Bullous Systemic Lupus Erythematosus: Case report

Abstract

Summary Introduction: Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated disease with subepidermal blisters. It is a rare form of presentation of SLE that occurs in less than 5% of cases of lupus. Case Report: A 27-year-old, female, FRS patient reported the appearance of painful bullous lesions in the left nasal wing and left buccal mucosa that displayed sudden and rapid growth. She sought advice from emergency dermatology staff 15 days after onset and was hospitalized with suspected bullous disease. Intravenous antibiotics and steroids were administered initially, but the patient showed no improvement during hospitalization. She displayed further extensive injuries to the trunk, axillae, and vulva as well as disruption of the bullous lesions, which remained as hyperemic scars. Incisional biopsy of a lesion in the left buccal mucosa was performed, and pathological results indicated mucositis with extensive erosion and the presence of a predominantly neutrophilic infiltrate with degeneration of basal cells and apoptotic keratinocytes. Under direct immunofluorescence, the skin showed anti-IgA, anti-IgM, and anti-IgG linear fluorescence on the continuous dermal side of the cleavage. Indirect immunofluorescence of the skin showed conjugated anti-IgA, was anti-IgM negative, and displayed pemphigus in conjunction with anti-IgG fluorescence in the nucleus of keratinocytes, consistent with a diagnosis of bullous lupus erythematosus. Discussion: BSLE is an acquired autoimmune bullous disease caused by autoantibodies against type VII collagen or other components of the junctional zone, epidermis, and dermis. It must be differentiated from the secondary bubbles and vacuolar degeneration of the basement membrane that may occur in acute and subacute cutaneous lupus erythematosus.

Bullous Systemic Lupus Erythematosus Mimicking Bullous Pemphigoid: A Case Report

“…Skin lesions occur in areas exposed to sunlight and flexure areas. 1 Bullous type systemic lupus erythematosus is rare; less than 5% of SLE cases occur. The mean incidence of BSLE was estimated to be 0.22 per million in three French regions.…”

“…[1][2][3] Clinical symptoms of BSLE patients include vesicles or tense bullae filled with serous or hemorrhagic fluid, spreading rapidly throughout the body, regarding areas exposed to sunlight, the neck, body, or areas not exposed to the sun and can affect the mucosa. 1 On BSLE negative indirect immunofluorescence examinations, positive direct immunofluorescence was found to be deposition linear or granular IgG and/or IgM and IgA frequently in the basement membrane zone (dermo-epidermal junction). 1,4 C3 can also be found but rare.…”

“…Histopathological examination of BSLE found sub-epidermal bullae in the presence of neutrophil infiltrates in the upper dermis with microabscess formation on papillary. 1,5 Differential diagnoses of this disease are dermatitis herpetiformis and bullous pemphigoid, which are clinically similar but different in histopathology and immunofluorescence examinations. 2,3 Bullous pemphigoid (BP) is an acquired, acute or chronic, inflammatory, subepidermal, immunobullous disease.…”

“…2 Azathioprine 100 mg / day, cyclophosphamide 100 mg / day, chloroquine (4 mg / kg), or mycophenolate mofetil (2.5-3 mg / kg) could be given in this patient. 1,2 It could be given as a combination of azathioprine with prednisone if there is no response to dapsone. 1,5…”

“…1,2 It could be given as a combination of azathioprine with prednisone if there is no response to dapsone. 1,5…”