Mikulicz病とその周辺

MIKULICZ DISEASE AND MIKULICZ SYNDROME

　I．はじめに

　1888年Mikulicz^1）が両側涙腺，耳下腺，顎下腺の無痛性の対称性の腫脹を伴う1症例を独立疾患として報告して以来，Mikulicz病の名のもとに多くの症例が報告されてきた。しかしMikuliczの報告した症例は経過観察が短かく，他因にて死亡しており，臨床検査，特に今日の免疫血清学的検索が行なわれ得なかつたためにその本態は不明であり，Mikulicz病またはMikulicz症候群の本態について種々の議論を生む事となつた。

　1927年に到りSchaffer^2）らは基礎疾患を明らかにできるものをMikulicz症候群，基礎疾患を明らかにできないものをMikulicz病（Mikulicz disease proper）とした。さらに1953年Morgan^3）4）らは従来基礎疾患を明らかにできずMikulicz病とされてきたものはSjögren症候群の1つの型であるとした。これが今日の一般的見解でもある。組織学的検索をもとにMikulicz病とSjögren症候群との関係を示唆したMorganらの功績は大きいが，免疫血清学的検査法の発達によりSjögren症候群^{5）-12）16）}の診断が容易になつた今日，従来のMikulicz病とされてきた症例の中にも免疫異常は伴わず，なお基礎疾患を明らかにできない症例がある。さらに免疫異常を伴う症例においてもSjögren症候群とは臨床像においていくつかの相違をみる事が多い。

　The etiology of Mikulicz disease has long been a topic of discussion since the former reported of his original case.

　Many cases of so-called Mikulicz disease may develop, if clinical course is followed for a long period, immunological abnormalities and these may be thought to be a variant of Sjögren syndrome as described by Morgan. But we still encounter cases with chronic bilateral swelling of the salivary and lacrimal glands and with typical histological findings as described by Mikulicz and Morgan, in which underlying diseases, such as Sjögren syndrome, malignant lymphoma hematological disorders, tuberculosis, syphilis, gaut, drug intoxocation, sarcoidosis and viral infections as mumps, adenovirus and cochaxie virus, can be ruled out. For proper understanding of the etiology of these obscure cases, careful follow-ups with thorough laboratory examinations including viral investigations are mandatory.

　The authors report 2 cases of Mikulicz disease.

　A 15 year-old girl with chronic bilateral swelling of parotid, submandibular and lacrymal glands developed an apparent immunological abnormality 10 months after the onset of symptoms. This case is presently under control by administration of irradiation and steroid therapy.

　The second case is a man, aged 49, with chronic bilateral swelling of the submandibular and lacrymal glands, without apparent underlying disorders. The swelling of the lacrymal glands subsided spontaneously 10 months after surgical extirpation of the bilateral mandibular glands without further recurrence of symptoms. During the period of 3 years observation, this case has failed to show any apparent immunoserological or hematological abnormalities.