ABSTRACT
Elastic pseudoxanthoma is a rare disease with autosomal recessive inheritance, also known as Grönblad-Strandberg syndrome, characterized by pathological mineralization of the elastic fibers in the connective tissue, affecting principally the dermis of skin, media, and intima of blood vessels and Bruch's membrane of the eye. The genetic defect of the disorder is located on chromosome 16p13.1 and disease is caused by the lack of functional ABCC6 protein, which in turn causes extracellular accumulation and deposition of calcium and other minerals in the elastic tissue. In this article we present two cases of this rare disease. We emphasize, in the diagnostic criteria, the importance of its early diagnosis and the current therapeutic approaches.
ABSTRACT
La terapia fotodinámica se basa en la interacción de un agente fotosensibilizante que cuando es estimulado por la longitud de onda apropiada inducela formación de radicales libres que causan necrosis celular. Es una de las técnicas más recientes empleada en el tratamiento de algunos tipos de cáncer,aunque actualmente ya se ha demostrado su eficacia en otras enfermedades inflamatorias. En dermatología su principal indicación es el carcinomabasocelular, pero también se ha empleado en carcinoma escamoso in situ enfermedad de Bowen y más recientemente en acné, rosácea, varices,fotorejuvenecimiento, liquen, liquen escleroso y atrófico y leshmaniasis, entre otras dermatosis1
Photodynamic therapy is based in the interaction of a photoactive agent that when is properly stimulated by a specific wavelength it induces free radi-cals that lead to cellular necrosis. This is one of the newest treatments for some types of cancer and other inflammatory diseases. In dermatology, pho-todynamic therapy is used for malignant neoplasias such as basal cell carcinoma, Bowen disease in situ squamous cell carcinoma and morerecently it has been applied to inflammatory skin diseases such as acne, rosacea, lichen plannus, lichen sclerosus et atrophicus, and other conditions leishmaniasis, photodamaged skin, telagiectasias1