We describe a patient with the rare diagnosis of 'Bullous Systemic Lup
us Erythematosus'. She is unusual in presenting with classical dermati
tis herpetiformis, before the emergence of systemic features. In addit
ion, on indirect immunofluorescence on sodium chloride split skin, the
re was epidermal binding. Immunoblotting was negative for type VII col
lagen.