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Clinical and genetic features of isolated and complex <jats:styled-content style=\"fixed-case\">PPK<\/jats:styled-content>s have been reviewed in part I of this 2\u2010part review. Here we focus on clinical and molecular classification of syndromic <jats:styled-content style=\"fixed-case\">PPK<\/jats:styled-content>s which are recognized by additional extracutaneous manifestations, in particular deafness, specific mucosal lesions, cardiomyopathy, inborn errors of metabolism, involvement of internal organs or disorders of sexual development. Other genetic diseases, which may show palmoplantar involvement, such as selected subtypes of hereditary epidermolysis bullosa, various hereditary ichthyoses and other keratinization disorders, several ectodermal dysplasias and some multisystem genetic disorders, are also briefly summarized. <jats:styled-content style=\"fixed-case\">PPK<\/jats:styled-content> diagnosis is based on inheritance pattern, age at onset, morphology, distribution and severity of hyperkeratosis, pattern of additional dermatological and systemic manifestations and laboratory findings. Molecular analysis is at present the gold standard to confirm the diagnosis in <jats:styled-content style=\"fixed-case\">PPK<\/jats:styled-content> forms due to mutations in known causative genes. No specific and curative therapy is currently available for <jats:styled-content style=\"fixed-case\">PPK<\/jats:styled-content>s which highly impair patients\u2019 quality of life. Topical treatments are symptomatic and offer only temporary relief. Among systemic treatments, retinoids improve disease symptoms in the majority of patients.<\/jats:p>","DOI":"10.1111\/jdv.14834","type":"journal-article","created":{"date-parts":[[2018,2,3]],"date-time":"2018-02-03T19:42:41Z","timestamp":1517686961000},"page":"899-925","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":47,"title":["Hereditary palmoplantar keratodermas. Part <scp>II<\/scp>: syndromic palmoplantar keratodermas \u2013 Diagnostic algorithm and principles of therapy"],"prefix":"10.1111","volume":"32","author":[{"given":"L.","family":"Guerra","sequence":"first","affiliation":[{"name":"Laboratory of Molecular and Cell Biology Istituto Dermopatico dell'Immacolata\u2010IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Castori","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics Casa Sollievo della Sofferenza\u2010IRCCS San Giovanni Rotondo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Didona","sequence":"additional","affiliation":[{"name":"Rare Skin Disease Center Istituto Dermopatico dell'Immacolata\u2010IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Castiglia","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular and Cell Biology Istituto Dermopatico 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