Long-term follow-up of lipoid proteinosis laryngeal manifestations

Abstract 

The disease course, management, and outcome of 5 cases of lipoid proteinosis followed up in our clinic for 10–16 years are presented. Two patients were males. Diagnosis was made at an age of <1 to 11 years but was missed originally in 3 patients due to unfamiliarity with the disease. Three patients underwent microlaryngoscopical excision of laryngeal lesions, 1 with a CO₂ laser and 2 with conventional microlaryngoscopy. Laryngeal symptoms as well as the other clinical manifestations of the disease improve as the child reaches puberty. Laryngeal surgical intervention should be reserved only for cases with compromised airways.

Keywords: Lipoid proteinosis, Urbach–Wiethe disease, Hoarseness, Carbon dioxide laser, Microlaryngeal surgery