We summarize isolated vulgar and congenital ichthyoses both with and without associated symptoms and focus on the common genetic changes and their clinical phenotype. Specific therapies are still not available for most of these genetic disorders. The use of different topical agents (e. g. urea, retinoids and salicylic acid) and baths followed by mechanical keratolysis (sometimes in combination with systemic retinoids) reduce symptoms. Patients with uncommon congenital ichthyoses often benefit from interdisciplinary management which involves specialized dermatological centers.
In this first part of the paper the vulgar ichthyoses as well as the diagnostic and therapeutic options are discussed. The second part focuses on the congenital ichthyoses and their differential diagnosis.