Bullous systemic lupus erythematosus M32.9

Gammon and Briggaman 1993

Rare vesicular or bullous manifestation of systemic lupus erythematosus, with clinical and immunohistological features of bullous pemphigoid or epidermolysis bullosa acquisita (antibodies against collagen type VII).

Especially young women

In addition to the clinical features of systemic lupus erythematosus , vesicles and blisters that heal with or without scarring can be found on normal or reddened skin, especially in areas not exposed to light (Fujimoto W et al. 2005). Rarely, mucosal involvement is found, including involvement of the esophagus (Tanaka H et al. 2020). The association with HLA-DR 2 is common.

See also the clinical overlap with the"acute syndrome of apoptotic pan-epidermolysis" and Rowell's syndrome.

Subepidermal blistering with neutrophil-rich inflammatory infiltrate

Band-shaped deposits at the dermo-epidermal junction zone of IgG, often also IgA, IgM and C3.

In NaCl-separated split skin, antibodies against type VII collagen bind to the bottom of the bladder. Less frequently, antibodies against laminin 332, laminin 331 or BP230 are detected.

Epidermolysis bullosa acquisita: In the rare epidermolysis bullosa acquisita (EBA), blisters and erosions often occur on mechanically stressed areas of the body. Healing of the lesions usually occurs with scarring and milia formation and may lead to nail dystrophy and scarring alopecia. The autoantibodies are directed against collagen type VII in the sublamina densa. Accordingly, serum autoantibodies (IgG, less frequently IgA) bind to the dermal side of human split skin in indirect immunofluorescence. Specific detection of antibodies against collagen type VII is possible by immunoblotting of dermal extracts.

Antibodies against collagen type VII are also found in patients with bullous SLE. In most cases, MSD is chronically progressive. In contrast to bullous lupus erythemtodes, the majority of these patients do not respond to treatment with glucocorticoids, azathioprine or cyclophosphamide.

Therapy of the first choice is Dapson (!). Systemic glucocorticoids can be helpful in case of relapsing activities.

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3. Hall RP et al (1982) Bullous eruption of systemic lupus erythematosus. Dramatic response to dapsone therapy. Ann Intern Med 97:165-170.
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