Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease. It usually affects people older than 70 years of age. The two main autoantigens are BP180 and BP230, both of which are components of hemidesmosomes. Immunoglobulin (Ig)G and IgE autoantibodies to BP180 detected by the enzyme-linked immunoassay (ELISA) show close associations with the activity and severity of BP. In addition, inflammatory cells (eosinophils, neutrophils and mast cells) and cytokines (e.g. interleukins and CC chemokine ligands) play an important part in the pathogenesis, activity and severity of BP. We summarized the potential contribution of each factor postulated to be associated with the activity and severity of BP, and provide guidance for clinicians to pay timely and close attention to such parameters. This review may also promote the development of novel therapies for BP. KEY MESSAGES Bullous Pemphigoid Disease Area Index (BPDAI) is a scoring system which can reflect the extent of clinical involvement of BP patients. The titres of IgE autoantibodies and IgG autoantibodies against the NC16A domain of BP180 are closely correlated with the activity and severity of BP. Many inflammatory cells and molecules, such as eosinophils and interleukins, can also reflect the activity and severity of BP.
Bullous pemphigoid (BP) is a senile autoimmune blistering disease with autoantibodies against the basement membrane. Less than 20 cases of localized BP in young adults have been reported and the understanding of localized BP is very limited. An unusual location of localized BP is here described. A 30-year-old woman presented with a 4-month history of itchy erythema on her trunk. The lesion, welldemarcated erythema and maculopapules in bra's shape, had been misdiagnosed as contact dermatitis.Laboratory finding was notable for serum autoantibodies to BP antigen 180 (BP180). Histopathological examination revealed a subepidermal blister with eosinophils and neutrophils infiltration. Salt-split indirect immunofluorescence revealed linear deposition of IgG at the dermoepidermal junction. After treating her with minocycline 200 mg and nicotinamide 1,500 mg per day, all lesions resolved within 1 month. Localized BP is usually misdiagnosed. It starts from various triggers and has a more benign disease course. It should be emphasized that a long-term follow-up of patients with localized BP may be important for management of the chronic disease, given a relatively high risk of developing generalized BP.
BackgroundBullous pemphigoid (BP) is a senile chronic autoimmune bullous skin disease with a high relapse rate, which significantly impairs patients’ quality of life and contributes to disease mortality. This observational case-control study explores the gene polymorphisms of cytokines and their clinical significance in Chinese patients with BP.MethodsIL-1α (rs1800587), IL-1β (rs16944, rs1143627, rs1143634), IL-4 (rs2243250), IL-6 (rs1800795), IL-10 (rs1800896, rs1800871, rs1800872), IL-13 (rs1800925, rs20541), TNF-α (rs1799964, rs1800630, rs1799724, rs361525), IFN-γ (rs1799964, rs1800630, rs361525, rs1800629, rs4248160, rs1800750), and TGF-β1 (rs2317130, rs1800469, rs4803457) genes were genotyped in the healthy controls and BP patients, respectively. Expression of these cytokines in serum was measured. Medical profiles of patients, including baseline characteristics and prognosis, were statistically analyzed.ResultsWe found that IL-1 β and IL-13 concentrations were higher in the BP patients’ sera compared to those in the controls. For IL-13, significant differences were found in the nucleotide ratio/genotype/haploid frequency/haplotype, respectively. IL-13 (rs20541, rs1800925) is related to gender, and the IL-13 genotype was significantly associated with recurrence.ConclusionsBP is associated with IL-13 gene polymorphism and IL-13 concentration is elevated in blood circulation in patients with BP. Our results support that IL-13 is relevant in the pathogenesis of BP, suggesting that IL-13 could potentially represent a promising target for BP therapy and a prognostic marker.
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