pseudoxanthoma elasticum-like papillary dermal elastolysis

・pseudoxanthoma elasticum-like papillary dermal elastolysis（PXE-like PDE） は，中高年の女性の頸部を中心に，自覚症状のない，半米粒大の軟らかい淡黄色の丘疹が敷石状に集簇する疾患である．

・臨床所見のみでは，弾性線維性仮性黄色腫（pseudoxanthoma elasticum：PXE）との鑑別は困難である．

・今回われわれは，典型的なPXE-like PDEの1例を経験したため，報告するとともにPXEとの鑑別について考察を加えた．

（「症例のポイント」より）

　

A case of pseudoxanthoma elasticum-like papillary dermal elastolysis

　

Nakada, Kaya1）Watanabe, Kousuke1）Takahashi, Misaki1）Niiyama, Shirou1）Ooharaseki, Toshiaki2） Fukuda, Hidetsugu1） 1）Department of Dermatology, Tokyo Kyosai Hospital 2）Department of Dermatology, Toho University Ohashi Medical Center 3）Department of Pathology, Toho University Ohashi Medical Center

　

An 82-year-old woman presented in our department because small nodules had developed on her neck and axillae 6 years previously. Physical examination revealed non-follicular, yellowish, soft, and multiple aggregated small nodules on the neck and axillae. On histopathological examination, no significant change in the epidermis or obvious change in the collagen fibers was observed, but dilated capillaries and melanophages were scattered in the upper layer of the dermis. Elastica-van Gieson staining showed reduction of the elastic fibers in the papillary dermis. Kossa staining showed no calcium deposition on the elastic fibers. According to the findings, the patient was diagnosed with pseudoxanthoma elasticum-like papillary dermal elastolysis.