Multiple Lentigenes Syndrome: Complex Comprising Multiple Lentigenes, Electrocardiographic Conduction Abnormalities, Ocular Hypertelorism, Pulmonary Stenosis, Abnormalities of Genitalia, Retardation of Growth, Sensorineural Deafness, and Autosomal Dominant Hereditary Pattern

Abstract

MULTIPLE lentigenes have been reported in association with a variety of abnormalities and a familial occurrence. It is likely, on the basis of the following literature review and additional case material, that multiple lentigenes commonly represent one facet of a more generalized hereditary syndrome. Zeisler and Becker 1 described a 24-year-old woman who experienced a marked increase in number of lentigenes from birth to puberty. There were none on the face, but otherwise they were generally distributed. The patient also had pectus carinatum, ocular hypertelorism, and mandibular prognathism. Rosen 2 reported three siblings (two male and one female) with large numbers of lentigenes, but no mention was made of other disorders. A large pedigree in which eight individuals in three generations had lentigenes was reported by Pipkin and Pipkin. 3 Seven affected members had nystagmus, and photographs of some members gave evidence of mandibular prognathism. The pedigrees suggested an autosomal