Urbach

Urbach is a German-language surname and a toponym. It may refer to several places in German-speaking countries, and to individuals who bear the name. The toponym often denotes a location associated with a hill or village, though precise etymology varies by locale.

Urbach–Wiethe disease is a rare genetic disorder also called lipoid proteinosis. It results from mutations in the ECM1 gene and is inherited in an autosomal recessive pattern. The condition is characterized by deposition of hyaline material in skin and mucous membranes, leading to thickened skin, hoarseness, and beaded eyelids, among other symptoms. Neurologically, individuals may show bilateral calcifications in the temporal lobes, particularly the amygdala, which can affect emotion processing and social behavior. Diagnosis is based on clinical features, histology of skin lesions, and genetic testing. There is no cure; treatment is supportive and focuses on symptom management.