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Nevus spilus

MedGen UID:
Concept ID:
Neoplastic Process
SNOMED CT: Nevus spilus (398660000); Speckled lentiginous nevus (398660000)
HPO: HP:0025510
OMIM®: 137550


A tan, regularly bordered patch with darker macules within the lesion. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNevus spilus

Conditions with this feature

Large congenital melanocytic nevus
MedGen UID:
Concept ID:
Neoplastic Process
Congenital melanocytic nevus syndrome is characterized by pigmentary skin defects apparent at birth. Most individuals have 1 or more large or giant lesions greater than 20 cm and up to over 60 cm in diameter, which may cover up to 80% of total body area. These lesions may or may not be hairy. Smaller 'satellite' pigmented lesions numbering in the hundreds may also be present all over the body. Congenital melanocytic nevi (CMN) can be associated with malignant melanoma (see CMM1, 155600), but the risk appears to be low, ranging from 1 to 2% for all individuals, but rising to 10 to 15% in those with very large nevi (greater than 40 cm). A small subset of patients with CMNS have abnormalities of the central nervous system, known as 'neurocutaneous melanosis' or 'neuromelanosis' (249400), which may be symptomatic. Patients with CMNS also tend to have a characteristic facial appearance, including wide or prominent forehead, periorbital fullness, small short nose with narrow nasal bridge, round face, full cheeks, prominent premaxilla, and everted lower lip (summary by Kinsler et al., 2008; Kinsler et al., 2012). Spitz nevi are benign melanocytic melanomas composed of epithelioid or spindle cell melanocytes. They usually present as solitary skin tumors but can occur in multiple patterns, having agminated, dermatomal, and disseminated forms (summary by Sarin et al., 2013). Nevus spilus, also known as speckled lentiginous nevus, is a congenital hyperpigmented patch that progressively evolves, with affected individuals developing dark macules and papules during childhood and adolescence. Over time, nevus spilus may give rise to common lentigines, melanocytic nevi, Spitz nevi, and melanomas (summary by Sarin et al., 2014).
Glycosylphosphatidylinositol biosynthesis defect 17
MedGen UID:
Concept ID:
Disease or Syndrome
Glycosylphosphatidylinositol biosynthesis defect-17 (GPIBD17) is an autosomal recessive disorder characterized by variable neurologic deficits that become apparent in infancy or early childhood. Patients may present with early-onset febrile or afebrile seizures that tend to be mild or controllable. Other features may include learning disabilities, autism, behavioral abnormalities, hypotonia, and motor deficits. The phenotype is relatively mild compared to that of other GPIBDs (summary by Nguyen et al., 2018). For a discussion of genetic heterogeneity of GPI biosynthesis defects, see GPIBD1 (610293).

Professional guidelines


Farabi B, Khan S, Jamgochian M, Atak MF, Jain M, Rao BK
J Cosmet Dermatol 2023 Dec;22(12):3213-3222. Epub 2023 Sep 27 doi: 10.1111/jocd.15827. PMID: 37759421
Chan HH, Kono T
Skinmed 2003 Mar-Apr;2(2):89-96; quiz 97-8. doi: 10.1111/j.1540-9740.2003.01706.x. PMID: 14673306
Van Leeuwen RL, Bastiaens MT, Grevelink JM
Pediatr Dermatol 1997 Mar-Apr;14(2):155-6. doi: 10.1111/j.1525-1470.1997.tb00227.x. PMID: 9144707

Recent clinical studies


Polubothu S, Bender N, Muthiah S, Zecchin D, Demetriou C, Martin SB, Malhotra S, Travnickova J, Zeng Z, Böhm M, Barbarot S, Cottrell C, Davies O, Baselga E, Burrows NP, Carmignac V, Diaz JS, Fink C, Haenssle HA, Happle R, Harland M, Majerowski J, Vabres P, Vincent M, Newton-Bishop JA, Bishop DT, Siegel D, Patton EE, Topf M, Rajan N, Drolet B, Kinsler VA
J Invest Dermatol 2023 Jun;143(6):1042-1051.e3. Epub 2022 Dec 23 doi: 10.1016/j.jid.2022.09.661. PMID: 36566878Free PMC Article
Torchia D, Happle R
J Am Acad Dermatol 2023 Oct;89(4):764-773. Epub 2021 Feb 12 doi: 10.1016/j.jaad.2020.12.082. PMID: 33583608
Meguerditchian AN, Cheney RT, Kane JM 3rd
J Cutan Med Surg 2009 Mar-Apr;13(2):96-101. doi: 10.2310/7750.2008.07090. PMID: 19298778
Yoneyama K, Kamada N, Mizoguchi M, Utani A, Kobayashi T, Shinkai H
J Dermatol 2005 Jun;32(6):454-8. doi: 10.1111/j.1346-8138.2005.tb00779.x. PMID: 16043919
Happle R
Arch Dermatol 2005 Mar;141(3):385-8. doi: 10.1001/archderm.141.3.385. PMID: 15781681


