Korean J Dermatol.  2010 Dec;48(12):1136-1139.

Cowden Syndrome Accompanied by Multiple Sclerotic Fibroma on the Face

• Kim ST
• Kang DY
• Kang JS
• Baek JW
• Jeon YS
• Suh KS

Abstract

Cowden syndrome (CS) is a rare genodermatosis that is characterized by multiple hamartomatous tumors of an ectodermal, mesodermal and endodermal origin. CS is associated with an increased risk of malignancy and especially breast cancer, thyroid cancer, uterine cancer and renal cell carcinoma. The characteristic features of the disease are mucocutaneous findings, including multiple facial trichilemmomas, oral mucosal papillomatosis and acral keratoses. Multiple sclerotic fibroma is also known to be a clue to diagnose CS. We herein emphasize that multiple sclerotic fibromas of the skin are an important marker of CS, which may also be helpful for making an early diagnosis.

Keyword

Cowden syndrome; Sclerotic fibroma

MeSH Terms

Breast Neoplasms
Carcinoma, Renal Cell
Early Diagnosis
Ectoderm
Endoderm
Fibroma
Hamartoma Syndrome, Multiple
Keratosis
Mesoderm
Papilloma
Skin
Thyroid Neoplasms
Uterine Neoplasms