汎発性鞏皮症の2剖検例

TWO AUTOPSY CASES OF DIFFUSE SCLERODERMA

I．緒言

　汎発性鞏皮症は単に皮膚の変化をきたすのみならず，全身諸器官を侵す系統的疾患であり，近時皮膚科のみならず，内科方面でも興味をもたれてきた。内臓諸器官のうちでも，特に心，肺，腎の変化は致命的であり，大多数の症例はこれが原因で死亡する。このうち特に肺における変化はかなり特異であり，最も注目されてきた。私共は最近，本症の発症から死亡まで1年という比較的急性の経過をとつた1例と同じく10年の長い経過をとつた1例を相ついで経験したが，いずれも呼吸器症状が著しく，剖検により肺，心の病変が著明であつた。この2例を報告し若干の考察を加えたい。

Case 1 : A 35-year-old man with a total course of about one year-onset in May '64 and death on May 11th, 1965.

Case 2 : A 54-year-old woman with a total course of ten years-onset in Spring of 1956 and death on Feb. 21st, 1966.

Both cases belonged to the peripheral sclerosing type. In case 1 sclerosis of face and peripheral parts of extremities were prominent but atrophy was not severe. In case 2 sclerosis of the skin developed over the entire body surface, and atrophy of the skin was marked. Abnormal shadows in chest roentgenogram were found in Dec. '64 in case 1 and in Feb. '62 in the second case. In both cases, chest roentgenogram showed maculocystic in the upper and middle field and reticular shadow in the lower lung field. They died on cardio-pulmonary dysfunction and bronchopneumonia.

ECG showed intraventicular stimulation conduction disturbance in both cases.

Autopsy findings of both cases were interstitial fibrosis and cystic enlargement of bronchus in the lung, edematous sclerosis and marked tylosis formation in the heart muscle, but slight changes in the kidney. The periods from the onset of the skin manifestation to the time of start of respiratory symptoms were 7 months in the first case and 6 years in the second case.It seemed that the respiratory disorder was main factor in determination of time of death in these cases.