Résumé
Xeroderma pigmentosum is a group of dermatoses characterized by autosomal recessive inheritance, photosensitivity, photophobia, early onset freckles, and subsequent cutaneous neoplastic changes on sun-exposed areas. It is due to enzymatic defects that render the cells unable to repair UV-induced DNA damages resulting in various clinical phenotypes. A 66-year-old female patient who has been diagnosed as xeroderma pigmentosum twenty years ago developed a recurrent basal cell carcinoma on the right nasal side-wall, resulting in a large defect that comprises multiple aesthetic units, after a Mohs micrographic surgery. A combination of dorsal nasal and cheek advancement flaps with full thickness skin graft was a useful method for the reconstruction of the large nasal defect.
Sujets)
Sujet âgé , Femelle , Humains , Carcinome basocellulaire , Joue , Altération de l'ADN , Mélanose , Chirurgie de Mohs , Phénotype , Photophobie , Peau , Maladies de la peau , Transplants , Testaments , Xeroderma pigmentosumRésumé
Xeroderma pigmentosum is a group of dermatoses characterized by autosomal recessive inheritance, photosensitivity, photophobia, early onset freckles, and subsequent cutaneous neoplastic changes on sun-exposed areas. It is due to enzymatic defects that render the cells unable to repair UV-induced DNA damages resulting in various clinical phenotypes. A 66-year-old female patient who has been diagnosed as xeroderma pigmentosum twenty years ago developed a recurrent basal cell carcinoma on the right nasal side-wall, resulting in a large defect that comprises multiple aesthetic units, after a Mohs micrographic surgery. A combination of dorsal nasal and cheek advancement flaps with full thickness skin graft was a useful method for the reconstruction of the large nasal defect.