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تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
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Differential diagnosis of disorders associated with oral lentiginosis

Differential diagnosis of disorders associated with oral lentiginosis
Syndrome Inheritance/gene Cutaneous pigmentation Testicular tumors Gastrointestinal involvement Malignancy Thyroid abnormalities Other endocrinopathies
McCune-Albright syndrome Somatic/GNAS "Coast of Maine" café-au-lait patches; oral mucosal lentigines Leydig cell hyperplasia; Sertoli cell hyperplasia Possible hamartomatous polyps; IPMN Small increase in risk of malignancies in affected tissues (thyroid, bone, pancreas) Patchy heterogeneity and cystic changes; hyperthyroid Precocious puberty; GH excess; neonatal Cushing syndrome
Peutz-Jeghers syndrome AD/STK11 (LKB1)* Mucosal and cutaneous lentigines Large cell calcifying Sertoli cell tumor Hamartomatous polyps Ovarian; breast; pancreatic; thyroid; cervix; cholangiocarcinoma Malignancy None
Carney complex AD/PRKAR1a Cutaneous and mucosal lentigines; blue nevi; melanocytic nevi; CALM; hypopigmented lesions Large cell calcifying Sertoli cell tumor; Leydig cell tumor Psammomatous melanocytic schwannoma; pancreatic lesions including IPMN Adrenal; hepatocellular; pancreatic; osteochondromyxomas Hyperplasia; cystic changes; malignancy PPNAD; GH excess; prolactin excess; pituitary adenomas; Cushing syndrome
Laugier-Hunziker syndrome Acquired Melanonychia striata; genital pigmentation; palmoplantar pigmentation None None None None None
GNAS: complex locus, guanine nucleotide alpha-stimulating; IPMN: intraductal papillary mucinous neoplasms of the pancreas; GH: growth hormone; AD: autosomal dominant; STK11: serine/threonine kinase 11; PRKAR1a: protein kinase cyclic adenosine monophosphate-dependent regulatory, type 1-alpha; CALM: café-au-lait macule; PNAD: primary pigmented nodular adrenocortical disease.
* In 50 to 70 percent of reported Carney complex cases.
¶ Majority due to mutation of PRKAR1a; second locus on chromosome 2p16 identified but gene unknown.
Reproduced with permission from: Pichard DC, Boyce AM, Collins MT, Cowen EW. Oral pigmentation in McCune-Albright syndrome. JAMA Dermatol 2014; 150:760. Copyright © 2014 American Medical Association. All rights reserved.
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