Laugier-Hunziker Syndrome
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Laugier-Hunziker syndrome (LHS) was initially described in 1970 as acquired, benign hyperpigmented macules of the lips and buccal mucosa frequently associated with longitudinal melanonychia. Extended mucocutaneous features have been observed since that original description, including macular pigmentation of the genitalia. No underlying systemic abnormalities are associated with Laugier-Hunziker syndrome, and no malignant predisposition exists. This lack of somatic abnormalities has moved some authorities to propose a name change to Laugier and Hunziker pigmentation. [1] When associated with nonclassic body locations or atypical features, the name idiopathic lenticular mucocutaneous hyperpigmentation has been used. [2]
The etiology of melanosis in Laugier-Hunziker syndrome is unknown. A lack of family members with Laugier-Hunziker syndrome is characteristic in most cases. To date, only one case of familial Laugier-Hunziker syndrome has been described, which involved a mother and 2 daughters. [3] Environmental risk factors have not been identified.
United States
The incidence is rare, but it is likely underreported. White patients and those of Latino descent have been described in the United States.
International
The prevalence appears to be higher in France and Italy when compared with the United Kingdom and the United States. Worldwide, approximately 60 cases were reported from 1970-1991. Among the patients described prior to 1989, 27 (93%) of 29 patients were from continental Europe. To date, more than 100 cases have been described. [4]
Laugier-Hunziker syndrome mainly affects whites; however, persons of Hispanic, Arabic, or Asian [5] descent have been described.
Idiopathic buccal melanosis without longitudinal melanonychia is a normal finding in 38% of black patients and in 5% of white patients. Physiologic melanoplakia, a term also used to describe idiopathic racial or ethnic melanosis, is most commonly noted on the gingiva of individuals with darker skin types. The histopathologic features of Laugier-Hunziker syndrome are indistinguishable from those of physiologic melanoplakia.
Longitudinal melanonychia (also known as melanonychia striata) without associated mucosal melanotic macules is a normal finding in 77% of blacks by age 20 years, and it is seen in 90% of blacks by the fifth decade of life. It most commonly occurs on the thumbs, with onset often during infancy or puberty.
Laugier-Hunziker syndrome was initially predominantly thought to affect females, with an estimated female-to-male ratio of 2:1. [6] However, the idea that Laugier-Hunziker syndrome is equally distributed between the sexes is gaining popularity. [4]
Essential melanotic pigmentation typically develops during early to middle adulthood, in persons aged 20-40 years, but it can occur as late as the sixth or seventh decade of life. A mean age of 52 years [7] and a median age of 42 years [8] have been reported.
Physiologic (racial or ethnic) melanosis characteristically occurs during the first 3 decades of life.
Peutz-Jeghers syndrome (PJS), a major differential diagnosis of Laugier-Hunziker syndrome, usually has its onset at birth or during the first few years of life. However, sporadic cases of PJS have been reported in as many as 40% of cases, and it can have a late onset. See Peutz-Jeghers Syndrome for more information on this syndrome.
Laugier-Hunziker syndrome has a benign course. One report has described spontaneous remission. [9] Systemic illness and malignancy are not features of Laugier-Hunziker syndrome. Such findings in association with mucocutaneous melanotic hyperpigmentation exclude the diagnosis of Laugier-Hunziker syndrome.
Moore RT, Chae KA, Rhodes AR. Laugier and Hunziker pigmentation: a lentiginous proliferation of melanocytes. J Am Acad Dermatol. 2004 May. 50(5 Suppl):S70-4. [Medline].
Gerbig AW, Hunziker T. Idiopathic lenticular mucocutaneous pigmentation or Laugier-Hunziker syndrome with atypical features. Arch Dermatol. 1996 Jul. 132(7):844-5. [Medline].
Makhoul EN, Ayoub NM, Helou JF, Abadjian GA. Familial Laugier-Hunziker syndrome. J Am Acad Dermatol. 2003 Aug. 49(2 Suppl Case Reports):S143-5. [Medline].
Lampe AK, Hampton PJ, Woodford-Richens K, Tomlinson I, Lawrence CM, Douglas FS. Laugier-Hunziker syndrome: an important differential diagnosis for Peutz-Jeghers syndrome. J Med Genet. 2003 Jun. 40(6):e77. [Medline].
Tan J, Greaves MW, Lee LH. Laugier-Hunziker syndrome and hypocellular marrow: a fortuitous association?. Clin Exp Dermatol. 2007 Sep. 32(5):584-5. [Medline].
Nikitakis NG, Koumaki D. Laugier-Hunziker syndrome: case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013 Jul. 116 (1):e52-8. [Medline].
Siponen M, Salo T. Idiopathic lenticular mucocutaneous pigmentation (Laugier-Hunziker syndrome): a report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003 Sep. 96(3):288-92. [Medline].
Lenane P, Sullivan DO, Keane CO, Loughlint SO. The Laugier-Hunziker syndrome. J Eur Acad Dermatol Venereol. 2001 Nov. 15(6):574-7. [Medline].
