Abstract
Bullous pemphigoid (BP) is an autoimmune bullous skin disease characterized by anti-BP180 and anti-BP230 autoantibodies (AAbs). Mucous membrane involvement is an uncommon clinical feature of BP which may evoke epidermolysis bullosa acquisita, another skin autoimmune disease characterized by anti-type VII collagen AAbs. We therefore evaluated the presence of anti-type VII collagen AAbs in the serum of BP patients with and without mucosal lesions at time of diagnosis and under therapy. Anti-BP180, anti-BP230, and anti-type VII collagen AAbs were measured by ELISA in the serum of unselected patients fulfilling clinical and histo/immunopathological BP criteria at baseline (n = 71) and at time of relapse (n = 24). At baseline, anti-type VII collagen AAbs were detected in 2 out of 24 patients with BP presenting with mucosal involvement, but not in patients without mucosal lesions (n = 47). At the time of relapse, 10 out of 24 BP patients either displayed a significant induction or increase of concentrations of anti-type VII collagen AAbs (P < 0.01), independently of mucosal involvement. Those 10 relapsing BP patients were also characterized by a sustained high concentration of anti-BP180 AAb, whereas the serum anti-BP230 AAb concentrations did not vary in BP patients with relapse according to the presence of anti-type VII collagen AAbs. Thus, our study showed that anti-type VII collagen along with anti-BP180 AAbs detection stratified BP patients at time of relapse, illustrating a still dysregulated immune response that could reflect a potential epitope spreading mechanism in those BP patients.
Highlights
Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering skin disease that preferentially affects the elderly with various clinical manifestations
We found that anti-BP OutcomeBP antigen 180 (BP180) AAb were indifferently present in BP patients with or without mucosal lesions, with similar titers (Table 1), while the percentage of patients with positive anti-BP antigen 230 (BP230) antibodies titer was lower in patients with mucosal involvement compared with patients without mucosal involvement (29 and 55%, respectively; P < 0.05)
The present study demonstrated that investigating both antiBP180 and anti-type VII collagen antibodies serum concentrations was useful to exclude epidermolysis bullosa acquisita (EBA) diagnosis in BP patients with mucosal involvement at time of diagnosis
Summary
Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering skin disease that preferentially affects the elderly with various clinical manifestations. BP patients typically present at diagnosis vesicles and tense clear blisters, which mainly occur on erythematous skin, together with erythematous or urticarial papules and plaques [1,2,3,4]. When presenting with mucosal involvement, BP may suggest the BP-like, inflammatory form of epidermolysis bullosa acquisita (EBA), another skin autoimmune disease characterized by AAb directed against type VII collagen, a protein of the basement membrane zone beneath the stratified squamous epithelia [8, 18,19,20,21,22]. No study has investigated yet the presence of serum anti-type VII collagen AAb at time of diagnosis of BP patients with mucosal involvement and whether these AAb are associated with BP outcome
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