Most reviews (= 42) describe the usage of corticosteroids (mainly mouth prednisolone) in a variety of doses, from suprisingly low dose in a few Japanese situations up to 2 mg/kg bodyweight

Most reviews (= 42) describe the usage of corticosteroids (mainly mouth prednisolone) in a variety of doses, from suprisingly low dose in a few Japanese situations up to 2 mg/kg bodyweight. strategies for the condition itself, but could also shed brand-new light in the pathophysiology of more prevalent and treatment-refractory autoimmune blistering illnesses. = 0.0032), MMP and LPP (= 1.07 10?13) and LPP and PG (= 3.08 10?43) were actually highly significant. In addition, BP and PG (= 3.82 10?13) and MMP and PG (= 1.95 10?24) had significantly different binding patterns, while MMP and BP did not. Epidemiology The exact prevalence of LPP is unknown. Only 4 cases of LPP were identified in a cohort of 68 patients with blistering diseases from Kuwait; equivalent to an incidence of 0.3/1,000,000 inhabitants (16). A study from India reported 3 patients with LPP in a series of 268 cases with autoimmune blistering dermatoses (17). In contrast, epidemiological studies in patients with blistering dermatoses, based in France, Germany, Greece, Serbia, and Singapore, with patient numbers ranging NK314 from 41 to 1 1,161, did not identify any cases of LPP (18C23). Based on ICD10 classification data from health insurance providers in Germany, the reported prevalence of L12.8 (other pemphigoid diseases) was 4.7 per million patients and 259 per million patients for BP (L12.0) (7). Unfortunately, the LPP ICD10 code L43.1, was not specifically evaluated. However, the epidemiological data analysis based upon ICD10 codes is complicated by the fact that the ICD10 code L43.1 is shared between LPP and bullous LP. Nevertheless, based on the available data the prevalence may be estimated at about 1 per 1,000,000 patients. The sex ratio NK314 (male/female) is described to be roughly 0.8/1 in adults and 3.3/1 in children and adolescents (8), failing to support a specific predilection according to sex. The mean age of onset is approximately 46 years (range between 4 and 85), which is well below the typical age of Hoxd10 onset of BP (7). Interestingly, it is not exceptionally rare for LPP to affect children and adolescents. Indeed, in a case report collection with 78 patients, 13 (~16%) were children or adolescents (8). Etiopathogenesis LPP is characterized by autoantibodies against type XVII collagen (COL17, BPAG2), a structural protein that resides in hemidesmosomes at the dermal-epidermal junction (4, 24, 25). Similarly to BP, autoantibodies in LPP may also bind to the 230 kDa BPAG1 (3). In most cases, the COL17-specific autoantibodies in LPP react with the membrane-proximal NC16A subdomain (amino acid residues 490C565 of UniProt entry “type”:”entrez-protein”,”attrs”:”text”:”Q9UMD9″,”term_id”:”146345399″,”term_text”:”Q9UMD9″Q9UMD9) (4, 24). In addition, the C-terminal portion of COL17 and desmoglein 1 have been identified as epitopes and antigens, respectively, in LPP (26). Other autoantibodies against unidentified antigens with a molecular weight of 130 kDa (27) and 200 kDa (28) have also been described. The reported variability in autoantigen specificity may result in NK314 clinical variants of LPP which appear similar to BP, with autoantibodies against NC16A (24, 29), and NK314 MMP with mucosal lesions and autoantibodies against the C-terminal portion of COL17 (26, 30). In fact, COL17 is a common autoantigen in a variety of autoimmune blistering dermatoses (31, 32), including LPP, BP, linear IgA dermatosis (33, 34), PG, MMP and paraneoplastic NK314 pemphigus (35). Autoantibodies against COL17 have been demonstrated to induce inflammation and blistering due to the effector functions of the Fc portion (36C38). Moreover, a deposition of complement factor C3 at the dermal-epidermal junction found in skin biopsies of LPP indicates an involvement of complement in the pathogenesis. In case of BP and epidermolysis bullosa acquisita, a similar subepidermal blistering disease, the activation of the complement system has been described as a crucial event in the pathogenesis (39, 40). However, a growing evidence suggests that both complement-dependent and complement-independent mechanisms may both be relevant and effective in subepidermal blistering dermatoses (41C44). The amount of complement-activating IgG1 and non-activating IgG4 autoantibodies (45) is variable between patients. Cases with only IgG4 autoantibodies and without.