Storiform collagenoma as a clue for Cowden disease or PTEN hamartoma tumour syndrome (PHTS)
- Wael Ismail Al-Daraji (waldaraji{at}aol.com)
- Dermatology department, Sheffield University Teaching Hospitals NHS foundation Trust, United Kingdom
- Histopathology department, Sheffield University Teaching Hospitals NHS foundation Trust, United Kingdom
- Published Online First 18 May 2007
Abstract
Cowden disease (CD) is a rare autosomal dominant condition with cutaneous and systemic findings. Classically, the cutaneous features of CD include tricholemmomas, oral fibromas, acral keratoses and palmar pits. Storiform collagenoma (or sclerotic fibroma) has also been described but less frequently. The age at presentation is usually around late adolescence. We report a unique case of an 18-year old male with a non-tender erythematous 10mm nodule on his back. Microscopic examination showed typical features of storiform collagenoma. Subsequent clinical evaluation suggested a diagnosis of CD. To the best of our knowledge, in the English literature, this is the first case of Cowden disease/PTEN hamartoma tumour syndrome diagnosed following initial presentation with a storiform collagenoma.
