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.
Study Type
OBSERVATIONAL
Enrollment
14
CHU de Nîmes - Hôpital Universitaire Carémeau
Nîmes, France
Plasmatic concentration of anti-proteasome autoantibodies (ng/ml)
Time frame: baseline
the daily number of new lesions
For patients suffering from bullous pemphigoid or pemphigus: the daily number of new lesions for the 3 days preceding blood sampling
Time frame: baseline
Presence/absence of mucosal disease
For patients suffering from bullous pemphigoid only
Time frame: baseline
Disease duration (weeks)
For patients suffering from bullous pemphigoid or pemphigus or lupus
Time frame: baseline
% surface area
For patients suffering from bullous pemphigoid or pemphigus or lupus: % of skin area affected in relation to total area
Time frame: baseline
Puritis score
For patients suffering from bullous pemphigoid only: severity of itching on a analog scale varying from 0 to 6
Time frame: baseline
concentration of anti-PB18 antibodies, measured by ELISA
For patients suffering from bullous pemphigoid only; U/ml
Time frame: baseline
Immunohistochemistry
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)
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Time frame: baseline
Tissue DNA expression
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)
Time frame: baseline
presence/absence of oral lesions
For patients suffering from pemphigus
Time frame: baseline
Presence/absence of Nikolsky's sign
For patients with pemphigus only
Time frame: baseline
Pemphigus disease area index
For patients with Pemphigus only; score varying from 0 to 120.
Time frame: baseline
Anti-desmogleine 1 and 3 antibody concentrations
For patients with Pemphigus only; ELISA (U/ml
Time frame: baseline
CLASI score for lupus
for lupus patients only; score varying from 0 to 70
Time frame: baseline
Karnofsky's score (%)
Time frame: baseline
Plasma proteasome concentration
ng/ml
Time frame: baseline
% 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