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.

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Association with Epstein-Barr virus (EBV) infection has been noted in various types of cutaneous lymphoproliferative disorders. We report a 57-year-old Japanese woman with T-cell lymphoma mimicking dermatomyositis that was associated with chronic active EBV infection. She presented with low-grade fever, bilateral erythematous swellings on the eyelids, and necrotic papules on the face. Serum creatine kinase levels were elevated and a diffuse reticular shadow was detected in both lung fields. The infiltrate of atypical lymphocytes found in skin and muscle, which contained EBV-encoded small nuclear RNA-1 and EBV, was also detected in the CD4+ peripheral blood cells. Treatment with prednisolone resolved her lesions with no relapse for 3 years, after which there was a recurrence in her left lung. Combination chemotherapy was not effective against the lung lesion and she died with multiple organ failure 2 months after the recurrence.

Platelet-derived growth factor (PDGF) is known to stimulate the proliferation of fibroblasts, although the role of PDGF and its receptors in the development of fibrohistiocytic tumors has not been clarified. In this study, we investigated this role by immunohistochemically staining PDGF and PDGF beta-receptors in paraffin-embedded dermatofibroma (DF), dermatofibrosarcoma protuberans (DFSP) and malignant fibrous histiocytoma (MFH) tissue. We also immunohistochemically investigated the relationship between PDGF beta-receptors and CD34, which is a known immunohistochemical marker for DFSP. Immunohistochemical studies using anti-PDGF-AA or BB antibodies showed that PDGF-AA and BB was found in 20 to 40% of the tumor cells in DF, DFSP, and MFH. No definite relationship for each tumor type was found. The expression of PDGF beta-receptors in DFSP and that of MFH tissue was significantly greater in comparison to DF and scar tissue. The expression of CD34 and PDGF beta-receptors in DFSP was observed in identical areas. These findings suggest that autocrine or paracrine growth stimulation, through PDGF beta-receptors, is related to the tumorous proliferation of fibrohistiocytic tumors, and the expression of PDGF beta-receptors might play a role in the proliferation of CD34 positive tumor cells.

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