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Cowden's disease (multiple hamartoma syndrome)
Mayo Clinic Proceedings
|March 1, 1981
Summary
Verrucous papules on the skin and in the mouth are key signs of Cowden
Area of Science:
- Dermatology
- Oncology
- Genetics
Background:
- Cowden's disease is a rare genetic disorder.
- Characterized by an increased risk of developing benign and malignant tumors.
Observation:
- Verrucous papules are common diagnostic findings.
- Lesions appear on the face, particularly around orifices, and on extremities.
- Oral lesions occur on the gingiva and tongue.
Findings:
- Skin and oral verrucous papules are diagnostic of Cowden's disease.
- Associated conditions include fibrocystic breast disease, breast cancer, thyroid tumors, ovarian cysts, and GI polyposis.
- These lesions are cutaneous markers for internal malignancies.
Implications:
- Early identification of these skin markers can aid in diagnosing Cowden's disease.
- Facilitates timely screening and management of associated cancers, especially breast and thyroid.
- Distinguishing these lesions from common warts is crucial for patient management.