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White sponge nevus 2(WSN2)

MedGen UID:
862758
Concept ID:
C4014321
Disease or Syndrome
Synonym: WSN2
 
Gene (location): KRT13 (17q21.2)
 
Monarch Initiative: MONDO:0014346
OMIM®: 615785

Definition

White sponge nevus can be present from birth but usually first appears during early childhood. The size and location of the nevi can change over time. In the oral mucosa, both sides of the mouth are usually affected. The nevi are generally painless, but the folds of extra tissue can promote bacterial growth, which can lead to infection that may cause discomfort. The altered texture and appearance of the affected tissue, especially the oral mucosa, can be bothersome for some affected individuals.

White sponge nevus is a condition characterized by the formation of white patches of tissue called nevi (singular: nevus) that appear as thickened, velvety, sponge-like tissue. The nevi are most commonly found on the moist lining of the mouth (oral mucosa), especially on the inside of the cheeks (buccal mucosa). Affected individuals usually develop multiple nevi. Rarely, white sponge nevi also occur on the mucosae (singular: mucosa) of the nose, esophagus, genitals, or anus. The nevi are caused by a noncancerous (benign) overgrowth of cells. [from MedlinePlus Genetics]

Clinical features

From HPO
Edema
MedGen UID:
4451
Concept ID:
C0013604
Pathologic Function
An abnormal accumulation of fluid beneath the skin, or in one or more cavities of the body.
Epidermal acanthosis
MedGen UID:
65136
Concept ID:
C0221270
Finding
Diffuse hypertrophy or thickening of the stratum spinosum of the epidermis (prickle cell layer of the skin).
Hyperparakeratosis
MedGen UID:
473366
Concept ID:
C1265968
Finding
Abnormal keratinization of the epidermal stratum coreum (horny layer) with increased keratin formation, preservation of the nuclei in the superficial cells, and absence of the stratum granulosum.

Recent clinical studies

Etiology

Garijo MF, Val D, Val-Bernal JF
Am J Dermatopathol 2001 Aug;23(4):329-33. doi: 10.1097/00000372-200108000-00010. PMID: 11481526
Yang JM, Nam K, Kim SW, Jung SY, Min HG, Yeo UC, Park KB, Lee JH, Suhr KB, Park JK, Lee ES
J Dermatol Sci 1999 Feb;19(2):126-33. doi: 10.1016/s0923-1811(98)00055-3. PMID: 10098704
Corden LD, McLean WH
Exp Dermatol 1996 Dec;5(6):297-307. doi: 10.1111/j.1600-0625.1996.tb00133.x. PMID: 9028791
Su L, Morgan PR, Thomas JA, Lane EB
J Oral Pathol Med 1993 Apr;22(4):183-9. doi: 10.1111/j.1600-0714.1993.tb01054.x. PMID: 7686226

Diagnosis

Westin M, Rekabdar E, Blomstrand L, Klintberg P, Jontell M, Robledo-Sierra J
J Oral Pathol Med 2018 Feb;47(2):152-157. Epub 2017 Nov 1 doi: 10.1111/jop.12652. PMID: 29047160
Pinto A, Haberland CM, Baker S
Dent Clin North Am 2014 Apr;58(2):437-53. Epub 2014 Jan 31 doi: 10.1016/j.cden.2013.12.003. PMID: 24655531
Haisley-Royster CA, Allingham RR, Klintworth GK, Prose NS
J Am Acad Dermatol 2001 Oct;45(4):634-6. doi: 10.1067/mjd.2001.116336. PMID: 11568764
Miller CS, Craig RM Jr
J Am Dent Assoc 1988 Aug;117(2):345-6. doi: 10.1016/s0002-8177(88)72021-8. PMID: 3166478
Jorgenson RJ, Levin S
Arch Dermatol 1981 Feb;117(2):73-6. PMID: 7469444

Therapy

Marrelli M, Tatullo M, Dipalma G, Inchingolo F
Int J Med Sci 2012;9(1):47-50. Epub 2011 Nov 18 doi: 10.7150/ijms.9.47. PMID: 22211089Free PMC Article
Su L, Morgan PR, Thomas JA, Lane EB
J Oral Pathol Med 1993 Apr;22(4):183-9. doi: 10.1111/j.1600-0714.1993.tb01054.x. PMID: 7686226

Prognosis

Marrelli M, Tatullo M, Dipalma G, Inchingolo F
Int J Med Sci 2012;9(1):47-50. Epub 2011 Nov 18 doi: 10.7150/ijms.9.47. PMID: 22211089Free PMC Article

Clinical prediction guides

Zhang J, Quan J, Ren Y, Chen M, Yang J, Zhang X
J Oral Pathol Med 2018 Jul;47(6):598-605. Epub 2018 May 27 doi: 10.1111/jop.12728. PMID: 29738605
Kürklü E, Öztürk Ş, Cassidy AJ, Ak G, Koray M, Çefle K, Palandüz Ş, Güllüoğlu MG, Tanyeri H, McLean WH
Med Oral Patol Oral Cir Bucal 2018 Mar 1;23(2):e144-e150. doi: 10.4317/medoral.21437. PMID: 29476668Free PMC Article
McGininis JP Jr, Turner JE
Oral Surg Oral Med Oral Pathol 1975 Nov;40(5):644-51. doi: 10.1016/0030-4220(75)90374-6. PMID: 52856

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