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Lipoid proteinosis of the larynx

LIPOID proteinosis, or hyalinosis cutis et mucosae, was first described by Urbach and Wiethe (1929) after whom this condition is known eponymously. It is a rare inherited disorder characterized by abnormal deposition of a lipoprotein and affects principally the skin and mucous membranes. Its importance to laryngologists lies in its occurrence in the larynx, necessitating tracheostomy on occasion. Our present case, a man then aged 29, sought treatment for hoarseness in July 1965. This had been present for many years, probably beginning at the age of 16. Since the age of 20 he had noticed dryness and thickening of the skin over the arms, hands and face with a tendency to bruise easily. On occasions he had suffered ulceration of the mouth but this feature had not been unduly troublesome. Of his immediate family he knew very little save that a maternal uncle had 'voice trouble' of uncertain cause. The skin was dry, pigmented and rough over the arms, hands, face and neck, with typical pearly deposits along the margin of the upper eyelids.* There was marked hoarseness. The nose was healthy. There were a few petechiae on the soft palate and uvula. The tongue was normal. Indirect laryngoscopy failed to give an adequate view of the larynx and mirror examination of the nasopharynx was likewise unsuccessful. Clinical examination of the nervous system, chest and abdomen revealed no abnormality, though there was radiological evidence of a healed peptic ulcer. He also had a ventricular septal defect. The haemoglobin level was 16 g. per 100 ml. and the white blood count 87oo/mm3. The serum protein level was 7-2 g. per 100 ml. with normal electrophoretic pattern. Direct laryngoscopy was undertaken on 21 July 1965 and the findings were as shown in Figure 1. The main feature was mammillation and thickening of the mucosa in the anterior commissure and in the inter-arytenoid region. The remainder of the larynx was normal at this time. Histological examination of the lesions in the larynx showed them to be covered by normal laryngeal epithelium (Fig. 2). Immediately deep to this epithelium were amorphous deposits of extracellular hyaline material. This gave a strongly positive P.A.S. reaction indicating the presence of carbohydrate. In the deposits, fine droplets of lipoid material could be demonstrated. In November of 1965 he was admitted to hospital for treatment of a spontaneous detached retina and a scleral buckling operation was performed. He was re-admitted for further laryngoscopy on 14 October 1971. At this time his voice was very hoarse but he was otherwise well and had no stridor. He had noticed recent worsening of the hoarseness. His skin condition was