Department of Dermatology, University of Oklahoma Health Science Center, Oklahoma City, Oklahoma, USA, Regional Medical Laboratory, Tulsa, OK.
Dermatol Online J. 2021 Feb 15;27(2):13030/qt1m50r4xk.
Rowell Syndrome is a rare presentation of lupus erythematosus manifesting as an eruption of erythema multiforme-like papules and plaques with immunological findings of positive rheumatoid factor, speckled antinuclear antibodies, and/or anti-Ro/La antibodies. This case highlights the unusual and highly debated presentation of Rowell Syndrome in a 66-year-old woman with newly-appearing erythematous, targetoid plaques in the setting of previously diagnosed systemic lupus erythematosus. Skin biopsy revealed histological features of full-thickness epidermal necrosis with focal sub-epidermal separation and a superficial perivascular lymphocytic infiltrate interpreted to favor Rowell Syndrome given her clinical history and presentation. Although no standard treatment exists, a prednisone taper and topical corticosteroids proved effective initially, with complete resolution at six months on mycophenolate mofetil and belimumab.
罗厄尔综合征是红斑狼疮的一种罕见表现,表现为多形红斑样丘疹和斑块的皮疹,免疫学检查可见类风湿因子阳性、斑点状抗核抗体和/或抗 Ro/La 抗体。本病例突出了罗厄尔综合征的一种不常见且极具争议的表现,发生于 1 例 66 岁女性,其先前诊断为系统性红斑狼疮,现出现新发红斑性靶形斑块。皮肤活检显示全层表皮坏死伴局灶性表皮下分离和浅表血管周围淋巴细胞浸润的组织学特征,鉴于其临床病史和表现,支持罗厄尔综合征的诊断。虽然尚无标准治疗方法,但泼尼松减量和局部皮质类固醇最初有效,在改用霉酚酸酯和贝利尤单抗后 6 个月完全缓解。