Pemphigoid gestationis: new insights into the pathogenesis lead to novel diagnostic tools

Pemphigoid gestationis autoantigen, transmembrane collagen XVII, promotes the migration of cytotrophoblastic cells of placenta and is a structural component of fetal membranes

“…Although the exact pathomechanism of PG is largely unknown, the autoimmune response in PG has been proposed to be initiated by either the presentation of abnormal MCH molecules of placental collagen XVII or an allogenic reaction of placental stromal cells to collagen XVII presented in the context of paternal MCH molecules (Shimanovich et al, 2002). In contrast to previous studies, we showed here that collagen XVII is already expressed during the first trimester of pregnancy.…”

“…Thus, the onset of the clinical symptoms of PG occurs only after the expression of autoantigen in placenta. If paternity remains unchanged in the patient's subsequent pregnancies, PG usually recurs earlier in the course of pregnancy and the symptoms last longer (Shimanovich et al, 2002), as was the case in the second PG pregnancy of our patient 2. Based on our findings, the earlier onset of PG in subsequent pregnancies could thus be explained by the presence of autoantigen in placenta during the first trimester already.…”

“…The clinical presentation of PG is polymorphic with intense pruritus and papules or bullous skin lesions typically in the periumbilical area (Shornick et al, 1983;Holmes and Black, 1983;Castro et al, 2006). The major antigen recognized by PG Available online at www.sciencedirect.com Matrix Biology 27 (2008) 190 -200 www.elsevier.com/locate/matbio autoantibodies is collagen XVII (BP180) (Lin et al, 2001;Shimanovich et al, 2002;Di Zenzo et al, 2007), which also acts as a major autoantigen in other diseases of the pemphigoid group, i.e. bullous pemphigoid, cicatricial pemphigoid and linear IgA dermatosis (see Van den Bergh andGiudice, 2003, Mihai andSitaru, 2007).…”

Clinical experience in pemphigoid gestationis: Report of 10 cases

“…Confirmation of the diagnosis depends on additional laboratory studies. [12][13][14][15] DIF studies were positive in 7 of 7 patients in our series. Specifically, strong continuous linear deposition along the BMZ with C3 was seen in all patients.…”

“…4,7,9,12 However, the HG factor was not as sensitive a marker for PG as previously reported. 2,[13][14][15] ELISA for detection of anti-BP180 antibodies in the serum of the 1 patient tested was positive. ELISA may be a useful technique to monitor autoantibody serum levels of anti-BP180.…”