RESUMEN
Brooke-Spiegler Syndrome [BSS] is a rare autosomal dominant disease characterized by the development of multiple adnexal cutaneous tumors such as cylindromas, trichoepitheliomas and spiradenomas. A 49 years old female patient presented with a history of gradual appearance of multiple nodules situated predominantly on the scalp and face. Pathologic examination of lesions revealed cylindroma with foci of spiradenoma. Brooke-Spiegler Syndrome [BSS] caused by various mutations of the cylindromatosis gene [CYLD], a tumor suppressor gene resulting in multiple cylindromas and trichoepitheliomas. Malignant transformation may develop within solitary cylindromas, but more common in multiple variant and requires careful followup surveillance
Asunto(s)
Humanos , Femenino , Neoplasias Cutáneas , Genes Supresores de Tumor , Carcinoma Adenoide Quístico , MutaciónRESUMEN
We assessed the effectiveness of photodynamic therapy in the treatment of cutaneous leishmaniasis in 5 patients. Delta-aminolevulinic acid in a water- in-oil emulsion was applied to the lesions and irradiation was performed. The treatment was repeated once a week for a month. Each time, direct smears of the lesions were prepared and cultured in NNN media. In direct staining, smears showed no amastigotes after 1 or 2 sessions. Healing and cosmetic outcome after photodynamic therapy was excellent. Only mild local inflammatory reaction was noted with no scarring and 4 months after the last treatment session, there were no clinical signs of recurrence