U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Blue nevus

MedGen UID:
104930
•Concept ID:
C0206736
•
Neoplastic Process
Synonym: Jadassohn's blue nevus
SNOMED CT: Blue nevus (63166000); Jadassohn's blue nevus (63166000); Dermal melanocytoma (63166000); Blue nevus of skin (254806009)
 
HPO: HP:0100814
Monarch Initiative: MONDO:0006680

Definition

A solitary, bluish, smooth surfaced macule, papule or plaque that is generally round or oval in shape. The histopathology of blue nevi varies by subtype, but general characteristics include a vertical wedge or bulbous shaped proliferation of spindle cells, dendritic melanocytes, and melanophages into a sclerotic dermis or subcutis. [from HPO]

Conditions with this feature

Intellectual disability, autosomal dominant 53
MedGen UID:
1623344
•Concept ID:
C4540481
•
Mental or Behavioral Dysfunction
See: Condition Record
Suleiman-El-Hattab syndrome
MedGen UID:
1738652
•Concept ID:
C5436458
•
Disease or Syndrome
Suleiman-El-Hattab syndrome (SULEHS) is an autosomal recessive multisystem developmental disorder characterized by hypotonia and feeding difficulties soon after birth, global developmental delay with impaired intellectual development and poor expressive speech, and a general happy demeanor. There is a distinctive facial appearance with microcephaly, thick arched eyebrows with synophrys, hypertelorism, epicanthal folds, low-set ears, broad nasal bridge, and thin upper lip. Additional more variable features include recurrent respiratory infections, cardiovascular malformations, cryptorchidism, seizures, and distal anomalies of the hands and feet (summary by Suleiman et al., 2019).
See: Condition Record
Liver disease, severe congenital
MedGen UID:
1823968
•Concept ID:
C5774195
•
Disease or Syndrome
Severe congenital liver disease (SCOLIV) is an autosomal recessive disorder characterized by the onset of progressive hepatic dysfunction usually in the first years of life. Affected individuals show feeding difficulties with failure to thrive and features such as jaundice, hepatomegaly, and abdominal distension. Laboratory workup is consistent with hepatic insufficiency and may also show coagulation defects, anemia, or metabolic disturbances. Cirrhosis and hypernodularity are commonly observed on liver biopsy. Many patients die of liver failure in early childhood (Moreno Traspas et al., 2022).
See: Condition Record
Intellectual disability, autosomal dominant 53
Liver disease, severe congenital
Suleiman-El-Hattab syndrome

Professional guidelines

PubMed

Clinical features and management of eyelid margin nevi.
Custer PL, Council ML
Orbit 2024 Feb;43(1):8-15. Epub 2023 Jan 23 doi: 10.1080/01676830.2023.2169717. PMID: 36688501
Blue nevus: classical types and new related entities. A differential diagnostic review.
González-Cámpora R, Galera-Davidson H, Vázquez-Ramírez FJ, Díaz-Cano S
Pathol Res Pract 1994 Jun;190(6):627-35. doi: 10.1016/S0344-0338(11)80402-4. PMID: 7984522
Differential diagnosis in uropathology. III. Melanotic lesions of prostate: blue nevus and prostatic epithelial melanosis.
Tannenbaum M
Urology 1974 Nov;4(5):617-21. doi: 10.1016/0090-4295(74)90508-1. PMID: 4428566

