Epidermolysis bullosa acquisita in a 53-year-old female with extensive cutaneous and mucosal involvement
DOI:
https://doi.org/10.18203/issn.2455-4529.IntJResDermatol20251054Keywords:
Epidermolysis bullosa acquisita, Autoimmune blistering disorder, Sub-epidermal blister, Direct immunofluorescence, Mucosal involvement, Type VII collagenAbstract
Epidermolysis bullosa acquisita (EBA) is a rare autoimmune blistering disorder characterized by subepidermal blisters, typically caused by an autoimmune response against type VII collagen, a key structural component of the dermal-epidermal junction. Here, we report a case of EBA in a 53-year-old female with extensive multiple bullae, erosions over extremities, trunk, genitaila. HPE revealed a sub-epidermal blister with fibrin, neutrophils, and some eosinophils, DIF showed IgG positivity (+3) in a linear pattern along the dermal-epidermal junction. enzyme immunoassay (EIA) for anti-collagen type VII antibodies was positive. Based on clinical features and investigations patient is diagnosed as Epidermolysis Bullosa Acquisita. She was treated with systemic steroids and topical steroids with gradual tapering. This case highlights the importance of early diagnosis and treatment in managing this condition effectively.
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