Korean J Dermatol.  2013 Jan;51(1):32-35.

Epidermal Nevus Syndrome Associated with Ipsilateral Monostotic Fibrous Dysplasia: An Uncommon Skeletal Abnormality

• Lee HJ
• Lee SJ
• Park JH
• Choi CW
• Lee GY
• Kim WS

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.

Keyword

Epidermal nevus syndrome; Fibrous dysplasia

MeSH Terms

Central Nervous System
Fibrous Dysplasia, Monostotic
Humans
Nevus
Nevus, Sebaceous of Jadassohn
Ribs
Skin
Nevus, Sebaceous of Jadassohn