Recent clinical studies

Etiology

Blue naevi and the blue tumour spectrum.
de la Fouchardiere A
Pathology 2023 Mar;55(2):187-195. Epub 2022 Dec 28 doi: 10.1016/j.pathol.2022.12.342. PMID: 36641378
Attempting to Solve the Pigmented Epithelioid Melanocytoma (PEM) Conundrum: PRKAR1A Inactivation Can Occur in Different Genetic Backgrounds (Common, Blue, and Spitz Subgroups) With Variation in Their Clinicopathologic Characteristics.
de la Fouchardiere A, Tirode F, Castillo C, Buisson A, Boivin F, Macagno N, Pissaloux D
Am J Surg Pathol 2022 Aug 1;46(8):1106-1115. Epub 2022 Mar 22 doi: 10.1097/PAS.0000000000001888. PMID: 35319526
Soft-tissue vascular malformations and tumors. Part 2: low-flow lesions.
Flors L, Hagspiel KD, Park AW, Norton PT, Leiva-Salinas C
Radiologia (Engl Ed) 2019 Mar-Apr;61(2):124-133. Epub 2018 Oct 3 doi: 10.1016/j.rx.2018.02.012. PMID: 30292466
Blue nevus and "malignant blue nevus:" A concise review.
Sugianto JZ, Ralston JS, Metcalf JS, McFaddin CL, Smith MT
Semin Diagn Pathol 2016 Jul;33(4):219-24. Epub 2016 Apr 19 doi: 10.1053/j.semdp.2016.04.006. PMID: 27199078
Unusual melanoma types.
Rivers JK
Dermatol Clin 2002 Oct;20(4):727-33, x. doi: 10.1016/s0733-8635(02)00036-0. PMID: 12380060

Diagnosis

Blue Rubber Bleb Nevus Syndrome.
Gu Z, Ma DL
Mayo Clin Proc 2024 Jun;99(6):1013-1014. Epub 2024 May 6 doi: 10.1016/j.mayocp.2024.02.002. PMID: 38713129
A skin-colored sacral papule.
Ng A, Wanat KA, Chiu YE
Pediatr Dermatol 2021 Sep;38(5):1312-1314. doi: 10.1111/pde.14753. PMID: 34750879
Successful endoscopic diagnosis and treatment of blue rubber bleb nevus syndrome.
Tang XY, He CH, Liao Z
Endoscopy 2021 Apr;53(4):E118-E119. Epub 2020 Jul 24 doi: 10.1055/a-1202-0716. PMID: 32707586
Phakomatosis pigmentovascularis.
Van Gysel D, Oranje AP, Stroink H, Simonsz HJ
Pediatr Dermatol 1996 Jan-Feb;13(1):33-5. doi: 10.1111/j.1525-1470.1996.tb01184.x. PMID: 8919522
Cervical melanosis.
Deppisch LM
Obstet Gynecol 1983 Oct;62(4):525-6. PMID: 6888833

Therapy

An Unusual Cause of Recurrent Melena.
Li A, Chen FX, Li YQ
Gastroenterology 2019 Aug;157(2):311-312. Epub 2019 Apr 11 doi: 10.1053/j.gastro.2019.04.009. PMID: 30981790
Gastrointestinal: Endoscopic injection sclerotherapy for duodenal vascular malformation in blue rubber bleb nevus syndrome.
Kumei T, Toya Y, Shiohata T, Kakuta F, Yanai S, Kawasaki K, Nakamura S, Matsumoto T
J Gastroenterol Hepatol 2019 Jun;34(6):963. Epub 2019 Jan 14 doi: 10.1111/jgh.14590. PMID: 30638279
Sirolimus in blue rubber bleb naevus syndrome: A systematic review.
Wong XL, Phan K, Rodríguez Bandera AI, Sebaratnam DF
J Paediatr Child Health 2019 Feb;55(2):152-155. Epub 2018 Dec 18 doi: 10.1111/jpc.14345. PMID: 30565378
Malignant blue nevus arising in a giant congenital cellular blue nevus in an infant.
Nakamura Y, Shibata-Ito M, Nakamura Y, Ishitsuka Y, Fujisawa Y, Kawachi Y, Otsuka F
Pediatr Dermatol 2012 Sep-Oct;29(5):651-5. Epub 2012 Jan 26 doi: 10.1111/j.1525-1470.2011.01662.x. PMID: 22277073
Dermatoses of the nipple.
Parlakgumus A, Yildirim S, Bolat FA, Caliskan K, Ezer A, Colakoglu T, Moray G
Can J Surg 2009 Apr;52(2):160-1. PMID: 19399214Free PMC Article

