COWDENS-DISEASE

Multiple hamartoma and neoplasia syndrome, or Cowden's disease, is a g enodermatosis inherited as an autosomal dominant trait with variable e xpressivity. We describe a woman with classic mucocutaneous features o f the syndrome: facial papules, acral keratoses, oral mucosal papillom atosis, oral polyps and scrotal tongue. As a child she had strabismus. Her family history is positive for the disease, and a sister died of malignant melanoma. The check-up revealed chronic lymphocytic thyroidi tis. A checklist far use in screening patients with Cowden's disease i s proposed.