TAILIEUCHUNG - Chapter 054. Skin Manifestations of Internal Disease (Part 11)

Also lentigines. b Polyostotic fibrous dysplasia. c See also "Papulonodular Skin Lesions." d Late 1980s. A proliferation of melanocytes results in the following pigmented lesions: lentigo, melanocytic nevus, and melanoma (Chap. 83). In an adult, the majority of lentigines are related to sun exposure, which explains their distribution. , in the Peutz-Jeghers and LEOPARD [lentigines; ECG abnormalities, primarily conduction defects; ocular hypertelorism; pulmonary stenosis and subaortic valvular stenosis; abnormal genitalia (cryptorchidism, hypospadias); retardation of growth; and deafness (sensorineural)] syndromes, lentigines do serve as a clue to systemic disease. In LEOPARD syndrome, hundreds of lentigines develop during childhood and are scattered over the entire surface of the body. . | Chapter 054. Skin Manifestations of Internal Disease Part 11 Also lentigines. Polyostotic fibrous dysplasia. cSee also Papulonodular Skin Lesions. Late 1980s. A proliferation of melanocytes results in the following pigmented lesions lentigo melanocytic nevus and melanoma Chap. 83 . In an adult the majority of lentigines are related to sun exposure which explains their distribution. in the Peutz-Jeghers and LEOPARD Zentigines ECG abnormalities primarily conduction defects ocular hypertelorism pulmonary stenosis and subaortic valvular stenosis abnormal genitalia cryptorchidism hypospadias retardation of growth and deafness sensorineural syndromes lentigines do serve as a clue to systemic disease. In LEOPARD syndrome hundreds of lentigines develop during childhood and are scattered over the entire surface of the body. The lentigines in patients with Peutz-Jeghers syndrome are located primarily around the nose and mouth on the hands and feet and within the oral cavity. While the pigmented macules on the face may fade with age the oral lesions persist. However similar intraoral lesions are also seen in Addison s disease and as a normal finding in darkly pigmented individuals. Patients with this autosomal dominant syndrome due to mutations in a novel serine threonine kinase gene have multiple benign polyps of the gastrointestinal tract testicular tumors and an increased risk of developing gastrointestinal primarily colon pancreatic and gynecologic cancers. In the Carney complex numerous lentigines are also seen but in association with cardiac myxomas. This autosomal dominant disorder is also known as the LAMB Zentigines atrial myxomas mucocutaneous myxomas and Mue nevi syndrome or NAME nevi atrial myxoma myxoid neurofibroma and ephelides freckles syndrome. These patients can also have evidence of endocrine overactivity in the form of Cushing s syndrome acromegaly or sexual precocity. The third type of localized hyperpigmentation is due to a local increase in pigment .