Bullous Systemic Lupus Erythematosus

Abstract

A 22-year-old woman presented to the internal medicine department with a 2 month history of progressive blistering eruption. She also complained of arthralgias and lower limb edema. Physical examination found blisters on her hands (Picture 1) and feet. Skin biopsy showed subepidermal blister with polymorphous inflammatory infiltrate (Picture 2). Direct immunofluorescence demonstrated linear deposits of IgG, IgM and complement at the dermoepidermal junction (Picture 3). Anti-nuclear antibody was positive. Complement components were decreased. Anti-double stranded DNA antibody was elevated. The patient was anemic. As she had proteinuria and hypoalbuminemia, a kidney biopsy was performed which showed changes consistent with class VI lupus nephritis. The diagnosis of bullous systemic lupus was made. The patient was treated with Mycophenolate mofetil and methylprednisolone pulses relayed by oral prednisone with a clinical improvement of the skin and renal lesions.Bullous lupus erythematosus is a rare autoimmune disease. The diagnostic criteria were proposed by Camisa and Sharma in 1983 (1). Dapsone is the essential treatment for lupus to control bullous skin lesions. Steroids and immunosuppressive drugs prescribed in cases of associated visceral lesions are equally effective for skin lesions (2). The authors state that they have no Conflict of Interest (COI). References1. Camisa C, Sharma HM. Vesiculobullous systemic lupus erythematosus. J Am Acad Dermatol 9: 924-933, 1983. 2. Vassileva S. Bullous systemic lupus erythematosus.

Bullous Lupus: An Atypical Case of Refractory Disease in a Patient with Sulfa Allergy

“…However, the neutrophils tend to be in a dispersed arrangement and may extend into the papillary dermal collagen in BSLE, whereas in DH, the neutrophilic infiltrate is focal and localized at the papillary dermis. In comparison, EBA has much less dermal inflammatory infiltrate [ 4 , 7 , 16 ].…”

“…However, the neutrophils tend to be in a dispersed arrangement and may extend into the papillary dermal collagen in BSLE, whereas in DH, the neutrophilic infiltrate is focal and localized at the papillary dermis. In comparison, EBA has much less dermal inflammatory infiltrate [4,7,16]. e immunopathology findings in BSLE include direct IF with linear IgG deposits at the basement membrane and occasional granular IgA, IgM, and C3 deposits [1,2], unlike DH and linear IgA bullous dermatosis where IgA is the unique immunoglobulin present at the direct IF.…”

“…Dapsone is considered first-line treatment. In cases of contraindication or systemic SLE manifestations, immunosuppressive drugs and corticosteroids can be used [6][7][8]. We present a case of BSLE with concomitant lupus nephritis and autoimmune hemolytic anemia refractory to systemic corticosteroids and other immunosuppressant drugs, requiring dapsone after desensitization due to prior history of angioedema with sulfa drugs.…”

Bullous Systemic Lupus Erythematosus and Cicatricial Pemphigoid

“…If there is, pruritus is mild. In contrast to DH, lesions are less often seen on extensor surfaces, however some BSLE cases involving extensors such as hands, knees, or feet have been reported [46,47].…”

Bullous systemic lupus erythematosus associated with increased activity of lupus nephritis: a case report and review of the literatur

“…Bullous systemic lupus erythematosus can be the initial presentation of SLE or it may be associated with SLE flare [ 2 , 3 ]. The association between BSLE and increased activity of lupus nephritis has been particularly underscored by several authors [ 4 – 9 ].…”