We describe a case of photosensitivity due to pyridoxine hydrochloride (vitamin B6) in a heterozygote of hypophosphatasia. Photopatch tests using pyridoxine hydrochloride and pyridoxal 5′-phosphate, compounds referred to as vitamin B6, with ultraviolet light A irradiation were positive. Laboratory examination showed low serum alkaline phosphatase. Tissue-nonspecific alkaline phosphatase exon amplification from DNA of the patient’s lymphocytes detected deletion 1154–1156 hypophosphatasia mutation, indicating that this patient was diagnosed to be a heterozygote of hypophosphatasia. The seric pyridoxal 5′-phosphate level of this patient with hypophosphatasia was higher than in normals. Furthermore, after oral administration of vitamin B6 this level increased greatly and long-lastingly, and this might be related to the low level of alkaline phosphatase in this patient. Photosensitivity in this patient may have been caused by abnormal metabolism of vitamin B6 under the hypophosphatic condition.

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