Diagnosis Of Bullous Systemic Lupus Erythematosus Research Articles

Clinical presentation of bullous systemic lupus erythematosus in a pediatric patient: a case report

Bullous systemic lupus erythematosus (BSLE) is a rare blistering eruption that can occur in patients with systemic lupus erythematosus. Although 59 to 85% of patients will have skin manifestations, less than 5% will develop the bullous disease, which is even rarer (1%) in the pediatric population. We present the case of a 16-year-old female patient in whom a diagnosis of BSLE was made through clinical presentation, histological study by biopsy, and direct immunofluorescence. Due to the low incidence of cases reported in pediatric patients, it is possible that the diagnosis may not be made immediately, which is why we emphasize the importance of obtaining an accurate diagnosis and providing timely treatment.

Bullous Systemic Lupus Erythematosus

Bullous systemic lupus erythematosus (BSLE) is extremely rare but distinct disease, characterized by vesicobullous skin eruptions in systemic lupus erythematosus (SLE). It can develop either before or after a diagnosis of SLE has been established. BSLE is characterized by a dermatitis herpetiformis-like histology and an autoimmunity to type VII collagen. It must be differentiated from other autoimmune vesicobullous diseases such as epidermolysis bullosa acquisita, dermatitis herpetiformis, linear IgA disease, and bullous pemphigoid. Its important to combine clinical, histological, and immunofluorescence findings to establish a diagnosis of BSLE. We report a case of BSLE to illustrate and emphasize the need for an integrative diagnostic approach.J MEDICINE JUL 2018; 19 (2) : 123-125

Bullous systemic lupus erythematosus

Bullous Systemic Lupus Erythematosus (BSLE) is a rare manifestation of Systemic Lupus Erythematosus (SLE), characterized histologically by subepidermal bullae with predominantly neutrophilic infiltrate and immunoglobulin (IgG, IgA, IgM) and C3 deposition at the basement membrane zone. Often associated is a heterogeneous profile of autoimmunity to components of type VII collagen. It needs to be differentiated from other subepidermal bullous diseases in view of potentially associated systemic manifestations of SLE. We report a 39-year-old lady who presented with an extensive vesicobullous eruption on face, neck, trunk, and mucosa. She was also found to have photosensitivity, significant anemia, and high antibody titers confirming SLE. Routine histopathology and immunoflorescence studies suggested BSLE and she responded favorably to dapsone. The importance of clinical and histopathological findings in confirming a diagnosis of BSLE along with the very encouraging response to dapsone therapy is highlighted.