| • | an example of LE-nonspecific bullous skin change |
| • | when patients with SLE develop autoantibodies to type VII collagen, they develop skin blisters and fall into a subset called “bullous SLE” |
| • | most commonly seen in young black women |
| • | the activity of the blistering disorder does not necessarily coincide with the activity of the systemic disease |
| • | interestingly, the primary lesions of chronic, subacute, and acute LE appear to be uncommon in bullous SLE |
| • | the histologic features of primary lupus lesions such as epidermal atrophy, basal keratinocyte vacuolization, basement membrane thickening, and chronic inflammation are absent |
| • | has the following in common with EBA: |
| • | antibodies recognize EBA antigen |
| • | the split occurs in the sublamina densa zone |
| • | indirect immunofluorescence may be positive in intact or separated skin |
| • | presence of HLA-DR2 is markedly increased in both conditions |
| • | distinctive features include: |
| • | a diagnosis of SLE by criteria of the ARA |
| • | a widespread, non-scarring vesicobullous eruption |
| • | (skin fragility, scarring, and milia are not features) |
| • | often begins in second or third decade (EBA in fourth or fifth) |
| • | histology characteristically like that of dermatitis herpetiformis (rare in EBA) |
| • | remitting and often resolves in less than a year (EBA often lasts many years) |
| • | most patients respond dramatically to dapsone (there is no one medication that EBA patients will predictably respond to) |
| • | several authors have reported cases of EBA that later developed into true SLE; should patients with EBA be monitored for evidence of SLE? |
Vesicobullous Skin Changes in LE:
LE-specific: may develop in ACLE or SCLE or DLE as manifestation of particularly aggressive liquefactive degeneration of the basal layer
LE-nonspecific: bullous SLE; pemphigus erythematosus
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