Multiple lentigines syndrome
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Definition
Multiple lentigines syndrome is an inherited disorder identified by an increased number of lentigines (freckle-like spots).
Alternative Names
Leopard syndrome
Causes, incidence, and risk factors
Multiple lentigines syndrome is inherited as an autosomal dominant trait. People with this condition have large numbers of lentigines. Lentigines are skin markings that are somewhat darker than true freckles. They are present from birth. They are located mostly on the trunk and neck.
Symptoms
Symptoms of multiple lentigines include:
- Abnormal genitalia
- Absent or delayed puberty
- Cafe-au-lait spots (light brown birthmarks)
- Hearing problems (partial deafness)
- Hypogonadism
- Multiple spots on neck and trunk
- Pectus carinatum (abnormalities of the sternum or breastbone)
- Pectus excavatum
- Prominent ears
- Slow growth
- Undescended testicles (cryptorchidism)
- Wide-set eyes (hypertelorism)
Note: Scattered lentigines is normal and does not indicate a problem.
Signs 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 cardiomyopathy.
Tests that may be done can include:
Treatment
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.
Support Groups
Expectations (prognosis)
Most patients adjust very well with proper attention to their specific problems.
Complications
Complications vary and include:
Calling your health care provider
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
Genetic counseling is recommended for people with a family history of multiple lentigines syndrome who want to have children.
References
Gibbs NF, Makkar HS. Disorders of Hyperpigmentation and Melanocytes. In: Eichenfield LF, Frieden IJ, Esterly NB, eds. Textbook of Neonatal Dermatology. 2nd Ed. Philadelphia, Pa: Saunders Elsevier; 2001: p. 196.
Version Info
- Last Reviewed on 05/13/2011
- Jonathan Kantor, MD, North Florida Dermatology Associates, Jacksonville, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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This page was last updated: May 31, 2013