Idiopathic lenticular mucocutaneous pigmentation or Laugier-Hunziker syndrome with atypical features.

Abstract

Laugier-Hunziker syndrome is a rare, acquired pigmentary disorder of the lips, oral mucosa, and nails. In 1970, Laugier and Hunziker1reported on 5 cases of essential melanotic pigmentation of the mouth and lips. Two of these patients also displayed pigmentary changes of the nails. In the meantime, several authors,2particularly from western Europe, presented more patients with this syndrome. Recently, we examined a patient with mucocutaneous features of both Laugier-Hunziker and Peutz-Jeghers syndrome, whose family history was negative, and in whom, on repeated examinations, intestinal polyposis was not detectable. Report of a Case. A 50-year-old white man first presented at our outpatient clinic in 1982 with a 12-year history of asymptomatic melanotic macules on his lips, oral mucosa, and several fingers. Apart from a noninsulin-dependent diabetes mellitus and coronary heart disease, which had been treated with glibenclamid and sotalol since 1981, his personal history was unremarkable. There was