Diffuse lentiginosis in a patient with Werner's syndrome-a possible association with incomplete leopard syndrome
- 1 January 1995
- journal article
- case report
- Published by Wiley in Clinical and Experimental Dermatology
- Vol. 20 (1) , 46-50
- https://doi.org/10.1111/j.1365-2230.1995.tb01282.x
Abstract
A classical case of Werner's syndrome is described. In addition to the numerous skin changes that are typically associated with Werner's syndrome, our patients also displayed diffuse lentiginosis, and several of the clinical features of leopard syndrome. Histopathological and ultrastructural findings from a hyperpigmented macule displayed the typical features of a simple lentigo. A striking feature was the presence of melanosomes in Langerhans cells as has been reported in the leopard syndrome. A possible generalized mesodermal defect has been suggested in Werner's syndrome, while the basic defect in the leopard syndrome is thought to be of neuroectodermal origin with pleiotropic changes in the organs derived from the mesoderm. Our patient, with incomplete leopard syndrome and typical Werner's syndrome, may be an example of an association of genetic defects affecting both tissues of neuroectodermal and mesodermal origin.Keywords
This publication has 12 references indexed in Scilit:
- Inherited patterned lentiginosis in blacksArchives of Dermatology, 1989
- Werner's syndrome. Evidence for preferential regional expression of a generalized mesenchymal cell defectArchives of Dermatology, 1988
- The Complex of Myxomas, Spotty Pigmentation, and Endocrine OveractivityMedicine, 1985
- Morphologic Alterations of Epidermal Melanocytes and Melanosomes in PUVA Lentigines: A Comparative Ultrastructural Investigation of Lentigines Induced by PUVA and SunlightJournal of Investigative Dermatology, 1984
- The PUVA Lentigo: An Analysis of Predisposing FactorsJournal of Investigative Dermatology, 1983
- Werner's syndrome: A review of recent research with an analysis of connective tissue metabolism, growth control of cultured cells, and chromosomal aberrationsHuman Genetics, 1982
- Cardio‐cutaneous syndrome (the “LEOPARD” syndrome). Review of the literature and a new familyClinical Genetics, 1976
- The multiple lentigines syndromeArchives of Dermatology, 1973
- Multiple Lentigenes SyndromeAmerican Journal of Diseases of Children, 1969
- WERNER'S SYNDROME (PROGERIA OF THE ADULT) AND ROTHMUND'S SYNDROME: TWO TYPES OF CLOSELY RELATED HEREDOFAMILIAL ATROPHIC DERMATOSES WITH JUVENILE CATARACTS AND ENDOCRINE FEATURES; A CRITICAL STUDY WITH FIVE NEW CASESAnnals of Internal Medicine, 1945