Physiopathologische, klinische und therapeutische Aspekte des Morbus Darier
摘要
Darierʼs disease (dyskeratosis follicularis) is a rare genodermatosis with autosomal dominant inheritance. It typically occurs during puberty with brown-yellowish, scaly, crusty papules in the skin folds and seborrhoeic areas, accompanied by nail changes and involvement of the mucous membranes. The disease manifests itself in relapses, which can be triggered by UV radiation, heat, friction or infections. Affected patients have an increased risk of type 1 diabetes, heart failure and neuropsychiatric disorders. Antibiotics/virostatics, topical corticosteroids and/or retinoids are used for short-term treatment. Long-term treatment includes systemic therapy with retinoids and the avoidance of potential trigger factors. As there is no standardised long-term therapy, numerous local and systemic treatments as well as physical and surgical measures are described in literature.