Immunopathology of Cicatricial Pemphigoid: Studies of Complement Deposition

Abstract

Immunopathologic investigations were conducted on the sera and oral mucosal tissue specimens of 23 patients with cicatricial pemphigoid. A linear, continuous basement membrane zone pattern was noted in 83% of oral mucosal biopsy specimens studied. This pattern is indistinguishable from the pattern noted in immunofluorescence studies of bullous pemphigoid, herpes gestationis, and some cases of desquamative gingivitis. Complement studies provided data supportive of classical pathway activation in cicatricial pemphigoid tissue. Deposition of IgA with Factor B, properdin, and C3 raised the possibility of alternative pathway activation, a question requiring further study. Circulating antibasement membrane zone antibodies were noted in the sera of two patients with cicatricial pemphigoid.

Localized Chronic Pemphigoid

“…Besides the identical histopathology and the linear deposition of Ig in the BM region, the occurrence of complement is an important characteristic which LCP shares with CP and BP. As has been demonstrated in CP [22] and BP [19], the presence of CU 1 and C4 in our case suggests that the complement system has been activated via the classical pathway, pointing to an antigen-antibody reaction in the BM region. Concomitant activation via the alternate pathway could not be demonstrated.…”

“…The latter two characteristics are important to distinguish LCP from the localized form of BP, which usually runs a limited course [21], LCP and CP share important features: the chronic, recurrent course, the scarring, the low incidence of circulating antibodies, the usually negative IF findings in the uninvolved skin, and perhaps the concomitant occurrence of IgG and IgM in the BM region. Another common feature may be the presence of fibrinogen in the region of the BM, observed by us and others [18] in LCP, since its presence was also found in 15 of 19 cases of CP [22]. In our experience the presence of fibrinogen is rare in BP (in 2 of more than 40 cases) [unpubl.…”

“…However, in contrast to BP, IgA has been found nearly as frequently as IgG [10,13,22] and also IgM has been demonstrated in a relatively high number of the cases [22], The uninvolved skin was only positive in a low number of cases [2,24]. The incidence of circulating IgG and IgA anti-BM antibodies is low in CP, but their occurrence had been reported [5,6,26],…”

Use of intravenous immunoglobulin therapy in autoimmune blistering diseases

“…The biopsies reveal a subepithelial blister with an inflammatory infiltrate that can be mixed predominantly neutrophilic or eosinophilic [91]. DIF of perilesional tissues reveals linear deposition of IgG and complement at the epithelial at the BMZ [92][93][94]. IIF studies are often negative and very low titers of circulating anti-BMZ antibodies may be detected [94,95].…”

High Frequency of HLA-DQB1*0301 Allele in Patients with Pure Ocular Cicatricial Pemphigoid

“…The term cicatricial pemphigoid can include a heterogeneous group of scarring, immune-mediated. subepithelial blistering diseases involving any and all mucous membranes, with or without skin involvement f 1-4], Under such a broad categorization, most investiga tors reported the frequent presence of in vivo basement membrane zone (BMZ) immune deposits in the patients' skin or mucous membranes [2-4], whereas circulating anti-BMZ antibodies were reported to be infrequently detected [2][3][4][5][6][7][8]. Whereas some investigators reported the presence of specific epidermal antigens recognized by patients' sera [5][6][7], other investigators did not detect any specific anti gen ¡8], In an effort to better classify this broad and hetero geneous group of diseases, we have undertaken a compre hensive 6-year study [9] on 87 patients who fall in this het erogeneous group of diseases named cicatricial pemphi goid.…”