Epidermodysplasia verruciformis in a setting of common variable immunodeficiency.
A 14-year-old native American female with common variable immunodeficiency was admitted for bone marrow transplantation. Preoperative evaluation showed a generalized lichenoid papular eruption present for several years. Light microscopy revealed expansion of the epidermis by atypical keratinocytes; electron microscopy showed intranuclear papillomavirus inclusions within the granular keratinocytes; DNA hybridization revealed a type 5-related human papilloma virus homology. Four days after bone marrow transplantation the lichenoid papules blackened and began to disappear. Within 30 days after bone marrow transplantation the distribution and appearance of the papules was similar to that of the pretransplantation evaluation. One year after transplantation the patient showed evidence of a successful T lymphocyte graft. No transformation to squamous cell carcinoma had occurred. Epidermodysplasia verruciformis has been associated with deficient cell-mediated immunity, the varying severity of which does not predict the tendency to neoplasm formation (2, 5). Several distinct human papillomavirus genomes have been recovered with DNA hybridization techniques in these patients. It is hoped that the bone marrow transplantation might be associated with diminished transformation to squamous cell carcinoma.
D000293:Adolescent; D000818:Animals; D016026:Bone Marrow Transplantation; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications; D007070:Immunoglobulin A; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D027383:Papillomaviridae / Q000276:immunology; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000150:complications / Q000276:immunology* / Q000473:pathology; D014412:Tumor Virus Infections / Q000209:etiology / Q000276:immunology* / Q000473:pathology / Q000628:therapy
D007070:Immunoglobulin A