Bullous Systemic Lupus Erythematosus: Case report

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.

Keywords

Lupus Erythematosus - Systemic - Stomatitis - Mucositis

• Reference

• 1 M Tincopa, Puttgen KB, Sule S, Cohen BA, Gerstenblith MR. Bullous lupus: an unusual initial presentation of systemic lupus erythematosus in an adolescent girl. Pediatr Dermatol 2010; Jul-Aug; 27 (4) 373-6
• 2 Bernard P, Vaillant L, Labeille B , et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions Bullous Diseases French Study Group. Arch Dermatol 1995; 131 (1) 48-52
• 3 Vieira FMJ, Oliveira ZNP. Bullous systemic lupus erythematosus. An Bras Dermatol 1998; 73: 143-7
• 4 Peter SD, Dahl MV. Direct immunofluorescence of Bullous systemic lupus erythematosus. Arch Dermatol 1973; 107: 118-20
• 5 Chan LS, LaPiere JC, Chen M, Traczyk T, Mancini AJ, Paller AS , et al. Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin-6, and type VII collagen. Arch Dermatol 1999; 135: 569-73
• 6 Obermoser G, Sontheimer RD, Zelger B. Overview of common, rare and atypical manifestations of cutaneous lupus erythematosus and histopathological correlates. Lupus 2010; Aug; 19 (9) 1050-70
• 7 Cato, Ellen Erie et al. Bullous systemic lupus erythematosus associated with lupus nephritis: report of two cases. An Bras Dermatol 2007; 82 (1) 57-61
• 8 Vassileva S. Bullous systemic lupus erythematosus. Clin Dermatol 2004; Mar-Apr; 22 (2) 129-38