Pigmented dry skin disease is a rare autosomal recessive disorder. It is characterized by a deficient or reduced function of the “DNA excision repair system”, which does not effectively remove UV-induced pyrimidine dimers. Clinical manifestations include dermatitis at the site of exposure, abnormal pigmentation changes, premature aging of the skin and secondary skin tumors. Patients often have ocular and neurological symptoms. The disease is seen in all ethnic groups, with the highest prevalence in the Japanese and Middle Eastern populations. The incidence in China is 1 in 250,000. The disease is inherited in an autosomal recessive manner. Most parents are related, and the incidence is equal in men and women. Clinical features: 1. Skin symptoms: freckles and dry skin on exposed areas at an early age, deepening after sun exposure, accompanied by atrophic rash, capillary dilatation and warty keratosis, lesions easily mutated into basal cell carcinoma, squamous cell carcinoma and malignant pigmented tumors, etc. Rarely, angiosarcoma and fibrosarcoma are seen. Patients often die before the age of 10 or so, and 1/3 patients die before the age of 20 due to tumor metastasis or infection. 2. Ocular symptoms: photophobia and tearing are the earliest and most common symptoms, with varying degrees of conjunctivitis, keratitis, ocular adhesions, lid entropion or ectropion, pterygium, etc. 3. Neurological symptoms: It can involve about 20% of patients, mostly seen in type A and D. The symptoms are mental retardation, cerebellar ataxia, weakened or absent reflexes, etc. 4, other: the risk of visceral malignant tumors is 10-20 times higher than the normal population, including leukemia, lung and central nervous system tumors. Prevention: Sunlight should be avoided. External shading agent should be applied to the exposed area. Good-onset tumors, diagnosed early and removed. Ophthalmic treatment should be performed for eye lesions. Prohibition of consanguineous marriage is an effective measure to reduce the occurrence of pigmented dry skin disease.