Zhang J, Xu Q, Deng D
Clin Genet 2023 Nov;104(5):593-597. Epub 2023 Jul 27 doi: 10.1111/cge.14402. PMID: 37501405
Habeshian KA, Kirkorian AY
Neoreviews 2021 Oct;22(10):e660-e672. doi: 10.1542/neo.22-10-e660. PMID: 34599064
Torchia D, Happle R
Eur J Dermatol 2019 Feb 1;29(1):2-5. doi: 10.1684/ejd.2018.3488. PMID: 30827948
Vaidya DC, Schwartz RA, Janniger CK
Cutis 2007 Dec;80(6):465-8. PMID: 18246877
Cohen HJ, Minkin W, Frank SB
Arch Dermatol 1970 Oct;102(4):433-7. PMID: 5470953


Abrusci V, Benzecry V
Dermatol Ther 2017 Jul;30(4) Epub 2017 Apr 20 doi: 10.1111/dth.12497. PMID: 28425593
Bukvić Mokos Z, Lipozenčić J, Ceović R, Stulhofer Buzina D, Kostović K
Acta Dermatovenerol Croat 2010;18(3):185-9. PMID: 20887701
Isago T, Kono T, Nozaki M, Kikuchi Y, Honda T, Nakazawa H
Surg Today 2006;36(9):765-8. doi: 10.1007/s00595-004-3230-6. PMID: 16937277
Carpo BG, Grevelink JM, Grevelink SV
Semin Cutan Med Surg 1999 Sep;18(3):233-43. doi: 10.1016/s1085-5629(99)80021-2. PMID: 10468043
Grevelink JM, González S, Bonoan R, Vibhagool C, Gonzalez E
Dermatol Surg 1997 May;23(5):365-9; discussion 369-70. doi: 10.1111/j.1524-4725.1997.tb00062.x. PMID: 9179247


Shih S, Dai C, Shih T, Khachemoune A
J Drugs Dermatol 2020 May 1;19(5):471-474. PMID: 32484627
Chiu HH, Chen GS, Wu CS, Ke CL, Cheng ST
Int J Dermatol 2009 Apr;48(4):416-8. doi: 10.1111/j.1365-4632.2009.03885.x. PMID: 19335431
Yoneyama K, Kamada N, Mizoguchi M, Utani A, Kobayashi T, Shinkai H
J Dermatol 2005 Jun;32(6):454-8. doi: 10.1111/j.1346-8138.2005.tb00779.x. PMID: 16043919
Happle R
Arch Dermatol 2005 Mar;141(3):385-8. doi: 10.1001/archderm.141.3.385. PMID: 15781681
Trattner A, Metzker A
J Am Acad Dermatol 1993 Nov;29(5 Pt 1):693-5. doi: 10.1016/0190-9622(93)70232-i. PMID: 8227540

Clinical prediction guides

Abrusci V, Benzecry V
Dermatol Ther 2017 Jul;30(4) Epub 2017 Apr 20 doi: 10.1111/dth.12497. PMID: 28425593
Sarin KY, Sun BK, Bangs CD, Cherry A, Swetter SM, Kim J, Khavari PA
JAMA Dermatol 2013 Sep;149(9):1077-81. doi: 10.1001/jamadermatol.2013.4745. PMID: 23884457
Corradin MT, Zattra E, Fiorentino R, Alaibac M, Belloni-Fortina A
J Cutan Med Surg 2010 Mar-Apr;14(2):85-9. doi: 10.2310/7750.2009.08090. PMID: 20338124
Grinspan D, Casala A, Abulafia J, Mascotto J, Allevato M
Int J Dermatol 1997 Jul;36(7):499-502. doi: 10.1046/j.1365-4362.1997.00076.x. PMID: 9268745
Hwang SM, Choi EH, Lee WS, Choi SI, Ahn SK
Am J Dermatopathol 1997 Jun;19(3):308-11. doi: 10.1097/00000372-199706000-00020. PMID: 9185922

Recent systematic reviews

Meyer SN, Simmons E, Studer AC, Rauen KA, Kiuru M
Melanoma Res 2023 Dec 1;33(6):437-446. Epub 2023 Aug 14 doi: 10.1097/CMR.0000000000000912. PMID: 37578532Free PMC Article

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