Epidermal nevus syndrome : Association with central precocious puberty and woolly hair nevus

The epidermal nevus syndrome is characterized by the association of epidermal nevi with abnormalities of the skin, skeletal system, central nervous system, eyes, and cardiovascular system, as well as with malignant conditions. We describe a 2-year-old girl with an extensive epidermal nevus involving...

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Bibliographic Details
Published in:Journal of the American Academy of Dermatology 1996-11, Vol.35 (5), p.839-842
Main Authors: TAY, Y.-K, WESTON, W. L, GANONG, C. A, KLINGENSMITH, G. J
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child, Preschool
Dermatology
Female
Hair Diseases
Humans
Medical sciences
Nevus, Pigmented
Puberty, Precocious
Scalp
Skin Neoplasms
Syndrome
Tumors of the skin and soft tissue. Premalignant lesions
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Summary:The epidermal nevus syndrome is characterized by the association of epidermal nevi with abnormalities of the skin, skeletal system, central nervous system, eyes, and cardiovascular system, as well as with malignant conditions. We describe a 2-year-old girl with an extensive epidermal nevus involving the left side of the body (nevus unius lateris) and associated with a woolly hair nevus on the left parietal area of the scalp and multiple acquired melanocytic nevi. Idiopathic central precocious puberty characterized by premature breast and public hair development and advanced bone age developed at the age of 20 months. A sharp increase in serum gonadotropins after a luteinizing hormone releasing hormone (LHRH) stimulation test confirmed the presence of central precocious puberty. This is the third reported case of precocious puberty associated with the epidermal nevus syndrome.
ISSN:0190-9622
1097-6787
DOI:10.1016/S0190-9622(96)90098-5