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Hospitals in Japan often see cases of XP as severely photosensitive in children, and as advanced pigment disorders of the sun-exposed area with multiple skin cancers in adults (aged in their 20–40s), making XP an important disease to differentiate in everyday clinical practice.Ĭlinical practice guidelines for XP are mentioned in Japan Dermatological Association-approved guidelines on genetic diagnosis of skin diseases, but the items on XP are not extensive or comprehensive. XP patients thus need to be strictly shielded from sunlight throughout their lives, and they often experience idiopathic neurodegenerative complications that markedly reduce the quality of life (QOL) for both the patients and their families. Meanwhile, XP is one of the extremely serious photosensitive disorders in which patients easily develop multiple skin cancers if they are not completely protected from UV. 4 The three diseases, except for XP, have strong elements of phacomatosis. However, the clinical image varies depending on each disease, and the clinical practice guidelines have been developed separately. Neurocutaneous syndromes comprise four diseases: neurofibromatosis type 1, neurofibromatosis type 2, tuberous sclerosis and XP. For this reason, in March 2007, the Japanese Ministry of Health, Labor and Welfare added XP to the neurocutaneous syndromes that are subject to government research initiatives for overcoming intractable diseases. Therefore, this disease does not merely present with dermatological symptoms, such as photosensitivity, pigment change and skin cancers, but is “an intractable neurological and dermatological disease”. ![]() 3 In Japan, more than half of patients (30% worldwide) with XP show complications of idiopathic progressive, intractable neurological symptoms with poor prognoses. He discovered that XP cells unable to repair DNA damage caused by ultraviolet radiation (UV). Cleaver, reported for the first time that XP is a genetic disease in humans, in which genetic abnormalities occur in the DNA repair process. 1, 2 In 1968, the American radiobiologist, James E. It was first recorded at the end of the 19th century by a dermatologist, Moritz Kaposi, who described a severe photosensitivity disorder that was accompanied by pigment change. Xeroderma pigmentosum (XP) is a genetic photosensitive disorder in which patients show a high susceptibility to skin cancers on the sun-exposed body sites. It was thus decided that there was a strong need for clinical practice guidelines dedicated to XP. Hospitals in Japan often see cases of XP as severely photosensitive in children, and as advanced pigmentary disorders of the sun-exposed area with multiple skin cancers in adults (aged in their 20–40s), making XP an important disease to differentiate in everyday clinical practice. 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