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An uncommon presentation of an uncommon disease: relapsing polychondritis overlap with systemic lupus erythematosus

机译:一种罕见的疾病呈现:与系统性红斑狼疮复发多档炎重叠

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Relapsing polychondritis (RP) is a rare rheumatologic disorder in which recurrent episodes of inflammation result in destruction of cartilage of the ears and nose. The joints, eyes, audio-vestibular system, heart valves, respiratory tract, kidneys, and skin can also be involved. Skin involvement is most frequently linked to concomitant myelodysplastic syndrome and has rarely been associated with systemic lupus erythematosus. A 47-year-old woman presented with violaceous, indurated, tender plaques on the bilateral cartilaginous ears with sparing of the lobes, consistent with RP. Further investigations revealed positive ANA and anti-Smith antibody, oral ulcers, a photo-distributed skin eruption, and biopsy-proven lupus nephritis, leading to a second concomitant diagnosis of systemic lupus erythematosus (SLE). The diagnosis of SLE associated with RP was made and the patient was started on oral prednisone and hydroxychloroquine. This is a rare report of SLE associated with RP. It is unclear whether RP occurring in patients with SLE represents another clinical manifestation of SLE or a coexisting disease. However, a significant ANA titer in a patient with RP strongly suggests the presence of an associated autoimmune disorder. If immunologic abnormalities usually found in SLE are detected in patients with RP, it is important to monitor patients for the development of other manifestations of SLE.
机译:复发多层炎(RP)是一种罕见的风湿病疾病,其中炎症的复发事件导致耳朵和鼻子的软骨破坏。联合,眼睛,音频口庭系统,心脏瓣膜,呼吸道,肾脏和皮肤也可以参与其中。皮肤参与最常与伴随的髓细胞增强综合征有关,并且很少与Systemic Lupus红斑狼疮有关。一名47岁的女性在双侧软骨耳朵上呈现紫罗兰,诱人的柔软斑块,其伴有裂片,与RP一致。进一步的研究显示阳性ANA和抗史密斯抗体,口腔溃疡,皮肤分布的皮肤喷发和活组织检查验证的狼疮性肾炎,导致Systemic Lupus红斑(SLE)的第二个伴随诊断。制备与RP相关的SLE的诊断,并在口服泼尼松和羟氯喹开始患者。这是与RP相关的SLE的罕见报告。目前尚不清楚SLE患者的RP是否发生了SLE或共存疾病的另一种临床表现。然而,患有RP的患者的重要ANA滴度强烈表明存在相关的自身免疫疾病。如果在RP患者中检测到SLE中通常发现的免疫学异常,则重要的是监测患者的SLE其他表现的患者。

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