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Rinsho Hifuka Volume 23, Issue 3 （March 1969）

臨床皮膚科 23巻3号（1969年3月発行）

Japanese English

原著

皮膚病変を伴える骨髄線維症の1例 MEDULLARY FIBROSIS WITH SKIN MANIFESTATIONS 菊池滋 ¹ , 宇野千春 ² Shigeru KIKUCHI ¹ , Chiharu UNO ² ¹弘前大学医学部皮膚科教室 ²弘前大学第1内科教室 ¹Department of Dermatology, Hirosaki University School of Medicine ²Department of the First Internal Medicine, Hirosaki University pp.249,295-301

発行日 1969年3月1日 Published Date 1969/3/1

DOI https://doi.org/10.11477/mf.1412200479

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Abstract 文献概要
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I．緒言

　骨髄線維症は骨髄の線維化，髄外造血巣，特異な血液像（leucoerythroblastic anemia）を3主徴とする疾患で，これまで報告された例では脾，肝，腎，リンパ節などの臓器に髄外造血巣が認められている。しかし，皮膚に髄外造血巣を生じる例は非常に稀なもので，特に骨髄細胞，赤芽球，巨核球の3系統を備えた症例は未だその報告に接しない。

　著者等は今回皮膚に髄外造血巣を伴つた骨髄線維症の1例を経験したので報告する。

A 57-year-old farmer had tinnitus in May, 1967 and swelling and oppressive sensation in the abdominal area in the middle of November.

He was addmitted to the Department of Internal Medicine, when xamination of the peripheral blood showed anemia and leukocytosis with nucleated erythrocytes and immature myelocytes. Biopsy of the bone marrow proved immature myelocytes, erythroblasts, mega-karyocytes and proliferation of reticulum fibers with partial fibrosis. Foci of extramedullary hematopoiesis were found in the spleen and lymph nodes.

The patient was diagnosed as having primary medullary fibrosis and treated with 6MP, prednisolone, antibiotics and blood transfusion. There were found several firm nodules without subjective sensations in the abdominal and lumbar regions in January 1968, but the patient was discharged from the hospital in March 19.

The patient visited the Department of Dermatology in April 16, 1968, when he had several, from rice grain-to pea-sized, small nodules of a normal or a dark brown color on the epigastrium and right lumbar region. After biopsy of the nodule in the lumbar region, lesions increased in number and size and the patient had general malaise, nausea, vomiting, swelling of the face and distension of the abdomen. He was addmitted again to the Department of Internal Medicine on May 14. On May 29, there were three, dark red, to walnut-sized nodules in the lumbar region, and several nodules to a pea in size, on the back, in the lumbar and abdominal regions.

A histologic specimen from the nodule in the left lumbar region showed a focal or a disseminated infiltrate of immature myclocytes, erythrocytes and megakaryocytes around the vessel, sweat duct and hair follicle and among the collagen bundles throughout the dermis.