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Bullous Systemic Lupus Erythematosus (BSLE)

Background

Bullous systemic lupus erythematosus is an autoantibody-mediated subepidermal blistering disease that occurs in patients with systemic lupus erythematosus (see the image below).

Tense vesiculobullous lesions on the neck of a pat

Tense vesiculobullous lesions on the neck of a patient with bullous systemic lupus erythematosus.

The diagnosis of bullous systemic lupus erythematosus requires the following elements (see Presentation and Workup):

Fulfillment of the American College of Rheumatology criteria for systemic lupus erythematosus (see also Systemic Lupus Erythematosus)

An acquired vesiculobullous eruption (see the image above)

Histologic evidence of a subepidermal blister and a predominantly neutrophilic dermal infiltrate

Direct immunofluorescence (DIF) microscopy demonstrating immunoglobulin G (IgG, with or without immunoglobulin A [IgA] and immunoglobulin M [IgM]) deposits at the basement membrane zone (BMZ)

Evidence of antibodies to type VII collagen via DIF or indirect immunofluorescence (IIF) on salt-split skin, immunoblotting, immunoprecipitation, enzyme-linked immunosorbent assay (ELISA), or immunoelectron microscopy

All 5 criteria listed above are needed for a diagnosis of type 1 bullous systemic lupus erythematosus, whereas only the first 4 criteria are needed to diagnose type 2 (undetermined location of antigen or dermal antigen other than type VII collagen) and type 3 (epidermal antigen) bullous systemic lupus erythematosus. Type VII collagen, a component of anchoring fibrils, is also targeted in epidermolysis bullosa acquisita (EBA). However, unlike epidermolysis bullosa acquisita, bullous systemic lupus erythematosus tends to respond dramatically to treatment with dapsone. (See Differentials and Treatment.)

Not all blistering eruptions that occur in patients with lupus erythematosus represent bullous systemic lupus erythematosus as defined above. Vesiculobullous skin lesions can also develop as a result of extensive damage to the epidermal basal layer (and even suprabasal keratinocytes), due to an intense interface dermatitis in the setting of lupus erythematosus–specific skin disease. Such patients may present with a severe form of acute or subacute cutaneous lupus erythematosus (SCLE) that resembles erythema multiforme (Rowell syndrome)
or toxic epidermal necrolysis (TEN). (See Differentials.)

Because epidermolysis bullosa acquisita and bullous systemic lupus erythematosus share the same target antigen, distinguishing between the 2 may be difficult. (See Etiology below.)

 For patient education information, see Lupus (Systemic Lupus Erythematosus).

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