Autoimmune Bullous Dermatoses
Conditions
Brief summary
Autoimmune bullous dermatoses are a group of diseases with chronic course. They are provoked by the production of autoantibodies against the dermal-epidermal junction or against the inter-keratinocyte junctions, resulting in the formation of intra-epidermal or sub-epidermal blisters. The diagnosis of autoimmune bullous dermatoses is based on clinical and immunopathological findings, including skin direct immunofluorescence. Systemic corticosteroid therapy is generally considered as the mainstay of treatment for many years both for bullous pemphigoid and pemphigus which are the most frequent diseases.
Detailed description
The aim of the study is the creation of a register of patients suffering from autoimmune bullous dermatoses to quickly identify patients who can be included in clinical trials or retrospective epidemiological studies
Interventions
OTHERData collection
Sponsors
CHU de Reims
Study design
Observational model
CASE_ONLY
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* patients with autoimmune bullous dermatosis * patients cared for in dermatology referral center of Reims, Rouen or Limoges * patient consenting to participate to the study
Exclusion criteria
\- patient \<18 years
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| autoimmune bullous dermatosis according to physical examination, skin biopsy and autoantibodies detection in blood sample | Day 0 | Each autoimmune bullous dermatosis is classified according one of the following diagnoses : 1/ bullous pemphigoid, 2/ Mucous membrane pemphigoid, 3/ Pemphigoid gestationis, 4/ Epidermolysis bullosa acquisita, 5/ Linear IgA bullous dermatosis, 6/ pemphigus (vulgaris or foliaceus) This classification will be performed according : * clinical characteristics including location, clinical aspect and number of cutaneous and/or mucosal blisters * skin biopsy for routine histology (subepidermal blister or intra-epidermal blister) and direct immunofluorescence (IgG and/or I gA and/or C3 deposits along the epidermal basement membrane zone or on the cell surface of keratinocytes) * blood sample for serum autoantibodies detection against epidermal autoantigens using ELISA or indirect immunofluorescence techniques |
Countries
France
Outcome results
None listed