A Case of Multiple Lentigines Sydrome with a Family History of Multiple Lentigines.
- Author:
So Jin KIM
1
;
Phil Seung SEO
;
Nyung Hoon YOON
;
Seok Don PARK
Author Information
1. Department of Dermatology, Wonkwang University School of Medicine, Iksan, korea. sdpark@wonkwang.ac.kr
- Publication Type:Case Report
- Keywords:
Multiple lentigines syndrome;
Hypertrophic cardiomyopathy;
Family history
- MeSH:
Adolescent;
Biopsy;
Cardiomyopathy, Hypertrophic;
Deafness;
Electrocardiography;
Genitalia;
Humans;
Hypertelorism;
Lentigo*;
LEOPARD Syndrome;
Male;
Melanins;
Melanocytes;
Mothers;
Panthera;
Penetrance;
Pulmonary Valve Stenosis
- From:Korean Journal of Dermatology
2004;42(12):1581-1584
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Multiple lentigines syndrome is an autosomal dominant disorder of variable penetrance and expressivity. LEOPARD has been used to describe the main features of the syndrome, including lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retardation of growth and deafness. We describe a case of multiple lentigines syndrome with a family history of multiple lentigines. A 15-year-old boy presented with multiple brown macules and patches on the whole body, which had developed since the age of 3. He also had hypertrophic cardiomyopathy, EKG abnormalities and retarded growth. His maternal grandfather, maternal uncle and mother also presented with multiple brown macules scattered over the body. The biopsy specimen taken from the macule revealed elongated rete ridges, increased melanin deposition and increased number of melanocytes.
