Official reprint from UpToDate®
www.uptodate.com ©2018 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

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 often 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 congenital 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 "Congenital and inherited hyperpigmentation disorders", section on 'Genetic syndromes associated with lentiginosis'.)

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Dec 2017. | This topic last updated: Mar 06, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2018 UpToDate, Inc.
  1. Ber Rahman S, Bhawan J. Lentigo. Int J Dermatol 1996; 35:229.
  2. Gupta G, Williams RE, Mackie RM. The labial melanotic macule: a review of 79 cases. Br J Dermatol 1997; 136:772.
  3. HODGSON C. Senile lentigo. Arch Dermatol 1963; 87:197.
  4. Bastiaens M, ter Huurne J, Gruis N, et al. The melanocortin-1-receptor gene is the major freckle gene. Hum Mol Genet 2001; 10:1701.
  5. Landau M, Krafchik BR. The diagnostic value of café-au-lait macules. J Am Acad Dermatol 1999; 40:877.
  6. Boyd KP, Gao L, Feng R, et al. Phenotypic variability among café-au-lait macules in neurofibromatosis type 1. J Am Acad Dermatol 2010; 63:440.
  7. Ahn JS, Kim SD, Hwang JH, et al. Halo-like disappearance of mongolian spot combined with café au lait spot. Pediatr Dermatol 1998; 15:70.
  8. Korf BR. Diagnostic outcome in children with multiple café au lait spots. Pediatrics 1992; 90:924.
  9. Nunley KS, Gao F, Albers AC, et al. Predictive value of café au lait macules at initial consultation in the diagnosis of neurofibromatosis type 1. Arch Dermatol 2009; 145:883.
  10. Pasmant E, Sabbagh A, Hanna N, et al. SPRED1 germline mutations caused a neurofibromatosis type 1 overlapping phenotype. J Med Genet 2009; 46:425.
  11. Arnsmeier SL, Riccardi VM, Paller AS. Familial multiple cafe au lait spots. Arch Dermatol 1994; 130:1425.
  12. Charrow J, Listernick R, Ward K. Autosomal dominant multiple café-au-lait spots and neurofibromatosis-1: evidence of non-linkage. Am J Med Genet 1993; 45:606.
  13. Abeliovich D, Gelman-Kohan Z, Silverstein S, et al. Familial café au lait spots: a variant of neurofibromatosis type 1. J Med Genet 1995; 32:985.
  14. Williams VC, Lucas J, Babcock MA, et al. Neurofibromatosis type 1 revisited. Pediatrics 2009; 123:124.
  15. Person JR, Longcope C. Becker's nevus: an androgen-mediated hyperplasia with increased androgen receptors. J Am Acad Dermatol 1984; 10:235.
  16. Tymen R, Forestier JF, Boutet B, Colomb D. [Late Becker's nevus. One hundred cases (author's transl)]. Ann Dermatol Venereol 1981; 108:41.
  17. Hori Y, Takayama O. Circumscribed dermal melanoses. Classification and histologic features. Dermatol Clin 1988; 6:315.
  18. Van Raamsdonk CD, Griewank KG, Crosby MB, et al. Mutations in GNA11 in uveal melanoma. N Engl J Med 2010; 363:2191.
  19. Shaffer D, Walker K, Weiss GR. Malignant melanoma in a Hispanic male with nevus of Ota. Dermatology 1992; 185:146.
  20. Shields CL, Kaliki S, Livesey M, et al. Association of ocular and oculodermal melanocytosis with the rate of uveal melanoma metastasis: analysis of 7872 consecutive eyes. JAMA Ophthalmol 2013; 131:993.
  21. Watanabe S, Takahashi H. Treatment of nevus of Ota with the Q-switched ruby laser. N Engl J Med 1994; 331:1745.