Abstract

Lipoid proteinosis (LP) is a rare disease. It may affect the skin, oral mucosa, pharynx, larynx and all visceral organs. In this article, we describe a 32-year-old female patient who applied with complaint of white swelling in mouth, limiting the movement of lip, and hoarseness. Papule-like itchy rashes particularly around the hands, elbows, and lips were observed in physical examination and bilateral blepharosis in eye examination. In videolaryngostroboscopy, thickened vocal cords and yellow papule formations covering the entire supraglottic region and oral mucosa drew attention. In punch biopsy samples taken from larynx and sublingual regions, hyperkeratosis-patterned multilayered epithelial and subepithelial amorphous hyaline material deposition was observed. In histochemical examination, positive staining with periodic acid-Schiff in basal membrane and negative staining with Congo red were obtained. We established a diagnosis of LP according to typical vocal cord involvement and histopathologic, genetic and clinical findings.