Mucous Membrane Pemphigoid-Associated Malignancies. Case Series and a Brief Overview of the Literature

Abstract

Background: Mucous membrane pemphigoid (MMP) is a heterogeneous group of blistering disorders affecting the mucosae with or without skin involvement, characterized by the presence of autoantibodies to components of the basement membrane zone, including the bullous pemphigoid antigen BP180 and β4 integrin. Current literature has shown that a minority of patients present circulating antibodies to laminin-332 and this population seems to be associated with a relatively high risk of malignancy. Objective: To present our personal case series of patients with MMP-associated malignancy from a dermatology university hospital. Methods: Twenty-two patients affected by MMP were seen in the period between 2001 and 2016; in 4 patients (18%) an associated cancer was detected. Results: These patients were 2 men and 2 women, with a mean age of 69.7 years (range, 48-83). The associated malignancies included a breast cancer, a pancreatic adenocarcinoma, a metastatic laryngeal carcinoma, and a hepatic carcinoma. All patients had negative results for both BP180 and laminin-332 autoantibodies. Conclusion: We confirm that MMP patients have a relatively high possibility of developing a solid cancer, but the autoantibody detection is not mandatory and is probably correlated with the severity of the disease.

Keywords

• mucous membrane pemphigoid
• malignancy
• autoimmune bullous disorders
• paraneoplastic pemphigus
• laminin-332
• blistering diseases