[Cowden's syndrome].
Multiple hamartoma and neoplasia syndrome, or Cowden's disease, is a genodermatosis inherited as an autosomal dominant trait with variable expressivity. We describe a woman with classic mucocutaneous features of the syndrome: facial papules, acral keratoses, oral mucosal papillomatosis, 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 thyroiditis. A checklist for use in screening patients with Cowden's disease is proposed.
Krasovec
M
M
Dermatologische Klinik, Universitätsspital, Zürich.
Elsner
P
P
Burg
G
G
D000328:Adult; D001706:Biopsy; D005260:Female; D006223:Hamartoma Syndrome, Multiple / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D006801:Humans; D008545:Melanoma / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D013967:Thyroiditis, Autoimmune / Q000175:diagnosis / Q000235:genetics / Q000473:pathology
Cowden-Syndrom.