Juvenile and adult vulvar pemphigoid, an under recognized entity: Case series of fourteen patients
Abstract
DIF: direct immunofluorescence microscopy EBMZ: epidermal basement membrane zone ELPV: erosive lichen planus affecting the vulva LS: lichen sclerosus MMP: mucous membrane pemphigoid VP: vulvar pemphigoid SSS: salt split skin IIF: indirect immunofluorescence microscopy ELISA: enzyme-linked immunosorbent assay C3c: complement component 3 Ig: immunoglobulin INTRODUCTION Vulvar pemphigoid (VP) is a rare subtype of mucous membrane pemphigoid (MMP), which is a heterogeneous group of autoimmune subepidermal blistering diseases with predominantly mucosal involvement and characterized by autoantibodies against structural proteins in the epidermal basement membrane zone (EBMZ). In MMP, various mucosal sites can be simultaneously or separately affected. Mucosal lesions tend to heal with scar formation and may result in loss of function of the affected area. In vulvar MMP, lesions are confined to the anogenital region but can also be a manifestation of a more extensive MMP with other mucosal involvement. Two variants can be distinguished: the juvenile form presenting in girls between 5 and 10 years old, and the adult form, which occurs mainly in postmenopausal women. However, symptoms may also occur in patients within these two age categories. Overlapping with other chronic vulvar diseases, VP can present with variable clinical and histopathologic features, including lichen sclerosus (LS) and erosive lichen planus affecting the vulva (ELPV). Careful examination of the mucosa and skin is…
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