Prognosis

Blue nevus-like melanoma of the uterine cervix. Case report and review of the literature.
Eniu DT, Staicu A, Şomcutian O, Buiga R, Albu C, Goidescu IG, Chiorean AR, Nistor-Ciurba CC
Rom J Morphol Embryol 2019;60(4):1317-1321. PMID: 32239111
The color of skin: black diseases of the skin, nails, and mucosa.
Qiu CC, Brown AE, Lobitz GR, Shanker A, Hsu S
Clin Dermatol 2019 Sep-Oct;37(5):447-467. Epub 2019 Aug 17 doi: 10.1016/j.clindermatol.2019.08.003. PMID: 31896402
Blue nevus-like and blue nevus-associated melanoma: a comprehensive review of the literature.
Borgenvik TL, Karlsvik TM, Ray S, Fawzy M, James N
ANZ J Surg 2017 May;87(5):345-349. Epub 2017 Mar 20 doi: 10.1111/ans.13946. PMID: 28318130
Unusual melanoma types.
Rivers JK
Dermatol Clin 2002 Oct;20(4):727-33, x. doi: 10.1016/s0733-8635(02)00036-0. PMID: 12380060
Plaque-type blue nevus.
Pittman JL, Fisher BK
Arch Dermatol 1976 Aug;112(8):1127-8. PMID: 952532

Clinical prediction guides

Clinical characteristics of blue rubber bleb nevus syndrome in adults: systematic scoping review.
Kozai L, Nishimura Y
Scand J Gastroenterol 2023 Jul-Dec;58(10):1108-1114. Epub 2023 May 21 doi: 10.1080/00365521.2023.2214263. PMID: 37211745
Blue naevi and the blue tumour spectrum.
de la Fouchardiere A
Pathology 2023 Mar;55(2):187-195. Epub 2022 Dec 28 doi: 10.1016/j.pathol.2022.12.342. PMID: 36641378
Attempting to Solve the Pigmented Epithelioid Melanocytoma (PEM) Conundrum: PRKAR1A Inactivation Can Occur in Different Genetic Backgrounds (Common, Blue, and Spitz Subgroups) With Variation in Their Clinicopathologic Characteristics.
de la Fouchardiere A, Tirode F, Castillo C, Buisson A, Boivin F, Macagno N, Pissaloux D
Am J Surg Pathol 2022 Aug 1;46(8):1106-1115. Epub 2022 Mar 22 doi: 10.1097/PAS.0000000000001888. PMID: 35319526
Blue nevus-like and blue nevus-associated melanoma: a comprehensive review of the literature.
Borgenvik TL, Karlsvik TM, Ray S, Fawzy M, James N
ANZ J Surg 2017 May;87(5):345-349. Epub 2017 Mar 20 doi: 10.1111/ans.13946. PMID: 28318130
Lentigo Maligna Melanoma With Local and Distant Blue Nevus-like Metastases.
Baird DS, Ioffreda MD, Helm K, Chung CG, Ferguson S
Am J Dermatopathol 2015 Oct;37(10):e126-8. doi: 10.1097/DAD.0000000000000292. PMID: 25943243

Recent systematic reviews

Treatment options for gastrointestinal bleeding blue rubber bleb nevus syndrome: Systematic review.
Rimondi A, Sorge A, Murino A, Nandi N, Scaramella L, Vecchi M, Tontini GE, Elli L
Dig Endosc 2024 Feb;36(2):162-171. Epub 2023 May 9 doi: 10.1111/den.14564. PMID: 37029779
Sirolimus in blue rubber bleb naevus syndrome: A systematic review.
Wong XL, Phan K, Rodríguez Bandera AI, Sebaratnam DF
J Paediatr Child Health 2019 Feb;55(2):152-155. Epub 2018 Dec 18 doi: 10.1111/jpc.14345. PMID: 30565378

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...