Bullous systemic lupus erythematosus

Abstract

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.

Keywords: Dapsone; immunobullous diseases; type VII collagen.

Figure 1

Presence of tense vesicles (marked with an arrow) filled with cleat fluid along with superficial erosions with polycyclic margins. Lesions are present on covered as well as exposed areas

Figure 2

Biopsy taken from vesicular lesion revealed a subepidermal split, containing numerous red blood cells. A marked neutrophilic infiltrate is seen in the papillary dermis (H and E, ×250). Inset shows a positive lupus band (IgG) at the basement membrane zone)

Figure 3

Marked improvement seen 1 week after starting dapsone therapy