Yiannias J A, el-Azhary R A, Gibson L E
Department of Dermatology, Mayo Clinic, Rochester, MN 55905.
J Am Acad Dermatol. 1992 Jan;26(1):38-44. doi: 10.1016/0190-9622(92)70003-x.
Erythema elevatum diutinum is a rare condition representing a chronic leukocytoclastic vasculitis.
Clinical and laboratory features of the disease were reviewed to better understand the disease.
The medical records and histopathologic slides of 13 patients with erythema elevatum diutinum were studied.
The lesions were violaceous, deep red, or brown and typically were papules or plaques. Lesions were most often located on the extensor surfaces of the extremities. Associated medical problems included hematologic abnormalities in six patients: IgA clonal gammopathies (four), multiple myeloma (one), and myelodysplasia (one). Erythema elevatum diutinum preceded the myeloproliferative disorders by an average of 7.8 years. All patients showed vasculitis. Leukocytoclasia was present in 27 of 35 specimens. The predominant cell type in the inflammatory infiltrate was polymorphonuclear leukocytes or a mixture of polymorphonuclear leukocytes and lymphocytes.
The most significant finding of this study is the association of erythema elevatum diutinum with hematologic disease, most frequently an IgA monoclonal gammopathy.
持久性隆起性红斑是一种罕见的慢性白细胞破碎性血管炎。
回顾该疾病的临床和实验室特征以更好地了解该疾病。
研究了13例持久性隆起性红斑患者的病历和组织病理学切片。
皮损为紫红色、深红色或棕色，典型表现为丘疹或斑块。皮损最常位于四肢伸侧。相关的内科问题包括6例血液学异常：IgA克隆性丙种球蛋白病（4例）、多发性骨髓瘤（1例）和骨髓发育异常（1例）。持久性隆起性红斑先于骨髓增殖性疾病出现，平均提前7.8年。所有患者均表现为血管炎。35份标本中有27份出现白细胞破碎现象。炎症浸润中的主要细胞类型为多形核白细胞或多形核白细胞与淋巴细胞的混合。
本研究最显著的发现是持久性隆起性红斑与血液系统疾病相关，最常见的是IgA单克隆丙种球蛋白病。
