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Alternative names Return to top
Leopard syndromeDefinition Return to top
Multiple lentigines syndrome is an inherited disorder characterized by an increased number of lentigines (freckle-like spots).
Causes, incidence, and risk factors Return to top
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.
Symptoms Return to top
Signs and tests Return to top
A physical examination may show signs of mild pulmonic stenosis (obstruction of the pulmonic heart valve) and obstructive cardiomyopathy.Treatment Return to top
Treatment is directed toward the correctable problems. The degree of hearing loss must be determined and appropriate hearing aids supplied. Intervention at the expected time of puberty may be necessary in order precipitate the normal changes of puberty.Expectations (prognosis) Return to top
Most patients adjust very well with proper attention to their specific problems.Complications Return to top
Complications are variable:Calling your health care provider Return to top
Call your health care provider if there are symptoms of this disorder.Prevention Return to top
Genetic counseling is recommended for prospective parents with a family history of multiple lentigines syndrome. Update Date: 10/27/2003 Updated by: Jeffrey Drayer, M.D., Dermatologist, Los Angeles, CA. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |