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Case of diffuse panbronchiolitis developed in a patient with epidermodysplasia verruciformis

Dear Editor, Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by susceptibility to infection by human papillomavirus (HPV) strains and subsequent risk of malignant transformation. These HPV types are commonly referred to as EV-associated HPV types and include HPV-5, -8, -9, -12, -14, -15, -17, -19–25, -36–38 and -49. Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease, principally affecting the respiratory bronchioles. Both disorders are related to immunodeficiency conditions; however, there has been no report on the association between these two diseases. Here, we report a case of EV accompanying DPB. A 36-year-old Japanese man was referred to our department for evaluation of his skin lesions that had begun in his adolescence. He had had DPB as an underlying disease since his early childhood. He had no family history of consanguineous marriage or other disease. Physical examination showed multiple round lesions such as red plaques with slight scales or vitiligo of 1–2 cm in diameter distributed on the trunk (Fig. 1a). He also presented verruca-like papillomatous lesions on his hands and feet. Laboratory tests showed no abnormal findings, including the CD4/8 T-cell ratio and T-cell proliferation ability. A skin biopsy from his right shoulder revealed epidermal acanthosis and parakeratosis with a characteristic steel blue cytoplasm within keratinocytes (Fig 1b). Polymerase chain reaction analysis using two different primers, CP65/70 and MY09/MY11, was performed to determine HPV type. The results indicated that HPV-22 was present in the lesion. Based on these findings, we diagnosed the patient as classic EV. The exact pathogenesis of EV is not fully known, although two inactivating mutations of the EVER1/TMC6 and EVER2/ TMC8 genes have been identified in familial forms. Data from a recently published gene atlas of the human protein-encoding transcriptome indicate that both EVER1 and EVER2 are highly expressed on T cells, B cells and natural killer (NK) cells. Specific abnormalities in NK cell cytotoxicity and T-cell activation have been reported in EV patients. Although the etiology of DBP remains unknown, it has been reported that human leukocyte antigen is associated with the disease, and that DBP is association with human T-cell leukemia virus type 1 infection. These results suggest that immunodeficiency, such as T-cell dysfunction, is related to the pathogenesis of DBP. Therefore, both disorders may share some underlying mechanisms in common, such as immunodeficiency due to T-cell dysfunction, but these two disorders are so rare that no cases of EV accompanied with DBP have been reported. Although the clinical manifestation suggested that T-cell functions may be attenuated, T-cell proliferation capacity in vitro and CD4/8 differentiation among peripheral blood mononuclear cells were intact in our case. Although it is limited to a single case and we still do not know the underlying common pathogenesis, our case may improve our understanding of the pathogenesis of EV and DBP.