Cowden syndrome.

Abstract

BACKGROUND:

Cowden syndrome is a rare genodermatosis charactarized by presence of multiple hamartomas. The aim of the study was to specify the clinical, therapeutic and prognostic aspects of Cowden syndrome.

CASES REPORT:

Our study included 4 patients with Cowden syndrome, 2 males and 2 females between 14 and 46 years old. Clinical examination of the skin revealed facials papules (4 cases), acral keratosis (1 case), translucent keratotic papules (2 cases). Oral examination revealed papules (4 cases), papillomatosis (4 cases), gingival hypertrophy (4 cases) and scrotal tongue (2 cases). Investigations revealed thyroid lesions (2 cases), fibrocystic disease and lipoma of the breast in 1 case, "glycogenic acanthosis" (1 case), macrocephaly (2 cases), dysmorphic face (1 case) and lichen nitidus (1 case). Oral etretinate and acitretine were temporary efficient in 2 patients. Topical treatment with tretinoin lotion resulted in some improvement in cutaneous, but not mucosal lesions in one patient. No cancer was revealed.

CONCLUSION:

The pathognomonic mucocutaneous lesions were found in all patients. However, no degenerative lesions have been revealed. A new association of Cowden syndrome with lichen nitidus was found. Treatment with oral retinoids was efficient on cutaneous lesions.

• Authors: Abderrahmen Masmoudi, Zied Mohamed Chermi, Slaheddine Marrekchi, Ben Salah Raida, Sonia Boudaya, Madiha Mseddi, Meziou Taha Jalel, Hamida Turki
• Journal: Journal of dermatological case reports (J Dermatol Case Rep) Vol. 5 Issue 1 Pg. 8-13 (Mar 26 2011) ISSN: 1898-7249 [Electronic] Poland
• PMID: 21886759 (Publication Type: Case Reports)