Assessment of the Prevalence of Mucosal Involvement in Bullous Pemphigoid

Abstract

ost patients with bullous pemphigoid (BP) classically present with tense blisters and erythema, seen predominantly in the limb flexures and the abdomen, and often in conjunction with urticarial plaques. Meanwhile, 20% present with atypical manifestations, including prurigo-like type, 1 urticaria-like type, 2 eczema-like type, 3 and dyshidrosiform type. 4 Involvement of the mucous membranes was considered an atypical physical feature by the clinical criteria of Vaillant et al, 5 which yields a positive predictive value of 95% for a clinical diagnosis of BP. Mucosal lesions in BP are typically restricted to the oral mucosa and may be observed in up to 20% of patients. [6][7][8] The involvement of other mucosal surfaces is less established and based on anecdotal case reports. [9][10][11][12][13] To our knowledge, the frequency of nonoral mucosal involvement among patients with BP was not evaluated previously in the setting of cohort or large case series studies. The knowledge about the precise mucosal structures that may be affected in BP and of the distinct features of patients with BP who have mucosal involvement is very limited.The objectives of the current study were to evaluate the prevalence of general mucosal involvement in patients with BP and to characterize the subgroup of patients with mucosal lesions, using a large cohort of patients with welldefined and immunopathologically validated BP. IMPORTANCE The prevalence of mucosal involvement in bullous pemphigoid (BP) is inconsistent. Nonoral mucosal involvement was reported anecdotally in few patients with BP.OBJECTIVE To evaluate the prevalence of mucosal involvement in patients with BP, and to characterize the subgroup of patients with mucosal lesions. DESIGN, SETTING, AND PARTICIPANTSA retrospective cohort study was performed including 328 consecutive patients diagnosed with immunopathologically validated BP at a tertiary care referral center for autoimmune bullous diseases in northern Israel between January 1, 2000, and December 31, 2017. MAIN OUTCOME AND MEASURESThe study was conducted to estimate the prevalence and distribution of mucosal involvement among patients with BP. Patients with mucosal involvement were compared with the remaining BP patients regarding clinical and immunological features, laboratory analyses, and treatments. RESULTSThe study cohort included 139 (42.4%) male and 189 (57.6%) female patients, with a mean (SD) age of 78.0 (11.8) years at presentation. Fifty-six patients (17.1%) presented with mucosal lesions. The oral mucosa was the most frequently affected mucosal surface (n = 44; 13.7%), followed by the laryngeal (n = 16; 4.9%) and the genital (n = 10; 3.0%) mucosae. Among patients with oral lesions, the most involved oral structures were the buccal mucosa (n = 25; 55.6%) and the soft palate (n = 24; 53.3%). Compared with other patients with BP, patients with mucosal involvement were younger (71.8 [14.4] years vs 79.3 [10.8] years; P < .001), presented more frequently with extensive disease (55.4% vs 39.7%; P = .002), had less p...

More Severe Erosive Phenotype Despite Lower Circulating Autoantibody Levels in Dipeptidyl Peptidase-4 Inhibitor (DPP4i)-Associated Bullous Pemphigoid: A Retrospective Cohort Study

“…Unlike other studies reporting a male predominance among patients with DPP4i-associated BP [3,6,8,24], the sex distribution in our cohort was similar between the two study attributing a higher frequency of mucosal lesions in patients with DPP4i-associated BP [6,25], mucosal involvement among our DPP4i-associated patients was as common as among the remaining patients. The current study suggests that patients with DPP4i-associated BP had a more severe bullous component, as indicated by a higher erosion/blister BPDAI score.…”

Assessment of the Characteristics and Associated Factors of Infectious Complications in Bullous Pemphigoid

“…Moreover, we found that the ratio of patients with mucosal involvement of BP was significantly higher in the infected outpatients than in the uninfected outpatients with BP. Kridin and Bergman have shown that a majority of mucosal involvements of BP occur in the mouth and are related to extensive cutaneous disease, lower peripheral eosinophilia, and more aggressive treatment (20). In our study, the association between infectious complications and increased doses of corticosteroids was also significant, suggesting that the correlation between mucosal involvement of BP and infection is dependent on treatment.…”

Nivolumab-induced severe bullous pemphigoid in a patient with renal cancer: a case report and literature review

“…Here, we summarise a list of selected case reports of immunotherapy induced BP by literature review in Table 1. Most reported immunotherapy-induced BP involving either skin or mucous membrane only Table 1; combination of cutaneous BP and mucous membrane pemphigoid (MMP) is rare [4] . Cutaneous BP typically presents with pruritic blisters that arise on erythematous or urticarial plaques.…”