title: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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: Department of Dermatology, Hedi Chaker University Hospital, 3029 Sfax, Tunisia.
authors: Abderrahmen
authors: A
authors: Masmoudi
authors: Zied Mohamed
authors: ZM
authors: Chermi
authors: Slaheddine
authors: S
authors: Marrekchi
authors: Ben Salah
authors: BS
authors: Raida
authors: Sonia
authors: S
authors: Boudaya
authors: Madiha
authors: M
authors: Mseddi
authors: Meziou Taha
authors: MT
authors: Jalel
authors: Hamida
authors: H
authors: Turki