Manifestaciones clínicas de la esclerosis de Monckeberg. Reporte de caso y revisión de la literatura / Clinical manifestations of Monckeberg's sclerosis. Report of case and literature review

Resumen La esclerosis de Monckeberg es una enfermedad de baja prevalencia y causa desconocida, en la cual arterias de mediano y pequeño calibre sufren calcificación de la capa media generándose una reducción de la luz. Suele manifestarse por la aparición de úlceras de tipo arterial en la piel de extremidades superiores e inferiores. Su diagnóstico requiere de estudios histopatológicos, con diagnósticos diferenciales como la poliarteritis nodosa y la arteritis de células gigantes. No existen guías de tratamiento, se han propuesto quelantes de calcio como el tiosulfato de sodio, pero los pocos estudios disponibles no han logrado demostrar resultados significativos.

Abstract Monckeberg's sclerosis is a rare and low prevalence disease of unknown cause in which small and medium size arteries suffer calcification of the middle layer, leading to a reduction in calibre. Clinically, the disease manifests by the appearance of arterial type ulcers on the skin of upper and lower extremities. Its diagnosis is difficult, and requires histopathological studies. Some rheumatic conditions, such as polyarteritis nodosa and giant cell arteritis may mimic this process. Treatment of the disease is not well established. Although the use of calcium chelators such as sodium thiosulfate has been proposed, the few available studies have failed to show significant results.

Radiographic manifestations of Mönckeberg arteriosclerosis in the head and neck region

Mönckeberg sclerosis is a disease of unknown etiology, characterized by dystrophic calcification within the arterial tunica media of the lower extremities leading to reduced arterial compliance. Medial calcinosis does not obstruct the lumina of the arteries, and therefore does not lead to symptoms or signs of limb or organ ischemia. Mönckeberg sclerosis most commonly occurs in aged and diabetic individuals and in patients on dialysis. Mönckeberg arteriosclerosis is frequently observed in the visceral arteries, and it can occur in the head and neck region as well. This report describes a remarkable case of Mönckeberg arteriosclerosis in the head and neck region as detected on dental imaging studies. To the best of our knowledge, this is the first case that has been reported in which this condition presented in the facial vasculature. The aim of this report was to define the radiographic characteristics of Mönckeberg arteriosclerosis in an effort to assist health care providers in diagnosing and managing this condition.