[1]王文菊,楚松林,李军友.原发性骨膜增生厚皮症伴重度贫血1例[J].中国皮肤性病学杂志,2021,(02):187-189.
　WANG Wenju,CHU Songlin,LI Junyou.A Case of Primary Hypertrophic Osteoarthropathy with Severe Anemia[J].The Chinese Journal of Dermatovenereology,2021,(02):187-189.
点击复制

原发性骨膜增生厚皮症伴重度贫血1例()
分享到：

《中国皮肤性病学杂志》[ISSN:1001-7089/CN:61-1197/R]

卷:
期数:
2021年02期
页码:
187-189
栏目:
病例报告
出版日期:
2021-02-01

文章信息/Info

Title:
A Case of Primary Hypertrophic Osteoarthropathy with Severe Anemia
文章编号:
1001-7089(2021)02-0187-03
作者:
王文菊1; 楚松林2; 李军友1
1.兰州大学第二医院皮肤科,甘肃 兰州 730030; 2.兰州大学第二医院血液科,甘肃 兰州 730030
Author(s):
WANG Wenju1; CHU Songlin2; LI Junyou1
(1.Department of Dermatovenereology,the Second Hospital of Lanzhou University, Lanzhou 730030,China; 2.Department of Hematology,the Second Hospital of Lanzhou University,Lanzhou 730030,China)
关键词:
原发性骨膜增生厚皮症;  重度贫血
Keywords:
Primary hypertrophic osteoarthropathy;  Severe anemia
分类号:
R 758.5; R 556
文献标志码:
B
摘要:
患者男,39岁,面部皮肤肥厚、四肢肢端肥大20年,间断头晕乏力1年。查体:重度贫血貌,睑结膜、口唇及甲床苍白; 头面部皮肤肥厚、皱褶、呈回状颅皮; 双下肢皮肤肥厚,手指及足趾呈杵状; 皮损颜色正常,触之略韧; 踝关节肿胀无压痛、活动自如。X线检查提示右腓骨中段髓腔密度增多,腓骨中段及胫骨下段后缘骨皮质增厚。血常规重度正常细胞低色素性贫血,骨髓活检提示骨髓造血衰竭,骨髓纤维化不明显。诊断:原发性骨膜增生厚皮症伴重度贫血。
Abstract:
A 39-year-old male presented thicked facial skin and extremities hypertrophy for 20 years,intermittent dizziness and fatigue for 1 year.Physical examination showed severe anemia appearance,pallid palpebral conjunctiva,lips and nail beds.The skin of his face,scalp and lower limbs became thickened,with prominent gyrus-like skin folds found in his scalp and forehead,clubbing fingers and toes were obvious.The lesions with normal skin colour and slightly tough.Ankle joint swelling without tenderness,free movement.X-ray images showed increased density of the medullary cavity in the middle part of the right fibula,and thickened cortical bone in the middle part of the right fibula and the posterior margin of the lower tibia.Blood routine examination showed severe anemia of normal cells,bone marrow biopsy showed hematopoietic failure of bone marrow,and myelofibrosis was not obvious.The diagnosis of primary hypertrophic osteoarthropathy with severe anemia was definite.

参考文献/References:

[1] 杜然,范亮亮,黄皓,等.原发性厚皮性骨膜病的遗传学研究进展[J].中华医学遗传学杂志,2016,33(1):105-107.
[2] Salah BI,Husari KI,Hassouneh A,et al.Complete primary pachydermoperiostosis:A case report from Jordan and review of literature[J].Clin Case Rep,2019,7(2):346-352.
[3] Matucci-Cerinic M,Lotti T,Jajic I,et al.The clinical spectrum of pachydermoperiostosis(primary hypertrophic osteoarthropathy)[J].Medicine(Baltimore),1991,70(3):208-214.
[4] Harbison JB,Nice CM.Familial pachydermoperiostosis presenting as an acromegaly-like syndrome[J].Am J Roentgenol Radium Ther Nucl Med,1971,112(3):532-536.
[5] 赵辨.中国临床皮肤病学[M].南京:江苏科学技术出版社,2010:1496-1497.
[6] 李莹洁,王东霞,叶珊珊,等.厚皮性骨膜病合并低钾血症1例并文献复习[J].中国皮肤性病学杂志,2019,33(3):320-323.
[7] Li S,Li Q,Wang Q,et al.Primary hypertrophic osteoarthropathy with myelofibrosis and anemia:a case report and review of literature[J].Int J Clin Exp Med,2015,8(1):1467-1471.
[8] Saigal R,Kansal A,Mittal M,et al.Pachydermoperiostosis with myelofibrosis and empty sella[J].J Assoc Physicians India,2010,58:253-255.
[9] Bachmeyer C,Blum L,Cadranel JF,et al.Myelofibrosis in a patient with pachydermoperiostosis[J].Clin Exp Dermatol,2005,30(6):646-648.
[10] Venencie PY,Boffa GA,Delmas PD,et al.Pachydermoperiostosis with gastric hypertrophy,anemia,and increased serum bone Gla-protein levels[J].Arch Dermatol,1988,124(12):1831-1834.
[11] de Risi-Pugliese T,Danière F,Legrand L,et al.Extrame-dullary hematopoiesis with spinal cord compression in pachydermoperiostosis[J].Joint Bone Spine,2017,84(4):509-510.
[12] Diggle CP,Parry DA,Logan CV,et al.Prostaglandin transporter mutations cause pachydermoperiostosis with myelo-fibrosis[J].Hum Mutat,2012,33(8):1175-1181.
[13] Ninomiya S,Hara T,Tsurumi H,et al.Myelofibrosis successfully treated with prednisolone in a patient with pachydermoperiostosis[J].Intern Med,2011,50(19):2207-2211.

更新日期/Last Update: 2021-02-01