Role of Proteasomes in a Dermatological Autoimmune Disease: Bullous Pemphigoid

• Plasmatic concentration of anti-proteasome autoantibodies (ng/ml) [ Time Frame: baseline ]
  

• the daily number of new lesions [ Time Frame: baseline ]
  For patients suffering from bullous pemphigoid or pemphigus: the daily number of new lesions for the 3 days preceding blood sampling
  
  
• Presence/absence of mucosal disease [ Time Frame: baseline ]
  For patients suffering from bullous pemphigoid only
  
  
• Disease duration (weeks) [ Time Frame: baseline ]
  For patients suffering from bullous pemphigoid or pemphigus or lupus
  
  
• % surface area [ Time Frame: baseline ]
  For patients suffering from bullous pemphigoid or pemphigus or lupus: % of skin area affected in relation to total area
  
  
• Puritis score [ Time Frame: baseline ]
  For patients suffering from bullous pemphigoid only: severity of itching on a analog scale varying from 0 to 6
  
  
• concentration of anti-PB18 antibodies, measured by ELISA [ Time Frame: baseline ]
  For patients suffering from bullous pemphigoid only; U/ml
  
  
• Immunohistochemistry [ Time Frame: baseline ]
  For the first 10 patients suffering from bullous pemphigoid included at the Nîmes University Hospital only; Skin biopsy immunohistochemistry scores for the pan-alpha, alpha6, beta1, beta2, beta1i, beta5i and rpt5 subunits (negative, weak, moderate, strong)
  
  
• Tissue DNA expression [ Time Frame: baseline ]
  For the first 10 patients suffering from bullous pemphigoid included at the Nîmes University Hospital only; Skin biopsy, plasma and circulating mononuclear cell DNA expression for the pan-alpha, alpha6, beta1, beta2, beta1i, beta5i and rpt5 subunits (weighted by beta-actin)
  
  
• presence/absence of oral lesions [ Time Frame: baseline ]
  For patients suffering from pemphigus
  
  
• Presence/absence of Nikolsky's sign [ Time Frame: baseline ]
  For patients with pemphigus only
  
  
• Pemphigus disease area index [ Time Frame: baseline ]
  For patients with Pemphigus only; score varying from 0 to 120.
  
  
• Anti-desmogleine 1 and 3 antibody concentrations [ Time Frame: baseline ]
  For patients with Pemphigus only; ELISA (U/ml
  
  
• CLASI score for lupus [ Time Frame: baseline ]
  for lupus patients only; score varying from 0 to 70
  
  
• Karnofsky's score (%) [ Time Frame: baseline ]
  
• Plasma proteasome concentration [ Time Frame: baseline ]
  ng/ml
  
  
• % trypsin-like plasma proteasome proteolytic activity [ Time Frame: baseline ]
  
• % chymotrypsin-like plasma proteasome proteolytic activity [ Time Frame: baseline ]
  
• % caspase-like plasma proteasome proteolytic activity [ Time Frame: baseline ]
  

The secondary objectives of this study are:

To compare the following parameters between the 3 groups:

• plasmatic proteasome concentrations
• plasmatic proteasome proteolytic activity

To explore the potential relationships between:

• plasmatic proteasome concentrations
• plasmatic proteasome proteolytic activity
• plasmatic anti-proteasome auto-antibody concentrations
• measures of disease severity for dermatological auto-immune diseases

To characterize plasmatic anti-proteasome auto-antibodies in patients suffering from bullous pemphigoide and other dermatological auto-immune diseases (other bullous auto immune diseases: pemphigus, cutaneous lupus, ...).

To characterize the expression and the activity of proteasomes in skin samples, in plasma and in circulating mononuclear cells in patients with bullous pemphigoide.