Multiple Hamartoma Syndrome With Endometrial Carcinoma and the Sign of Leser-Trélat

Abstract

Multiple hamartoma syndrome (MHS) is a rare, autosomal dominant genodermatosis that is important for physicians to recognize, since it is associated with a high incidence of hamartomas and carcinomas in numerous sites. The sign of Leser-Trelat is likewise important, since the sudden appearance of numerous, often pruritic, seborrheic keratoses may be associated with the development of an occult malignant neoplasm. 1-3 Both entities are rare and so would not be expected to occur together. As they both are related to the development of malignant neoplasms, however, an association greater than that dictated by chance alone might be expected. We describe herein a patient with MHS in whom endometrial carcinoma developed who also demonstrated the sign of Leser-Trelat. Report of a Case A 57-year-old woman was seen in May 1980 for evaluation of multiple perineal warts. At 22 years of age, she had had a partial thyroidectomy for goiter, which had