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Linear and whorled nevoid hypermelanosis(LWNH)

MedGen UID:
473394
Concept ID:
C1304501
Disease or Syndrome
Synonyms: LWNH; NEVOID HYPERMELANOSIS, LINEAR AND WHORLED
SNOMED CT: Linear and whorled nevoid hypermelanosis (403803002)
 
Monarch Initiative: MONDO:0013688
OMIM®: 614323
Orphanet: ORPHA79150

Definition

Linear and whorled hypermelanosis (LWNH) is a benign skin condition characterized by onset in infancy of hyperpigmented regions composed of small light brown spots that coalesce with age and follow the lines of Blaschko on the trunk and limbs. The soles, palms, face, and mucous membranes are spared. The lesions are asymptomatic and progress with age; affected individuals have no accompanying extradermal features. There is no previous history of inflammation on affected areas (summary by Kalter et al., 1988). [from OMIM]

Clinical features

From HPO
Eosinophilia
MedGen UID:
41824
Concept ID:
C0014457
Disease or Syndrome
Increased count of eosinophils in the blood.
Hyperpigmented streaks
MedGen UID:
400951
Concept ID:
C1866245
Finding

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLinear and whorled nevoid hypermelanosis
Follow this link to review classifications for Linear and whorled nevoid hypermelanosis in Orphanet.

Professional guidelines

Recent clinical studies

Etiology

Kromann AB, Ousager LB, Ali IKM, Aydemir N, Bygum A
Orphanet J Rare Dis 2018 Mar 5;13(1):39. doi: 10.1186/s13023-018-0778-6. PMID: 29506540Free PMC Article
Yuksek J, Sezer E, Erbil AH, Arca E, Taştan HB, Kurumlu Z, Demiriz M
Dermatol Online J 2007 Jul 13;13(3):23. PMID: 18328217
Di Lernia V
Pediatr Dermatol 2007 May-Jun;24(3):205-10. doi: 10.1111/j.1525-1470.2007.00387.x. PMID: 17542865
Alrobaee AA, Alsaif F
Int J Dermatol 2004 Feb;43(2):145-7. doi: 10.1111/j.1365-4632.2004.01855.x. PMID: 15125509
Kalter DC, Griffiths WA, Atherton DJ
J Am Acad Dermatol 1988 Dec;19(6):1037-44. doi: 10.1016/s0190-9622(88)70269-8. PMID: 3204178

Diagnosis

Naveen KN, Reshme P
Dermatol Online J 2014 Mar 17;20(3) PMID: 24656274
Yuksek J, Sezer E, Erbil AH, Arca E, Taştan HB, Kurumlu Z, Demiriz M
Dermatol Online J 2007 Jul 13;13(3):23. PMID: 18328217
Di Lernia V
Pediatr Dermatol 2007 May-Jun;24(3):205-10. doi: 10.1111/j.1525-1470.2007.00387.x. PMID: 17542865
Mendiratta V, Sharma RC, Arya L, Sardana K
J Dermatol 2001 Jan;28(1):58-9. doi: 10.1111/j.1346-8138.2001.tb00089.x. PMID: 11280469
Delaporte E, Janin A, Blondel V, Copin MC, Piette F, de Martinville B, Bergoend H
Dermatology 1996;192(1):70-2. doi: 10.1159/000246321. PMID: 8832959

Therapy

Shi Z, Duan X, Jiang M, Zhang C, Xiang L
J Cosmet Laser Ther 2022 Jul 4;24(1-5):56-59. Epub 2022 Apr 16 doi: 10.1080/14764172.2022.2066135. PMID: 35430930

Prognosis

Han HT, Park JJ, Lee JS, Lee SH
Acta Derm Venereol 2024 Aug 23;104:adv40565. doi: 10.2340/actadv.v104.40565. PMID: 39175455Free PMC Article
Cohen J 3rd, Shahrokh K, Cohen B
Pediatr Dermatol 2014 Jul-Aug;31(4):471-6. doi: 10.1111/pde.12346. PMID: 25039703
Yuksek J, Sezer E, Erbil AH, Arca E, Taştan HB, Kurumlu Z, Demiriz M
Dermatol Online J 2007 Jul 13;13(3):23. PMID: 18328217
Alrobaee AA, Alsaif F
Int J Dermatol 2004 Feb;43(2):145-7. doi: 10.1111/j.1365-4632.2004.01855.x. PMID: 15125509
Kalter DC, Griffiths WA, Atherton DJ
J Am Acad Dermatol 1988 Dec;19(6):1037-44. doi: 10.1016/s0190-9622(88)70269-8. PMID: 3204178

Clinical prediction guides

Shi Z, Duan X, Jiang M, Zhang C, Xiang L
J Cosmet Laser Ther 2022 Jul 4;24(1-5):56-59. Epub 2022 Apr 16 doi: 10.1080/14764172.2022.2066135. PMID: 35430930
Kromann AB, Ousager LB, Ali IKM, Aydemir N, Bygum A
Orphanet J Rare Dis 2018 Mar 5;13(1):39. doi: 10.1186/s13023-018-0778-6. PMID: 29506540Free PMC Article
Lu Y, Zhu WY
J Dermatol 2007 Nov;34(11):765-8. doi: 10.1111/j.1346-8138.2007.00380.x. PMID: 17973817
Delaporte E, Janin A, Blondel V, Copin MC, Piette F, de Martinville B, Bergoend H
Dermatology 1996;192(1):70-2. doi: 10.1159/000246321. PMID: 8832959
Akiyama M, Aranami A, Sasaki Y, Ebihara T, Sugiura M
J Am Acad Dermatol 1994 May;30(5 Pt 2):831-3. doi: 10.1016/s0190-9622(94)70090-7. PMID: 8169255

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