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Benign pigmented skin lesions other than melanocytic nevi (moles)

Julie V Schaffer, MD
Jean L Bolognia, MD
Section Editors
Moise L Levy, MD
Robert P Dellavalle, MD, PhD, MSPH
Deputy Editor
Rosamaria Corona, MD, DSc


Benign pigmented skin lesions and melanocytic nevi (moles) are common in children and adolescents. Benign pigmented skin lesions, including lentigines, café-au-lait macules, Becker nevi, and dermal melanocytoses (Mongolian spots, nevus of Ota, and nevus of Ito), will be discussed below. Melanocytic nevi and melanocytic nevi variants are discussed separately. (See "Congenital melanocytic nevi" and "Acquired melanocytic nevi (moles)".)


Lentigines are benign pigmented macules that result from increased activity of epidermal melanocytes [1]. In contrast to the ephelides (freckles) that are commonly seen in lightly pigmented children and fade in the absence of sun exposure, lentigines are persistent. There are two major types of lentigines: simple lentigo and solar lentigo. The mucosal melanotic macule is a variant of simple lentigo that is located on mucosal surfaces, in particular the lower lip.

Simple lentigo — Simple lentigines often appear during childhood as sharply circumscribed, round-to-oval, uniformly brown or brownish-black macules that are usually <5 mm in diameter. There are typically few lesions, with no predilection for sun-exposed sites. However, multiple lentigines may be seen in a variety of disorders (table 1), and lentigines may increase in number or darken in patients with Addison's disease or other syndromes associated with elevated circulating levels of adrenocorticotropic hormone. (See "Causes and clinical manifestations of primary adrenal insufficiency in children".)

Mucosal melanotic macule — Mucosal melanotic macules, which most commonly develop on the vermilion portion of the lower lip, have a predilection for white adolescent girls and young women [2]. Mucosal melanotic macules may also occur on the oral mucosa and genitalia. Patients present with one or more brown to black macules, sometimes with irregular borders and mottled pigmentation. Genital lesions are occasionally >1 cm in diameter.

Multiple perioral and oral mucosal melanotic macules characterize disorders such as Peutz-Jeghers and Laugier-Hunziker syndromes. Multiple genital melanotic macules are a feature of Bannayan-Riley-Ruvalcaba syndrome (a type of PTEN hamartoma-tumor syndrome) (table 1). (See "Peutz-Jeghers syndrome: Epidemiology, clinical manifestations, and diagnosis".)


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Literature review current through: Sep 2016. | This topic last updated: Jul 28, 2014.
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