Antiepiligrin (laminin 5) cicatricial pemphigoid associated with an underlying gastric carcinoma producing laminin 5

Abstract

Although bullous pemphigoid and cicatricial pemphigoid are sometimes associated with malignancy, it remains uncertain whether such an association is pathogenetically related or just a coincidence attributable to the advanced age of the patients. We report a 61-year-old patient with antiepiligrin (laminin 5) cicatricial pemphigoid (AeCP) associated with an advanced gastric carcinoma. The gastric carcinoma cells in this patient were shown to produce laminin 5 by immunofluorescence microscopy, and the patient's serum contained autoantibodies directed against laminin 5 on immunoprecipitation. Furthermore, the blistering symptoms and the titre of antibasement membrane zone antibodies coordinately changed with the resection and subsequent relapse of the gastric cancer. These observations suggest that the gastric carcinoma producing laminin 5 may have induced the production of autoantibodies to this laminin, which were pathogenic to the skin and mucous membranes in this patient. This report demonstrates a link between this autoimmune subepithelial blistering disease and malignancy. It is of interest and potential great importance to examine other cases of AeCP for such a potential association.

A Case of Antiepiligrin Cicatricial Pemphigoid Successfully Treated by Plasmapheresis

“…More than 20 cases of antiepiligrin CP have been reported in the literature [7], and the present case is the fourth of its kind in Japan [8, 9, 10]. In our case, the extraocular lesions improved in correlation with a fall in the antibody titer following treatment with a combination of systemic steroids and immunosuppressive agents.…”

Histopathology of anti-laminin 5 mucous membrane pemphigoid

“…15 The subsequent 16 case reports, containing any kind of histopathological data, describe findings of 12 skin and 2 oral mucosa biopsies. [16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] While subepithelial split formation was present in all samples, no detailed information on the density and composition of the inflammatory infiltrate was provided in a majority of cases. In 6 cases the infiltrate was described as absent or mild 18,19,24,26,29,30 and in one case as moderately dense.…”

Antiepiligrin Cicatricial Pemphigoid with Autoantibodies to the Beta Subunit of Laminin 5 and Associated with Severe Laryngeal Involvement Necessitating Tracheostomy

“…Some patients have antibodies to laminin β3, and others show reactivity to the α3- and γ2-subunits [8, 9, 10]or to the β3- and γ2-subunits [11]. In addition, an association between antiepiligrin CP and neoplasm has recently been reported in patients with gastric carcinoma [12, 13], lung carcinoma [14]and endometrial carcinoma [15]. Our patient’s serum was reactive to the laminin β3-subunit on immunoblotting and there was no evidence of associated neoplasm.…”