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.
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
Study Type
OBSERVATIONAL
Enrollment
750
Chu Reims
Reims, France
autoimmune bullous dermatosis according to physical examination, skin biopsy and autoantibodies detection in blood sample
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
Time frame: Day 0
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.