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Multiple lentigines syndrome |
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| Definition: | An inherited disorder characterized by an increased number of lentigines (freckle-like spots).
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| Alternative Names: | Leopard syndrome
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| Causes, incidence, and risk factors: | Multiple lentigines syndrome is inherited as an autosomal dominant trait. Affected people have large numbers of lentigines (freckle-like lesions present from birth and somewhat darker than true freckles) as the most obvious sign. These are located mostly on the trunk and neck. Affected people also have wide-set eyes (hypertelorism), prominent ears, nerve deafness (partial), and cafe-au-lait spots (light brown birthmarks).
Additional findings include mild pulmonic stenosis and changes in the ECG. Affected people may have abnormal genitalia (cryptorchidism), hypogonadism, or delayed puberty.
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Review Date: 12/2/2001
Reviewed By: Michael Lehrer, M.D., Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
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