皮膚粘膜ヒアリノーシスの1症例

抄録

Otorhinolaryngologists in Japan appear to be unfamiliar with hyalinosis cutis et mucosae, because this disease has not been reported in the otorhinolaryngological literature of Japan. This paper presents a case of hyalinosis cutis et mucosae with laryngeal involvements.
A 59-year-old man was referred to our clinic, complaining of hoarseness and multiple papular lesions of the mucosa of the pharynx and larynx and the skin of the face and extremities. The pharyngeal mucosa was whitish-yellowish and atrophic. The laryngeal mucosa was irregularly thickened. The epiglottis, aryepiglottic folds and vocal cords were involved. Histological examinations of the skin of the left elbow revealed hyaline material diffusely deposited in the dermis. Hyaline substance was found also in the lamina propria of the vocal cord mucosa. An electron microscopic examination revealed that the hyaline material was composed of fine filaments and fine granules.Multiplication of basal laminae was seen around the capillaries. These findings led us to diagnose hyalinosis cutis et mucosae.