Shimizu N, Ito M, Ito K, Nakamura A, Sato Y, Maruyama T
Department of Dermatology, University School of Medicine, Niigata, Japan.
Arch Dermatol. 1990 Oct;126(10):1342-6. doi: 10.1001/archderm.126.10.1342.
The plantar hyperkeratotic skin lesion in a case of Richner-Hanhart's syndrome was investigated using ultrastructural examination. Light microscopic examination showed remarkable hyperkeratosis and some aberrant keratinocytes with multiple nuclei. On ultrastructural examination, some abnormal structures were seen in the affected keratinocytes: aggregations of tonofilaments and intracytoplasmic inclusions. The inclusions were needle shaped and were considered to be "crystal ghosts," presumably of tyrosine. The formation of tyrosine crystalline inclusions seems to be an important factor in the pathogenesis of the cutaneous lesions in Richner-Hanhart's syndrome.
运用超微结构检查对一例Richner-Hanhart综合征患者的足底角化过度性皮肤损害进行了研究。光镜检查显示显著的角化过度以及一些具有多个细胞核的异常角质形成细胞。超微结构检查发现，受累角质形成细胞中有一些异常结构：张力丝聚集和胞浆内包涵体。这些包涵体呈针状，被认为是“晶体残影”，推测为酪氨酸。酪氨酸结晶包涵体的形成似乎是Richner-Hanhart综合征皮肤损害发病机制中的一个重要因素。
