Werner症候群の1例

WERNER'S SYNDROME

I．はじめに

　本症はWerner^1）（1904）により"Über Kat-arakta in Verbindung mit Sklerodermie"の下に報告された遺伝性疾患である。その主要症状は，若年性白内障，特徴ある短躯を示す思春期以後の発育障害，早期老化，皮膚の萎縮性変化であり，遣伝形式は劣性遺伝を示し，家族内発生，時に同一同胞内に多数の本症患者を発生する点が特徴的であり，欧米では現在までに百数十例の報告がみられている。Werner自身が眼科の医者であり，患者も多くは眼科にて発見される事が多い為に，本症候群の皮膚変化は充分に検討される事なく，Sclerodermieとの異同も不明のまま，一時混乱した時代もあつたが，Oppenheimer^2）　（1934），Thannhauser^3）（1945）の報告以来，漸く両者の異同もはつきりして来た。

　本邦においても石田^4）の報告例以来，同症と思われる報告例は40例以上に達するが，主に眼科領域からの報告例が多く，皮膚科領域での報告例は，坂本^5），小林^6），江原^7）以下僅か数例しか見られず，従つて，本症の皮膚変化に関する検討もまだ充分にはなされていない様である。

A case of this disease in a 33-year-old man hospitalized in the department of psychiatry by paranoia and illusion was reported.

By the family's remarks a patient of cataract and a member with small stature were present in this family.

The patient's growth stopped at the age of 14, canities developed after 20 years of age, ca-taract was found at the age of 31. He has been receiving hormone therapy for eunuchoidism.

He was 149cm high, weighing 36kg, and had rather thin extremities and underdeveloped muscles and subcutaneous tissue. Reticular brown pigmentation and telangiectasia on the face, neck and preauricular region, atrophic skin of the earlobe, atrophic thin skin with reticular pigmentation on the forearms and lower legs, clavus-like hyperkeratosis on the soles, under-development of the external genitalia, and mild diabetes mellitus were proved.

Histologic specimen from the forearm showed atrophy of the epidermis, hair follicles and other skin appendages, decreased thickness of the dermis, homogenization of the connective tissue of the subpapillary layer, and fragmentation and swelling of the elastic fibers in the upper and middle dermis.

Histology from the face skin showed a "senile elastosis"-like picture.

Although histologic pictures of this syndrome have been various according to investigators, the picture of the author's case was the same as that of Knoth's case.