Epidermal Nevus Syndrome Associated with Ipsilateral Monostotic Fibrous Dysplasia: An Uncommon Skeletal Abnormality.
- Author:
Heun Joo LEE
1
;
Seung Jae LEE
;
Ji Hye PARK
;
Chong Won CHOI
;
Ga Young LEE
;
Won Serk KIM
Author Information
1. Department of Dermatology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea. gylee0716@hanmail.net
- Publication Type:Case Report
- Keywords:
Epidermal nevus syndrome;
Fibrous dysplasia
- MeSH:
Central Nervous System;
Fibrous Dysplasia, Monostotic;
Humans;
Nevus;
Nevus, Sebaceous of Jadassohn;
Ribs;
Skin
- From:Korean Journal of Dermatology
2013;51(1):32-35
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Epidermal nevus syndromes are a group of congenital syndromes comprising epidermal nevi in conjunction with the central nervous system, ocular, musculoskeletal, and other organ anomalies. Skeletal manifestations in epidermal nevus syndromes are common, ranging from 50% to 66% of patients. However, fibrous dysplasia of the bone has been rarely described in association with epidermal nevus syndromes. Herein, we report a 46-year-old-male patient who presented with extensive epidermal nevi associated with monostotic fibrous dysplasia of the rib which is located ipsilateral to the skin findings.
