Fanconi's anaemia associated with multicentric Bowen's disease and decreased NK cytotoxicity.
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.
Lebbé
C
C
Service de Dermatologie, Hôpital Saint-Louis, Paris, France.
Pinquier
L
L
Rybojad
M
M
Chomienne
C
C
Ochonisky
S
S
Miclea
J M
JM
Gluckman
E
E
Morel
P
P
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