Many systemic diseases go along with dermal involvement. Only a few of systemic diseases show characteristic skin derangements directly leading to the diagnosis of the underlying disease. Ten of these entities are described within this article. Some of these are autoimmune diseases, such as systemic lupus erythematodes, dermatomyositis or the anti-synthetase-syndrome. Others are genetic diseases with benign or malignant neoplasias (Peutz-Jeghers-syndrome, neurofibromatosis, tuberous sclerosis, Birt-Hogg-Dubé-syndrome, Cowden-syndrome). Other genetically based diseases with typical skin manifestations are characterised by vascular malformations (Pseudoxanthoma elasticum and Osler-Weber-Rendu syndrome) or metabolic and structural organ defects (Morbus Fabry, cystic fibrosis). Being familiar with the typical skin-appearance of these diseases will allow physicians and general practitioners to make a timely diagnosis.
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