Bourneville-Pringle氏病ノ一例

• 飯田 四郎

  京都府立醫科大學皮膚科泌尿器科教室

書誌事項

タイトル別名

• A case of Bourneville-Pringle's Disease

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History. -H. K., a girl aged 14, whose family history was completely negative, was admitted to our clinic became of eruptions an her face, neck and body. The eruptions had been present for the past 12 years. According to her mother's statement, a few red papules appeared in the right zygomatic region of her child when she was two years old. They increased and spread gradually over her face. Examination of the skin. -There were numerous brownish red fairly well defined papules, the size of a miliary to a grain of rice, with minute teleangiectases symmetrically an her forehead, nose, chin, in the nasolabial folds and cheeks. There was a tendency towards confluence in the nasolabial folds and chin. The consistency was generally elastic and firm. On her neck, thorax, abdomen and back were found sporadical papules and nodules in the same quality mentioned above with rather normal skin color. Slightly raised normal skin colored or slightly brown plaques, just like a fibroma molluseum in appearance, some definitely pedunculated and others not so peduneulated, were detected an the neck, thorax, abdomen and genitals though small in number. Another firm socalled shark leather-like nodular plaque, approximately one by two centimetre, were distributed over the upper part of the back and loin. Physical examination. -The patient was moderate in stature, well nourished, irrexpressive and slow in action. Her heart and Jungs were normal. No abnormalities were found in the abdomen by palpation. Wassermann's reaction of the blood was negative. Both Pirquet's cutaneous reaction and Mantoux's percuta-neous reaction were negative. The erythrocytes numbered 4,000,000 and the leucocytes numbered 7,400 per cubic millimetre and the differential ratio of the leucocytes was almost normal. The hemoglobin content was 70 per cent (Sahli). The average worth of the descending velocity of the rod blood corpuseles was 22 millimetre (Westergren). The blond sugar content was 102 milligram decilitre (Hagedorn). The urine was microscopieally normal. The funetion of panereas was also normal. There was no evidence of the disturbance of the function of the kidney. The chromocystopsia, pneumoren, phenolsulfonphthalein test and water test gave negative results. The psychodynamic examination proved that the patient had subnormal intelligence. There were no epileptic seizures after her admission to the clinic. The cerebrospinal fluid was normal. In the left putamen region of tue pneumoencephalogram a sign of a cerebral tumor was found. On the ophthalmologic examination a tumor slightly larger than a papilla composed probably of the connective tissue or the cells of neuroglia was discovered along vasa tempolalis superior of retina of the both eyes. Roentgenologic examination of the bone gave negative results. Pharmacodynamic examination of the visceral nerve System resulted negatively. On examination of the function of the liver the following results were obtained: Takata's reaction was negative. Diazo-reaction was also negative. The metabolism of sugar examined by the charge test with levulose after Strauss and the charge test with lactose after Bauer resulted both slightly positive. The metabolism of albumin examined by amin acid test and the stability test with strange albumin both gave positive results. On the examination of the excretive function of the pigment it was proved that the methylen-blue test after Roch was positive, while that after Rosenthal and Falkenhausen, the indigo-carmin test after Lepehne and the asorbin test after Kuraya were all negative. Both the urobilin test and the urobilinogen fest of the urine gave positive results while the bili-rubin test was negative. I have never seen in former reports such findings which prove that a disturbance of the function of the liver could occur in this disease, though it is not unimaginable.Histopathological examination. -There were little to choose among the histopathological findings of the small tumors. They will be summarized here accordingly. Epidermis: There was no evident charge in the epithelial layers of epidermis. Corium: There was a noticeable hyperplasia of the connective tissue fibres in the whole layers of corium. The sebaceous glands and the sweat glands behaved almost normally, though some of them were hyperplastic and some were hypoplastic. The elastic fibres showed segmentation, diminution and eben disappearance in the highly hyperplastic part of the connective tissue, There were dilated and even newly formed blood vessels with perivaseular round Gell infiltration in the focus. As a peculiar finding in tumors from the nasolabial folds I can enumerate cysts of various sizes, localized sporadically in corium, filled with cholesterin-like substance and surrounded with stratified pavement epithelium. Some pair follicles in this specimen were atrophic, some were cornificated and some were dilated and filled with light reddish substance in the double staining with hematoxylin-eosin. The hair follicles were surrounded notably with round cells. Judging from the findings which indicate various stages of the hair follicles changing into cysts due to pressure, I was able to conclude that the hyperplasia of the sebaceous glands in this case occurred with that of the connective trssue or later time.

収録刊行物

• 京都府立医科大学雑誌

  京都府立医科大学雑誌 38 (1), 653-661, 1943

  京都府立医科大学

詳細情報

• CRID: 1571980077467755008
• NII論文ID: 110007120435
• NII書誌ID: AN00061975
• ISSN: 00236012
• 本文言語コード: ja
• データソース種別:
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