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冷球蛋白血症的皮肤表现:72例患者的临床与组织病理学研究

Cutaneous manifestations of cryoglobulinemia: clinical and histopathologic study of seventy-two patients.

作者信息

Cohen S J, Pittelkow M R, Su W P

机构信息

Department of Dermatology, Mayo Clinic, Rochester, MN 55905.

出版信息

J Am Acad Dermatol. 1991 Jul;25(1 Pt 1):21-7. doi: 10.1016/0190-9622(91)70168-2.

Abstract

In the 72 cases of cryoglobulinemia reviewed, erythematous to purpuric macules or papules were present in 92%. Infarction, hemorrhagic crusts, and ulcers were present in 10% to 25% of the patients and were relatively more common in type I cryoglobulinemia than in the other types. Postinflammatory hyperpigmentation was noted in 40%. Lesions on the leg were common in all types of cryoglobulinemia; however, lesions on the head and mucosal surfaces suggested type I cryoglobulinemia. Histopathologic features were classified as vasculitis in 50%, inflammatory or noninflammatory purpura in 15%, noninflammatory hyaline thrombosis in 10%, and postinflammatory sequelae in 10%. Noninflammatory hyaline thrombosis was relatively more frequent in type I. Thus erythematous to purpuric lesions on the legs and leukocytoclastic vasculitis are the common cutaneous findings in cryoglobulinemia. Type I cryoglobulinemia is suggested by noninflammatory hyaline thrombosis, cutaneous infarction, hemorrhagic crusts, skin ulcerations, and lesions of the head and neck and of oral or nasal mucosa.

摘要

在回顾的72例冷球蛋白血症病例中,92%的患者出现了从红斑到紫癜性的斑疹或丘疹。10%至25%的患者出现梗死、出血性痂皮和溃疡,且在I型冷球蛋白血症中相对比其他类型更常见。40%的患者有炎症后色素沉着。腿部病变在所有类型的冷球蛋白血症中都很常见;然而,头部和黏膜表面的病变提示为I型冷球蛋白血症。组织病理学特征分类为血管炎的占50%,炎症性或非炎症性紫癜占15%,非炎症性透明血栓形成占10%,炎症后后遗症占10%。非炎症性透明血栓形成在I型中相对更常见。因此,腿部的红斑到紫癜性病变和白细胞破碎性血管炎是冷球蛋白血症常见的皮肤表现。非炎症性透明血栓形成、皮肤梗死、出血性痂皮、皮肤溃疡以及头颈部和口腔或鼻腔黏膜的病变提示为I型冷球蛋白血症。

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