RESUMO
McCune-Albright syndrome (MAS) is a rare, heterogenous, clinical condition caused by a rare genetic mutation. The disorder is more common in females and is characterized by a triad of cutaneous, bone and endocrine abnormalities. We describe a male patient with MAS having multiple café-au-lait macules and deforming polyostotic fibrous dysplasia involving long bones of the limbs, skull and spine without any endocrine abnormality. Severe bone deformities involving almost all bones have not been described previously and this prompted us to present the current case.
Assuntos
Manchas Café com Leite/diagnóstico por imagem , Displasia Fibrosa Poliostótica/diagnóstico por imagem , Crânio/diagnóstico por imagem , Adolescente , Braço/diagnóstico por imagem , Manchas Café com Leite/complicações , Manchas Café com Leite/diagnóstico , Displasia Fibrosa Poliostótica/complicações , Displasia Fibrosa Poliostótica/diagnóstico , Humanos , Perna (Membro)/diagnóstico por imagem , Masculino , RadiografiaRESUMO
Bilateral lower limb angiography was performed on 58 patients with leprosy and compared with bilateral lower limb angiography performed on 63 non-leprosy patients. The vessels in leprosy were narrow and constricted. Decreased blood flow through arteries in the distal third of the lower limb was present. Abnormal AV fistula formation in the thigh and calf muscles and in trophic ulcers of the feet was also seen. Deep inflammatory granulomatous reaction surrounding the arteries as a result of leprosy is a possible explanation for these angiographic findings and is felt to be related to trophic ulceration. These findings were specific for leprosy, precede clinical manifestations, and are helpful in management.