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Clinical Features of Bullous Systemic Lupus Erythematosus

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Abstract

Bullous systemic lupus erythematosus (BSLE) is a rare bullous dermatosis in patients with systemic lupus erythematosus. It is characterised by clinical and histological features resembling either bullous pemphigoid or dermatitis herpetiformis and a heterogeneous immunological profile, characterised by autoimmunity to components of type VII collagen, much like epidermolysis bullosa acquisita. As our understanding of the pathology of this interesting dermatological condition has evolved, so too have the criteria and profiling of BSLE. The distinct clinical, histological and immunological features of BSLE represent a unique bullous disease phenotype.

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Abbreviations

ARA:

American Rheumatology Association

BMZ:

Basement membrane zone

BP:

Bullous pemphigoid

BSLE:

Bullous systemic lupus erythematosus

DH:

Dermatitis herpetiformis

DIF:

Direct immunofluorescence

EBA:

Epidermolysis bullosa acquisita

IEM:

Immunoelectron microscopy

IIF:

Indirect immunofluorescence

LAD:

Linear IgA disease

NC:

Non-collagenous

SLE:

Systemic lupus erythematosus

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Correspondence to Dédée F. Murrell MA(Camb),BMBCh(Oxf),FAAD,MD,FACD,FRCP .

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Sebaratnam, D.F., Woodley, D.T., Murrell, D.F. (2015). Clinical Features of Bullous Systemic Lupus Erythematosus. In: Murrell, D. (eds) Blistering Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45698-9_41

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  • DOI: https://doi.org/10.1007/978-3-662-45698-9_41

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