Описан в 1963 году Lloyd & Dennis.Назван по имени первого пациента - 20 летней девушки с фамилией Cowden

International Cowden Consortium: Operational Criteria (Version 2000)

Diagnosis requires: 1. Mucocutaneous lesions alone if: 

• a. > 6 Facial papules, of which >3 are trichilemmomas, or 
• b. Cutaneous facial papules arid oral mucosal papillomatosis, or 
• c. Oral mucosal papillomatosis and au-al keratoses, or 
• d. > 6 Palmoplantar keratoses 

OR 

2. 2 major criteria, where I includes macrocephaly or Lhermitte-Duclos disease (LDD)  

OR 

3. 1 major + 3 minor criteria 

OR 

4. 4 minor criteria

Pathognomonic Criteria: (Mucocutaneous Lesions)

1. Trichilemmomas: face 
2. Keratoses (au-al) 
3. Papillomatous papules 
4. Mucosal lesions

Major Criteria:

1. Breast cancer 
2. Thyroid cancer (non-medullary, especially follicular) 
3. Macrocephaly (> 95th percentile) 
4. Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) 
5. Endometrial cancer

Minor Criteria:

1. Other thyroid lesions (e.g. adenoma, multinodular goiter) 
2. Mental retardation (IQ < 75) 
3. GI hamartomatous polyposis 
4. Breast: fibrocystic disease 
5. Lipomas 
6. Fibromas 
7. GU tumours (e.g. renal cell carcinoma, uterine fibroids) or malformation