Validation of a Simplified Severity Score (Investigator Global Assessment: IGA) in Bullous Pemphigoid

Validation of a Simplified Severity Score (Investigator Global Assessment: IGA) in Bullous Pemphigoid

The aim of the study is to validate a global and simple score : IGA (Investigator Global Assessment) score for the evaluation of the extent and severity of the disease in patients with bullous pemphigoid

Inclusion Criteria:

• Consecutive adult patients aged ≥ 18 years
• Newly diagnosed or relapsing BP
• Clinical features suggestive of classic BP AND suggestive histological features AND deposition of IgG and/ or C3 deposits on the dermal epiderma junction, AND detection of circulating anti-epidermal antibodies labelling the epidermal side of salt-split skin
• Patient having read and understood the information letter and not opposed to participation
• Must be willing and able to adhere to all specified requirements, including but not limited to adherence to the follow-up visits

Exclusion Criteria:

• Predominant or exclusive mucosal involvement leading to suspect the diagnosis of mucous membrane pemphigoid
• Pemphigoid gestationis
• Linear IgA dermatosis (predominant or exclusive IgA deposits on the DEJ)
• Skin lesions suggesting the diagnosis of epidermolysis bullosa acquisita (skin fragility, atrophy, milia)