title: Fanconi's anaemia associated with multicentric Bowen's disease and decreased NK cytotoxicity.
abstract: We report a patient with Fanconi's anaemia and multiple lesions of vulvo-anal Bowen's disease. She was thrombocytopenic and lymphopenic, and NK-mediated cytotoxicity was undetectable. The vulvar lesions did not contain papillomavirus DNA. In vitro studies showed a possible benefit from acitretin treatment on bone marrow stem cells. However, low-dose acitretin given for 14 months did not prevent the development of an anal squamous carcinoma. Acitretin therapy was, however, associated with a sustained rise in the platelet count.
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Lebbé
authors.forename: L
authors.initials: L
authors.lastname: Pinquier
authors.forename: M
authors.initials: M
authors.lastname: Rybojad
authors.forename: C
authors.initials: C
authors.lastname: Chomienne
authors.forename: S
authors.initials: S
authors.lastname: Ochonisky
authors.forename: J M
authors.initials: JM
authors.lastname: Miclea
authors.forename: E
authors.initials: E
authors.lastname: Gluckman
authors.forename: P
authors.initials: P
authors.lastname: Morel
mesh_terms: D000328:Adult; D001005:Anus Neoplasms / Q000150:complications / Q000276:immunology; D001913:Bowen's Disease / Q000150:complications* / Q000276:immunology; D003602:Cytotoxicity, Immunologic / Q000502:physiology*; D005199:Fanconi Anemia / Q000150:complications* / Q000276:immunology; D005260:Female; D006801:Humans; D007694:Killer Cells, Natural / Q000502:physiology*; D012878:Skin Neoplasms / Q000150:complications* / Q000276:immunology; D014846:Vulvar Neoplasms / Q000150:complications / Q000276:immunology