Cowden's disease

Cowden's disease, or multiple hamartoma syndrome, is a rare genodermatosis. Multiple facial trichilemmomas, acral keratoses, and oral papillomas are the most common mucocutaneous lesions in this disease. It is associated with increased rates of cancer of the breast and thyroid as well as benign tumors and hamartomas of multiple organ systems. Multiple facial trichilemmomas are essentially pathognomonic of this syndrome and may appear as lesions of the eyelid and periorbital skin. A case of Cowden's disease is described and previously reported cases reviewed, with emphasis on the ocular aspects of the disease.