Systemic lupus erythematosus complicated with liver cirrhosis in a patient with Papillon-Lefèvre syndrome.

We report the first case of a girl who presented with Papillon-Lefèvre syndrome (PLS) and subsequently developed systemic lupus erythematosus and liver cirrhosis. This indicates that autoimmune diseases can be a complication in patients with PLS. Cathepsin C gene mutations were not found in our patient or her mother. Thus, other genetic factors may have been involved in this patient.

What muscle is responsible for lateral pharyngeal wall movement?

In an attempt to discover what muscle is responsible for medial movement of the lateral pharyngeal wall, the levator muscle was anesthetized with lidocaine under visual control by fiberscope in 3 normal subjects. The results indicate that the medial movement of the lateral pharyngeal wall results from the contraction of the levator. The attendant roll-up of the uvula toward the nasal side induced by the anesthesia was regarded as the action of the uvular muscle without the counterbalance of levator action.

Laryngeal fricative in cleft palate speech.

Some of the substitutions for or [integral of] in cleft palate speech, which was earlier regarded as a pharyngeal fricative, on the basis of listener's judgement, was found to be a laryngeal fricative by means of fluorovideoscopy and nasopharyngofiberscopy. The sound was most likely produced at the stricture between the posteriorly inclined epiglottis and the elevated arytenoids. During the production of laryngeal fricatives, the velopharyngeal port remained open just as for nasal sounds.

Elongation of the nostril and columella using an island flap.

Secondary repair of the cleft lip nose often involves elongating the aperture of the nostril and elongating the columella. A technique is described in which this result can be achieved using an island flap to supplement a septal cartilage strut and local adjustment of the tissues.