MedlinePlus Medical Encyclopedia: Multiple lentigines syndrome

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Multiple lentigines syndrome

Contents of this page:
Illustrations Alternative names Definition Causes, incidence, and risk factors Symptoms Signs and tests	Treatment Expectations (prognosis) Complications Calling your health care provider Prevention

Illustrations

Pectus excavatum

Alternative names Return to top

Leopard syndrome

Definition 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

a family history of multiple lentigines
multiple lentigines on neck and trunk (may be on any cutaneous surface)
cafe-au-lait spots
wide-set eyes (hypertelorism)
prominent ears
pectus excavatum
pectus carinatum (abnormalities of the sternum or breastbone)
undescended testicles (cryptorchidism)
slow growth
delayed puberty
absent puberty

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.

Tests:

ECG, may show abnormalities
hearing test
endocrine evaluation

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:

deafness
delayed puberty
heart problems
infertility

Calling your health care provider Return to top

Call your health care provider if there are symptoms of this disorder.

Call for an appointment with your health care provider if you have a family history of this disorder and plan to have children.

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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