Pemphigus & Pemphigoid
- Bullous pemphigoid (BP) and dermatitis herpetiformis (DH) are autoimmune bullous skin diseases. DH has been described to evolve into BP and the two diseases can have overlapping clinical appearances and diagnostic findings, but the association between DH and BP has not previously been studied in a large population. To evaluate DH and celiac disease as risk factors for BP, we conducted a retrospective case-control study of patients with BP and matched controls with basal cell carcinoma diagnosed in Finland between 1997 and 2013.
- Neurologic patients have an increased risk for bullous pemphigoid (BP), in which autoantibodies target BP180, a cutaneous basement membrane protein also expressed in the brain. Here we show that 53.6% of sera from patients with multiple sclerosis (MS) (n = 56) had IgG reactivity against full-length BP180 in immunoblotting, while in BP180 non-collagenous 16A ELISA (n = 143), only 7.7% of MS samples studied were positive. Epitope mapping with 13 fusion proteins covering the entire BP180 polypeptide revealed that in MS and Alzheimer’s disease (AD) patients, IgG autoantibodies target regions located in the intracellular and mid-extracellular parts of BP180, but not the well-known BP epitopes located in the non-collagenous 16A domain and the distal part of extracellular domain.
- Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease (Schmidt and Zillikens, 2013). BP has become more common over the past two decades (Försti et al., 2014; Joly et al., 2012; Langan et al., 2008). However, the underlying causes of the increasing incidence of BP are poorly understood. Altogether, over 50 drugs have been reported to induce BP (Stavropoulos et al., 2014). The use of dipeptidyl peptidase-4 inhibitors (DPP-4i), a class of drug used for the treatment of diabetes, has recently been scrutinized as a risk factor for BP, both in case reports (see Supplementary Table S1 online) and in national pharmacovigilance database reports (Bene et al., 2016; García et al., 2016), but large population-based studies are lacking.
- Bullous pemphigoid (BP) is a subepidermal blistering skin disease, which has shown a strong association with neurological diseases in epidemiological studies. The BP autoantigens BP180 and BP230 are expressed in the cutaneous basement membrane and the central nervous system. Using BP180 and BP230 ELISA assays and immunoblotting against BP180, we analyzed the IgG reactivity in the sera of 115 patients with Alzheimer’s disease (AD) and 40 neurologically healthy controls. BP180 autoantibodies were found in 18% of patients with AD, whereas only 3% of controls had positive results (P = 0.019).