Description

Bullous systemic lupus erythematosus (BSLE) is a rare form of cutaneous lupus.


Clinical features:

(1) tense vesiculobullous eruption

(2) more often seen in sun-exposed skin

(3) pruritic

(4) may be drug-induced

 

Autoantibodies may include:

(1) type VII collagen

(2) BP180 and/or BP230

(3) laminin 5 and/or laminin 6

 

A biopsy of affected skin shows:

(1) subepidermal blisters

(2) neutrophilic infiltrate along the basement membrane

(3) IgG deposition at the dermo-epidermal junction on immunofluorescence.

 

Histologic subtypes:

(1) dermatitis herpetiformis-like

(2) epidermolysis bullosa acquisita-like

(3) bullous pemphigoid-lie

 

The patient may also have:

(1) esophagitis with odynophagia

(2) lupus nephritis

(3) other mucosal involvement

 

The diagnosis requires exclusion of other conditions with bullae.


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