Multiple lentigines syndrome is an inherited disorder in which there is an increased number of lentigines (freckle-like spots) on the body.
Multiple lentigines syndrome is inherited as an
Persons with this condition have large numbers of lentigines. Lentigines are skin markings that are darker than true freckles. They are present from birth. They are located mostly on the trunk and neck.
Symptoms of multiple lentigines include:
- Abnormal genitalia
- Absent or delayed puberty
Cafe-au-lait spots(light brown birthmarks)
- Hearing problems (partial deafness)
- Heart defects (electrocardiographic conduction)
- Multiple spots on neck and trunk
- Pectus carinatum (abnormalities of the sternum or breastbone)
- Prominent ears
- Pulmonary artery narrowing
- Slow growth
- Wide-set eyes (hypertelorism)
Note: Scattered lentigines is normal and does not indicate a problem.
Exams and tests
The health care provider will perform a physical exam and listen to your heart with a stethoscope. There may be signs of a heart valve problems or obstructive
Tests that may be done can include:
ECG Hearing test
- CT scan of the brain
- Skull x-ray
Symptoms are treated as appropriate. A hearing aid may be needed. Hormone treatment may be necessary at the expected time of puberty to cause the normal changes to occur.
Laser or bleaching creams may help lighten some of the brown spots on the skin.
Most patients adjust very well with proper attention to their specific problems.
Complications vary and include:
- Delayed puberty
- Heart problems
When to Contact a Medical Professional
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.
Genetic counseling is recommended for people with a family history of multiple lentigines syndrome who want to have children.