Lentigines

▶Lentigines are persistent macular areas of hyperpigmentation that can occur on any skin or mucosal surface, regardless of sun exposure.

▶The incidence is unknown, but isolated lentigines appear to be very common.

▶Lentigines are usually small (≤5 mm), hyperpigmented macules that mimic ephelides (ie, freckles).

■Lentigines may be brown to black, are well-defined, and may be widely distributed (ie, not limited to sun-exposed areas).

■Lesions do not become more apparent after sun exposure.

■Isolated lentigines have no clinical significance.

▶Multiple lentigines may be associated with systemic disorders; the most common of these are as follows:

■LEOPARD syndrome: LEOPARD is an acronym for the major defects in this autosomal dominantly inherited disorder: lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, retarded (delayed) growth, and sensorineural deafness (Figure 73.1).

■Peutz-Jeghers syndrome: autosomal dominant disorder consisting of face, lip, and oral mucosa lentigines associated with benign intestinal polyposis (Figure 73.2). These patients may also have lentigines on the fingers, toes, palms, and soles.

■Lentiginosis with cardiocutaneous myxomas consists of multiple lentigines associated with cardiac and subcutaneous myxomas. Disorders considered within this disease category include the following:

– Carney complex: lentigines, cardiac and other myxomas, and endocrine tumors.

– LAMB syndrome: lentigines, atrial myxomas, mucocutaneous myxomas, and blue nevi.

– NAME syndrome: nevi, atrial myxomas, myxoid neurofibromas, ephelides, and endocrine neoplasia.

Figure 73.1. Patient with Leopard Syndrome. Multiple Lentigines with Relative Sparing of the Mucous Membranes. Reproduced with Permission from Cohen Ba. Pediatric Dermatology. 3rd Ed. Mosby; 2005:144.

Figure 73.2. In Peutz-Jeghers Syndrome, Lentigines Appear on the Face, Lips, and Oral Mucosa. They are Associated with Gastrointestinal Polyposis.

Look-alikes

Disorder	Differentiating Features
Ephelides	Small, hyperpigmented macules located in sun-exposed areas such as face, upper chest, and back. Unlike lentigines, ephelides become darker after sun exposure and often fade in absence of sun (ie, winter months).
Café au lait macules	Hyperpigmented macules typically larger than lentigines.
Labial melanotic macule	Small (1–8 mm), brown to dark brown or black macule. Most often on the lower lip; typically solitary (Figure 73.3).
Melanocytic nevi	Typically more deeply pigmented than lentigines. Often slightly elevated and have surface textural change. May develop hypertrichosis.

Figure 73.3. Labial Melanotic Macule. Small, Dark Brown, Solitary Macule on the Left Side of the Lower Lip of a Young Child with Eczema.

▶The diagnosis is made clinically on the basis of the appearance and distribution (ie, not limited to sun-exposed areas) of lesions.

▶Lentigines do not require treatment unless desired for cosmetic reasons.

▶Identification of multiple lentiginosis syndromes is of paramount importance.

▶If multiple lentigines are present, assess for clinical features of an associated syndrome (eg, LEOPARD, Peutz-Jeghers, lentiginosis with cardiocutaneous myxomas) and consider dermatology and genetics referral.

▶If multiple lentigines without mucosal lentigines, consider electrocardiography, echocardiography, hearing test, endocrine evaluation.

▶If multiple lentigines including mucosal lentigines, consider referral to gastroenterology.

▶Excellent if isolated lentigines. Lesions that appear early in life may fade or spontaneously resolve.

▶Presence of an associated syndrome alters prognosis.

▶Multiple or mucosal lentigines are observed (may indicate the presence of an associated disorder).

section name header

Introduction/Etiology/Epidemiology ⬇

Signs and Symptoms ⬆ ⬇

How to Make the Diagnosis ⬆ ⬇

Treatment ⬆ ⬇

Treating Associated Conditions ⬆ ⬇

Prognosis ⬆ ⬇

When to Worry or Refer ⬆