Risk Factors for Mucosal Involvement in Bullous Pemphigoid and the Possible Mechanism: A Review

Abstract

Bullous pemphigoid (BP) is the most common type of autoimmune bullous disease and is characterized by the presence of circulating anti-BP180 and/or anti-BP230 autoantibodies. Patients with BP often present with tense blisters and erythema, mainly on the trunk and limbs, but a few patients also have mucosal involvement. In this article, we discuss the fact that BP patients with mucosal involvement tend to have more serious conditions and their disease is more difficult to control. Potential risk factors for mucous involvement include earlier age at onset, drugs such as dipeptidyl peptidase-4 inhibitors, cancer, and blood/serum biomarkers, including lower eosinophil count, higher erythrocyte sedimentation rate, IgG autoantibodies against both the NH2- and COOH-termini of BP180, and the absence of anti-BP230 antibodies. IgA and C3 deposition at the dermo-epidermal junction may also be present. Understanding these risk factors may benefit earlier diagnosis of these patients and promote the development of novel treatments. What's more, it's helpful in deeper understanding of BP development and the relationship between BP and mucous membrane pemphigoid (MMP).

An Unusual TEN-Like Presentation of Juvenile Bullous Pemphigoid: A Diagnostic Challenge

“…Mucosal lesions are rare in BP, and if present, may be a sign of a more severe and refractory disease [ 7 ]. The presence of oral lesions in our case and the relatively intractable course of the disease further supports this idea.…”

Desquamative Gingivitis in the Context of Autoimmune Bullous Dermatoses and Lichen Planus—Challenges in the Diagnosis and Treatment

“…Clinically, BP is characterized by the presence of large, tense blisters that may begin in the form of erythematous macules, urticarial papules, or plaques [ 3 ] electively located on the trunk and limbs, but there may be atypical lesions involving the mucous membranes [ 53 ].…”

“…The diagnosis of this disease is based on the clinically suggestive appearance of the lesions (large, tense blisters that do not extend peripherally and have a spontaneous tendency to heal), histopathological examination (subepidermal blister, lack of acantholysis), direct immunofluorescence (linear IgG/C deposits in the basement membranel) and serological investigations. Serum autoantibody levels can be detected using enzyme immunoassays (ELISA) [ 53 , 56 ]. There are insufficient studies in the literature to correlate the clinical severity of the disease with the level of antibodies in the serum with the presence of proteins in the basement membrane.…”

“…BP blisters often heal without scarring, being a self-limiting condition, unlike CP, which has a chronic course and the healing of the lesions is sometimes accompanied by a loss of substance. The diagnosis of CP with bullous skin lesions or the diagnosis of BP with extensive oral lesions remains a challenge, especially in the presence of a patient with anti-BP180 autoantibodies present [ 53 ].…”

Deciphering the Contribution of BP230 Autoantibodies in Bullous Pemphigoid

“…Patients also typically experience severe pruritus. Mucosal involvement, almost invariably limited to the oral cavity, can be seen in nearly one-fifth of patients [ 2 , 3 , 4 ].…”