Paraneoplastic epidermolysis bullosa acquisita associated with thyroid carcinoma

Epidermolysis bullosa acquisita (EBA) is a rare acquired subepithelial blistering disease of the skin and mucous membranes associated with autoantibodies against type VII collagen (1, 2). Two main clinical variants are recognized: a classic mechanobullous and an inflammatory type (1, 2). Inflammatory EBA mimics other autoimmune bullous diseases and has at least 4 different clinical presentations: bullous pemphigoid (BP)-like EBA, mucous membrane pemphigoid (MMP)-like EBA, Brunsting-Perry pemphigoid-like EBA and linear IgA bullous dermatosis-like EBA (1, 2). Clinical features, histopathological or immunofluorescence studies are necessary to identify EBA, but they are not sufficient to confirm the diagnosis (1, 2). For a long time direct immunoelectron microscopy has represented the gold standard for EBA diagnosis (2). However, nowadays other methods such as u-serrated pattern analysis by direct immunofluorescence (DIF) microscopy (3), together with detection of circulating autoantibodies to type VII collagen by enzyme-linked immunosorbent assay (ELISA) or immunoblotting (IB) (4), are most frequently used due to their easier availability and feasibility. EBA has frequently been found in association with systemic diseases, in particular inflammatory bowel disease and systemic lupus erythematosus, but also rheumatoid arthritis, amyloidosis, diabetes mellitus, thyroiditis and other endocrinopathies (1, 2). Association with haematological diseases and solid tumours has also been described (5-14). We report here a case of BP-like inflammatory EBA in which search for an associated disease, prompted by treatment resistance, disclosed an underlying thyroid papillary carcinoma.