MIXED CONNECTIVE-TISSUE DISEASE SYNDROME - CUTANEOUS MANIFESTATIONS OF PATIENTS WITH EPIDERMAL NUCLEAR STAINING AND HIGH TITER SERUM ANTIBODY TO RIBONUCLEASE-SENSITIVE EXTRACTABLE NUCLEAR ANTIGEN
MIXED CONNECTIVE-TISSUE DISEASE SYNDROME - CUTANEOUS MANIFESTATIONS OF PATIENTS WITH EPIDERMAL NUCLEAR STAINING AND HIGH TITER SERUM ANTIBODY TO RIBONUCLEASE-SENSITIVE EXTRACTABLE NUCLEAR ANTIGEN
复制标题
DOI：
10.1001/archderm.113.5.583
复制
发表时间：
1977-01-01
影响因子：
--
通讯作者：
PRYSTOWSKY, SD
中科院分区：
文献类型：
--
作者：
GILLIAM, JN;PRYSTOWSKY, SD
15例正常皮肤直接免疫荧光和血清抗核糖核酸酶敏感的可提取核抗原(ENA)抗体阳性的患者，以弥漫性无瘢痕和局限性脱发、色素异常、手部肿胀伴硬指症、慢性皮肤红斑狼疮(LE)为最常见的皮肤病特征。直接免疫荧光显示15例患者均为颗粒状(斑点)的表皮核染色，5例为表皮下免疫球蛋白沉积。高滴度的核糖核蛋白抗体与这种特殊类型的表皮核染色有关。这些发现为侵袭性较弱的LE亚型提供了易于检测的标志物，其特征是与混合性结缔组织病相一致的独特的临床和实验室特征。
Fifteen patients with epidermal nuclear staining on direct immunofluorescence of normal skin and high titer serum antibody to RNase sensitive extractable nuclear antigen (ENA) had diffuse nonscarring and focal alopecia, abnormal pigmentation, swollen hands with sclerodactyly, and chronic cutaneous lupus erythematosus (LE) as the most common dermatologic features. Direct immunofluorescence of normal, unexposed skin revealed a particulate (speckled) epidermal nuclear staining pattern in all 15 patients and subepidermal immunoglobulin deposits in 5. Ribonucleoprotein antibodies in high titer are associated with this characteristic type of epidermal nuclear staining. These findings provide easily detectable markers for a less aggressive subset of LE characterized by distinctive clinical and laboratory features consistent with mixed connective tissue disease.