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These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first\u2010 and advanced\u2010line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this guideline provides clinicians with an overview of the diagnosis and treatment of scleromyxedema, scleredema (of Buschke) and nephrogenic systemic sclerosis (nephrogenic fibrosing dermopathy).<\/jats:p>","DOI":"10.1111\/jdv.14466","type":"journal-article","created":{"date-parts":[[2017,8,8]],"date-time":"2017-08-08T11:42:38Z","timestamp":1502192558000},"page":"1581-1594","source":"Crossref","is-referenced-by-count":95,"title":["European dermatology forum S1\u2010guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis"],"prefix":"10.1111","volume":"31","author":[{"given":"R.","family":"Knobler","sequence":"first","affiliation":[{"name":"Department of Dermatology Medical University of Vienna  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Moinzadeh","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology 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