Ann Dermatol.  1997 Jul;9(3):219-223. 10.5021/ad.1997.9.3.219.

A Case of Multiple Lentigines Syndrome

• Shin DY
• Koo DW
• Roh JY

• KMID: 2360368
• DOI: http://doi.org/10.5021/ad.1997.9.3.219

Abstract

The multiple lentigines syndrome or LEOPARD syndrome is an autosomal dominantly inherited disorder with a variety of abnormalities and a familial occurrence. This syndrome is characterized by the presence of numerous dark brown macules on the skin but not the mucous surface, and by a marked increase in the number of lentigines from birth to puberty. The eponym LEOPARD stands for lentigines, EKG abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of the genitalia, retardation of growth and deafness. We report a case of multiple lentigines syndrome in 7-year-old boy. He had numerous pinhead to pea sized, dark brownish macules scattered on the entire body and also had pulmonary stenosis, EKG abnormality, ocular hypertelorism and right exotropia. Interestingly, he also had a labial melanotic macule on the lower lip, which is usually spared in the multiple lentigines syndrome. Histologically, the biopsy specimen taken from the macule revealed an elongation of rete ridges, an increase of melanin pigments in the basal layer and mild inflammatory infiltrates intermingled with the melanophages in the upper dermis.

Keyword

Multiple lentigines syndrome; LEOPARD syndrome

MeSH Terms

Adolescent
Biopsy
Child
Deafness
Dermis
Electrocardiography
Eponyms
Exotropia
Genitalia
Humans
Hypertelorism
Lentigo
LEOPARD Syndrome*
Lip
Male
Melanins
Panthera
Parturition
Peas
Puberty
Pulmonary Valve Stenosis
Skin
Melanins