BULLOUS SYSTEMIC LUPUS ERYTHEMATOSUS: A CASE SERIES AND 5-YEAR RETROSPECTIVE REVIEW

Abstract

Bullous systemic lupus erythematosus (BSLE) is a rare presentation of systemic lupus erythematosus (SLE) that mainly occurs in females (30-40 years old) and less frequently in children and adolescents. BSLE has many clinical and histological feautures that may lead to misdiagnosis and delayed treatment.

We report 14 patients with blistering lesions at admission, who were diagnosed with BSLE over the last 5 years in The National Hospital of Dermatology and Venereology (NHDV), Vietnam. Our cases were reviewed carefully of the clinical presentation, histopathology, immunofluorescence and therapeutic regimens. Because these manifestations may be confused with several other blistering diseases, such as bullous pemphigoid (BP), dermatitis herpetiformis (DH) or epidermolysis bullosa acquisita (EBA). All of our cases presented with the rapid and widespread development of tense vesicles and bullae over erythematous macules or plaques predominantly on the face, neck, upper extremities and trunk with symmetrical distribution. Mucosal involvement is common on oral and genital areas. The lesions usually progress without scarring and milia, but hypo or hyperchromia may be present. These cases shared the histopathology in common with a subepidermal bullae with neutrophil-predominant inflammatory infiltrate below the bullae formation. Both the direct and indirect immunofluorescence findings were made to confirm the diagnosis of BSLE. Some of our cases were well-responded to treatment with dapson, but several ones required systemic corticosteroids and immunosuppressants. We emphasize the relevance of recognizing BSLE - a rare presentation of SLE - which may evolve with marked clinical presentation.