Abstract

Lipoid proteinosis or Urbach-Wiethe disease is a genodermatosis of genetic origin with an autosomal recessive inheritance pattern, without sex predilection. It is characterized by varying degrees of intercellular hyaline material deposits in the skin, mucous membranes, and internal organs, leading to infiltration and thickening of the affected organs. The course is slow and benign. Two clinical cases are presented: a 7-year-old schoolboy, who
presented with dry skin and dysphonia since the age of 2, and a 13-year-old adolescent with similar symptoms.
Clinical and histopathological diagnoses were established. Treatment was initiated with oral pentoxifylline at
a dose of 10 mg per kilogram of body weight per day, resulting in significant improvement of the lesions. The
efficacy and safety of pentoxifylline as adjunctive therapy in pediatric patients diagnosed with lipoid proteinosis
are demonstrated.