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恶性萎缩性丘疹病(德格罗斯病)。两例临床与组织学研究报告。

Malignant atrophic papulosis (Degros disease). A report of two cases with clinical and histological studies.

作者信息

Muller S A, Landry M

出版信息

Arch Dermatol. 1976 Mar;112(3):357-63. doi: 10.1001/archderm.112.3.357.

DOI:10.1001/archderm.112.3.357
PMID:1259448
Abstract

Two women had malignant atrophic papulosis that affected the skin only. Immunologic studies showed normal amounts of serum immunoglobulins and complement and normal delayed hypersensitivity skin test results. Fibrinolysis autografts showed complete loss of fibrinolysis within the lesions but normal fibrinolysis in uninvolved skin. Histopathologic studies showed moderate inflammatory infiltration consisting of lymphocytes in addition to the typical cone-shaped regions of necrobiosis. Large amounts of mucin also were seen. Endovasculitis, thrombosis, and perivascular lymphocytic inflammation were noted in the subcutaneous arterioles of the lesions in only one of the patients. Enzyme histochemical studies showed oxidative enzymes, although in reduced amounts, in the epidermal and dermal cells of the affected skin. Electron microscopic studies of lesions showed paromyxovirus-like inclusions in endothelial cells and fibroblasts. The possibility of an unusual viral causation should be examined further.

摘要

两名女性患有仅累及皮肤的恶性萎缩性丘疹病。免疫学研究显示血清免疫球蛋白和补体含量正常,迟发型超敏皮肤试验结果正常。纤维蛋白溶解自体移植显示病变部位纤维蛋白溶解完全丧失,但未受累皮肤的纤维蛋白溶解正常。组织病理学研究显示,除了典型的坏死性锥形区域外,还有由淋巴细胞组成的中度炎症浸润。还可见大量黏蛋白。仅在其中一名患者的病变皮下小动脉中观察到血管内皮炎、血栓形成和血管周围淋巴细胞炎症。酶组织化学研究显示,受累皮肤的表皮和真皮细胞中存在氧化酶,尽管含量减少。病变的电子显微镜研究显示内皮细胞和成纤维细胞中有类副黏液病毒样包涵体。应进一步研究不寻常病毒病因的可能性。

相似文献

1
Malignant atrophic papulosis (Degros disease). A report of two cases with clinical and histological studies.恶性萎缩性丘疹病(德格罗斯病)。两例临床与组织学研究报告。
Arch Dermatol. 1976 Mar;112(3):357-63. doi: 10.1001/archderm.112.3.357.
2
The role of dermal blood vessels in the pathogenesis of malignant atrophic papulosis (Degos' disease). A study of two cases using enzyme histochemical, fibrinolytic, electron-microscopical and immunological techniques.真皮血管在恶性萎缩性丘疹病(德戈斯病)发病机制中的作用。应用酶组织化学、纤维蛋白溶解、电子显微镜及免疫学技术对两例病例的研究
Br J Dermatol. 1973 Mar;88(3):213-9. doi: 10.1111/j.1365-2133.1973.tb07537.x.
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Malignant atrophic papulosis--Degos' syndrome.恶性萎缩性丘疹病——德戈斯综合征
Acta Chir Scand. 1979;145(4):279-83.
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[Degos malignant atrophic papulosis].[德戈斯恶性萎缩性丘疹病]
An Esp Pediatr. 1980 May;13(5):437-40.
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[Degos' malignant atrophic papulosis. Ultrastructural study of a new case].
Ann Anat Pathol (Paris). 1980;25(2):111-24.
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Clinical and histologic findings in Degos' syndrome (malignant atrophic papulosis).
Cutis. 1985 Feb;35(2):131-8.
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Atrophie blanche lesions closely resembling malignant atrophic papulosis (Degos' disease) in systemic lupus erythematosus.系统性红斑狼疮中与恶性萎缩性丘疹病(德戈斯病)极为相似的白色萎缩性病变。
Br J Dermatol. 1976 Dec;95(6):649-52. doi: 10.1111/j.1365-2133.1976.tb07040.x.
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Lymphocytes and necrosis of the cutaneous microvasculature in malignant atrophic papulosis: a refined light microscope study.恶性萎缩性丘疹病中淋巴细胞与皮肤微血管坏死:一项改良的光学显微镜研究
J Am Acad Dermatol. 1982 Nov;7(5):620-30. doi: 10.1016/s0190-9622(82)70142-2.
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Exchange autografts in malignant atrophic papulosis (Degos' disease).恶性萎缩性丘疹病(德戈斯病)中的自体移植置换术。
Mayo Clin Proc. 1974 Nov;49(11):884-8.
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Malignant atrophic papulosis (Kohlmeier-Degos disease) in childhood.儿童期恶性萎缩性丘疹病(科尔迈耶-德戈斯病)。
Neurology. 1976 Apr;26(4):317-21. doi: 10.1212/wnl.26.4.317.

引用本文的文献

1
Lethal systemic degos disease with prominent cardio-pulmonary involvement.伴有显著心肺受累的致死性系统性德戈斯病。
Indian J Dermatol. 2011 Sep-Oct;56(5):564-7. doi: 10.4103/0019-5154.87157.
2
An atypical paediatric case of malignant atrophic papulosis (Köhlmeier-Degos disease).一例非典型小儿恶性萎缩性丘疹病(科尔迈尔-德戈斯病)。
Eur J Pediatr. 1990 Apr;149(7):457-8. doi: 10.1007/BF01959394.