汎発性鞏皮症—特に4症例と本症の皮膚と内臓変化の関係について

DIFFUSE SCLERODERMA:REPORT OF FOUR CASES AND RELATIONSHIP BETWEEN THE SKIN MANIFESTATIONS AND THE INTERNAL INVOLVEMENTS

I．緒言

　本症の記載は古く，皮膚の硬化という特異な症状のためか，紀元前から本症らしい記載があるという^1）。しかし本症が皮膚のみならず内臓諸器官にも変化を伴う系統的な疾患と明確にしたのは，有名なKlemperer等（1942）のいう所謂膠原病という概念の中に含められてからである。その後Goetz^2）は本症の臓器変化を詳細に報告した。しかし皮膚病変と内臓変化の関係について詳しく述べたものは少ない。

　我々も最近4例の本症を相次いで経験し，経過を観察中に皮膚変化の進行程度あるいは罹患範囲と種々内臓変化との間には，何等関係なく症状が進行することを経験した。

In four cases of diffuse scleroderma, the authors found that the systemic involvements progressed independently with the extent and degree of the skin manifestations.

Statistical analysis of the 213 reported cases of this disease in Japan from 1955 to 1964, especially in correlation between the degrees of involvements of the skin and the internal organs is performed.

The results are as follows;

(1) The ratio of incidence in male and female is 1 : 4.7.

(2) The order of incidence in the age of onset is the third, second, fourth and fifth decades.

(3) The cases with severe and extensive skin manifestations are not necessarily associated with advanced internal lesions.

(4) Even in the cases with mild cutaneous symptoms, definite internal involvement can be frequently proved.

(5) The results of the laboratory tests also indicate that there is no relationship between the degree of skin process and that of internal disturbances.

(6) There is no relation among the area of involved skin, subjective symptoms and labora-tory informations.

Although patients consult a dermatologist on account of skin manifestation as a chief complaint, these results indicate that investigation should be made about internal involvement.