Bullous Systemic Lupus Erythematosus: A Case Report And A Review Of The Literature

ObjectionThe aim is to investigate the clinical manifestations, auxiliary examination features, of Bullous systemic lupus erythematosus (BSLE) and to improve the acquaintance of this rare disease, to decrease the rate of misdiagnosis and missed diagnosis.MethodsWe retrospectively analyzed the clinical data of one case which is confirmed by department of dermatology Shandong University Qilu Hospital, and reviewed foreign and domestic literatures. And gave a Systematic exposition of the clinical manifestations, auxiliary examination and pathological features, diagnosis and treatment of BSLE.ResultsA 19-year-old woman with a 5 month history of systemic lupus erythematosus (SLE) developed a widespread urticated, erythematous eruption associated with tense, fluid-filled blisters, erosions and crusting. rheumatism series showed SSA (+), SSB (±), ANA 1:100(+), dsDNA(±), C30.36IU/ml, C4<0.063IU/ml, CRP 8.27mg/L urine analysis showed PRO (1+), BLD (2+), Routine analysis of blood showed WBC 11.15xl09/L, HGB 89g/L, RBC 3.0x1012/L, Biopsy showed subepidermal blistering with a prominent neutrophilic infiltrate. Direct immunofluorescence showed markedly positive granular IgG IgM IgA and C3 deposition at the dermoepidermal junction. A diagnosis of bullous systemic erythematosus was made. Treatment with Glucocorticoids was effective. ConclutionsBullous systemic lupus erythematosus (SLE) is a rare, distinctive subepidermal blistering disorder that occurs in systemic lupus erythematosus. This blistering disease presents itself with a widespread and non-scarring vesiculo-bullous eruption. The blistering eruptions are non-specific for LE and may also occur in LE in the context of a coexisting immunobullous disease. Pemphigus, bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA),dermatitis herpetiformis, and linear IgA bullous dermatosis have been all reported in association with LE. Their differentiation relies upon characteristic clinical, histologic, and immunopathologic features. View of Bullous systemic lupus erythematosus is a rare clinical disease and their clinical manifestations are not specific, and clinicians made more clinically misdiagnosis, which resulted in delays in patient condition. Therefore, clinicians should raise the awareness of the disease and ensure early detection, early diagnosis and early treatment.