Generalized Lentiginosis

November 1, 2008

Numerous brown macules were sparsely distributed over the torso, head, and extremities of an African American newborn. The infant also had a mongolian spot on the buttocks. The baby's mother, a great aunt on the father's side, and the great aunt's daughter and grandson had similar brown macules at birth. All family members were healthy.

Numerous brown macules were sparsely distributed over the torso, head, and extremities of an African American newborn. The infant also had a mongolian spot on the buttocks. The baby's mother, a great aunt on the father's side, and the great aunt's daughter and grandson had similar brown macules at birth. All family members were healthy.

 Congenital lentigines are dark brown to black, 1- to 2-mm macules; they can be widespread over multiple cutaneous areas as well as on mucous membranes. Histological examination of lentigines reveals elongated rete ridges, increased melanocytes in the basal layer, and increased melanization of the basal keratinocytes. These lesions do not change with sun exposure. In African American infants or those with a dark complexion, the lesions may blend in with the skin hyperpigmentation that develops naturally within the first few months of life. In infants with fair skin, however, the lesions may persist.

Lentigines are associated with several major syndromes:

•LEOPARD syndrome-lentigines, ECG abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of the genitalia, growth retardation, and deafness.1,2 Skin changes do not appear until after 1 year of age.

•NAME syndrome-nevi (lentigines, ephelides, and blue nevi), atrial myxoma, myxoid skin tumors, and endocrine abnormalities.3

•LAMB syndrome-lentigines, atrial myxoma, mucocutaneous myxoma, and blue nevi.4

•Carney syndrome-a complex syndrome that combines characteristics of NAME and LAMB syndromes.5,6

•Peutz-Jeghers syndrome-an autosomal dominant disorder characterized by mucocutaneous melanocytic macules, usually on the lips and buccal and gingival mucosae, and melanin spots on the fingers and toes; it may be congenital.

•Centrofacial lentiginosis (or Touraine syndrome)-an autosomal dominant disorder characterized by lentigines, particularly on the nose and cheeks, that appear during the first year of life, and by a multitude of associated abnormalities.7

Generalized lentigines that are not associated with other abnormalities have been referred to as generalized lentiginosis. Regular screening for systemic abnormalities is advised in infants with generalized lentigines. This baby had no evidence of any associated abnormalities at birth. By 1 month of age, the macules had combined with the natural pigmentation and could no longer be distinguished.

 

References:

REFERENCES:


1.

Coppin BD, Temple IK. Multiple lentigines syndrome (LEOPARD syndrome or progressive cardiomyopathic lentiginosis).

J Med Genet.

1997;34: 582-586.

2.

Arnsmeier SL, Paller AS. Pigmentary anomalies in the multiple lentigines syndrome: is it distinct from LEOPARD syndrome?

Pediatr Dermatol

. 1996;13:100-104.

3.

Atherton DJ, Pitcher DW, Wells RS, MacDonald DM. A syndrome of various cutaneous pigmented lesions, myxoid neurofibromata and atrial myxoma: the NAME syndrome.

Br J Dermatol

. 1980;103:421-429.

4.

Rhodes AR, Silverman RA, Harrist TJ, Perez- Atayde AR. Mucocutaneous lentigines, cardiomucocutaneous myxomas, and multiple blue nevi: the "LAMB" syndrome.

J Am Acad Dermatol.

1984;10: 72-82.

5.

Carney JA, Gordon H, Carpenter PC, et al. The complex of myxomas, spotty pigmentation, and endocrine overactivity.

Medicine

(

Baltimore

). 1985; 64:270-283.

6.

Egan CA, Stratakis CA, Turner ML. Multiple lentigines associated with cutaneous myxomas.

J Am Acad Dermatol.

2001;44:282-284.

7.

Paller AS, Mancini AJ, eds.

Hurwitz Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence

. 3rd ed. Philadelphia: Elsevier Saunders; 2006:285.