Freckles (Ephelides)

Freckles are the most common hyperpigmented macules seen in childhood. They are light to dark brown in color, smaller than 3 mm in diameter, irregular in contour, and found on sun-exposed areas such as the face, upper back and chest, and dorsal surface of the arms. They are not present at birth and develop over childhood. They are found most often in individuals with red hair and fair skin. The typical pattern is for freckles to increase in number and prominence in the summer and fade in the winter. The development of excessive freckling may suggest an underlying photosensitivity, as seen in xeroderma pigmentosum. Although benign in nature, individuals with lighter pigmented skin who are predisposed to the development of freckles are at higher risk of developing malignant melanoma.

Lentigines

Lentigines are round, brown to black macules, 4 to 10 mm in diameter that increase in number during adolescence and, at times, can be almost indistinguishable from ephelides. Clinically, lentigines occur anywhere on the body, not just in sun-exposed areas, and do not fade in the winter. Lentigines are quite common and benign when few in number. However, when multiple, lentigines constitute the primary clinical feature of a number of syndromes. The most important syndromes with lentigines as a defining feature are LEOPARD (lentigines, electrocardiogram abnormalities, ocular hypertelorism, pulmonary stenosis, abnormal genitalia, retardation of growth, and deafness) syndrome, also known as multiple lentiginous syndrome, Peutz-Jeghers syndrome, and Carney’s complex (Figure 124-1 and Table 124-1). The clinician evaluating a patient with innumerable lentigines on examination should keep the above diagnoses in mind, especially if lentigines are noted on mucosal surfaces or cross the vermillion border because these are not features of benign lentiginosis.

Figure 124-1 Lentigines.

Table 124-1 Major Lentiginous Syndromes