興味あるRecklinghausen氏病の4例

FOUR CASES OF RECKLINGHAUSEN'S DISEASE

I．はじめに

　Recklinghausen氏病（以下R病と略す）は，皮膚腫瘍及び色素斑の他に全身諸臓器に多彩な症状を示すため，そこに現われる症状は個々の症例によつてまちまちである。今回，臨床的・組織学的に興味深い像を示す4例を経験したので報告し，いささかの考察を加えてみたい。

Four cases of this disease were reported.

Case 1: An 18-year-old female with typical pigmented spots and disseminated skin tumors had a large tumor which covered the entire right, upper extremity. Its roentgenogram revealed abnormal development of the brachial, radial and ulnar bones, scoliosis, erosive defect and cyst formation of bone. Its histologic picture was so-called neurofibroma.

Case 2: A 17-year-old female had pigmented macules, skin tumors, subcutaneous nodules arranged in rosary and scoliosis. The subcutaneous nodules were composed of myelinated nerve fibers, most of which showed slight degeneration.

Case 3 : A 25-year-old, elder brother of case 2, had pigmented spots and skin tumors.

Case 4 : A 6-year-old girl showed pigmented spots which were disseminated over the entire body surface, and large pigmented hairy spots in the gluteal and right femoral regions under which soft tumors were found. She also had spina bifida and erosive defect of the pelvic bones. Histologic specimen from the skin tumor was composed partly of a so-called neurofibromatous structure and partly of severely degenerated neuromatous structure.

Pathologic changes of nerve fibers such as those seen in case 2 might be an early picture of this disease, which might gradually degenerate into such a situation as seen in case 4.

Relationships between proliferation of nerve fibers and that of schwannian cells were not de-monstrated.