Role of Proteasomes in a Dermatological Autoimmune Disease: Bullous Pemphigoid

Role of Proteasomes in a Dermatological Autoimmune Disease: Bullous Pemphigoid

The primary objective of this study is to describe and compare plasmatic anti-proteasome auto-antibody concentrations among three distinct groups: (1) patients suffering from bullous pemphigoide; (2) patients suffering from other dermatological auto-immune diseases; (3) an elderly control group.

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.

Inclusion Criteria:

• The patient must have given his/her informed and signed consent
• The patient must be insured or beneficiary of a health insurance plan
• The patient is not taking systemic treatment
• The patient has not been treated with topical steroids for more than 15 days.

For the bullous pemphigoid group:

• clinical signs: erythematous-based lesions, especially on flexion areas of the arms and legs, not afflicting mucous membranes, and without atrophic scaring
• histology: without epidermal acantholysis

For the pemphigus group:

• patient with pemphigus

For the lupus group:

• systemic lupus patients: presence of the 4 diagnostic criteria for systemic lupus erythematosus as defined by the American College of Rheumatology (amended 1997)
• or characteristics of subacute cutaneous lupus: clinical, histological and immunological (anti-SSa)
• or clinical and histological characteristics of chronic lupus

For the control group:

• hospitalized patients with no history of auto-immune, inflammatory or evolving neoplastic disease

Exclusion Criteria:

• The patient is participating in another study
• The patient is in an exclusion period determined by a previous study
• The patient is under judicial protection, under tutorship or curatorship
• The patient refuses to sign the consent
• It is impossible to correctly inform the patient
• The patient is pregnant, parturient, or breastfeeding

For patients with bullous pemphigoid, pemphigus or lupus:

• The patient is taking systemic treatment
• The patient has been taking topical steroids for more than 15 days.

For the controls:

• autoimmune disease
• inflammatory disease
• evolving neoplastic disease
• surgery during the last 6 months