Ferreira MJ, Ferreira AM, Soares AP, Rodrigues JC. Laugier-Hunziker syndrome: case report and treatment with the Q-switched Nd-Yag laser. J Eur Acad Dermatol Venereol. 1999 Mar. 12(2):171-3. [Medline].
Buchner A, Hansen LS. Melanotic macule of the oral mucosa. A clinicopathologic study of 105 cases. Oral Surg Oral Med Oral Pathol. 1979 Sep. 48(3):244-9. [Medline].
Lamey PJ, Nolan A, Thomson E, Lewis MA, Rademaker M. Oral presentation of the Laugier-Hunziker syndrome. Br Dent J. 1991 Jul 20. 171(2):59-60. [Medline].
Mowad CM, Shrager J, Elenitsas R. Oral pigmentation representing Laugier-Hunziker syndrome. Cutis. 1997 Jul. 60(1):37-9. [Medline].
Yago K, Tanaka T, Asanami S. Laugier-Hunziker Baran Syndrome. Journal Oral Surgery Oral Medicine Oral Pathology Oral Radiology & Endodontics. August. 106:e20=25.
Baran R. Longitudinal melanotic streaks as a clue to Laugier-Hunziker syndrome. Arch Dermatol. 1979 Dec. 115(12):1448-9. [Medline].
Baran R, Barriere H. Longitudinal melanonychia with spreading pigmentation in Laugier-Hunziker syndrome: a report of two cases. Br J Dermatol. 1986 Dec. 115(6):707-10. [Medline].
Sterling GB, Libow LF, Grossman ME. Pigmented nail streaks may indicate Laugier-Hunziker syndrome. Cutis. 1988 Oct. 42(4):325-6. [Medline].
Porneuf M, Dandurand M. Pseudo-melanoma revealing Laugier-Hunziker syndrome. Int J Dermatol. 1997 Feb. 36(2):138-41. [Medline].
Sabesan T, Ramchandani PL, Peters WJ. Laugier-Hunziker syndrome: a rare cause of mucocutaneous pigmentation. Br J Oral Maxillofac Surg. 2006 Aug. 44(4):320-1. [Medline].
Yamamoto O, Yoshinaga K, Asahi M, Murata I. A Laugier-Hunziker syndrome associated with esophageal melanocytosis. Dermatology. 1999. 199(2):162-4. [Medline].
Kanwar AJ, Kaur S, Kaur C, Thami GP. Laugier-Hunziker syndrome. J Dermatol. 2001 Jan. 28(1):54-7. [Medline].
Gencoglan G, Gerceker-Turk B, Kilinc-Karaarslan I, Akalin T, Ozdemir F. Dermoscopic findings in Laugier-Hunziker syndrome. Arch Dermatol. 2007 May. 143(5):631-3. [Medline].
Sendagorta E, Feito M, Ramírez P, Gonzalez-Beato M, Saida T, Pizarro A. Dermoscopic findings and histological correlation of the acral volar pigmented maculae in Laugier-Hunziker syndrome. J Dermatol. 2010 Nov. 37(11):980-4. [Medline].
Ozawa T, Fujiwara M, Harada T, Muraoka M, Ishii M. Q-switched alexandrite laser therapy for pigmentation of the lips owing to Laugier-Hunziker syndrome. Dermatol Surg. 2005 Jun. 31(6):709-12. [Medline].
Papadavid E, Walker NP. Q-switched Alexandrite laser in the treatment of pigmented macules in Laugier-Hunziker syndrome. J Eur Acad Dermatol Venereol. 2001 Sep. 15(5):468-9. [Medline].
Zuo YG, Ma DL, Jin HZ, Liu YH, Wang HW, Sun QN. Treatment of Laugier-Hunziker syndrome with the Q-switched alexandrite laser in 22 Chinese patients. Arch Dermatol Res. 2010 Mar. 302(2):125-30. [Medline].
Sheridan AT, Dawber RP. Laugier-Hunziker syndrome: treatment with cryosurgery. J Eur Acad Dermatol Venereol. 1999 Sep. 13(2):146-8. [Medline].
Christen M Mowad, MD Professor, Department of Dermatology, Geisinger Medical Center
Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, Noah Worcester Dermatological Society, Pennsylvania Academy of Dermatology, American Academy of Dermatology, Phi Beta Kappa
Disclosure: Nothing to disclose.
Lauren N Taglia, MD, PhD Resident Physician, Department of Dermatology, Geisinger Medical Center
Disclosure: Nothing to disclose.
Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society
Disclosure: Nothing to disclose.
Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology
Disclosure: Received income in an amount equal to or greater than $250 from: Lilly; Amgen <br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Allergen; Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.
Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.
Günter Burg, MD Professor and Chairman Emeritus, Department of Dermatology, University of Zürich School of Medicine; Delegate of The Foundation for Modern Teaching and Learning in Medicine Faculty of Medicine, University of Zürich, Switzerland
Günter Burg, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, International Society for Dermatologic Surgery, North American Clinical Dermatologic Society, and Pacific Dermatologic Association
Disclosure: Nothing to disclose.
Lindsey Ann Dohse, MD Resident Physician, Department of Dermatology, Geisinger Health System
Lindsey Ann Dohse, MD is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Nothing to disclose.
Michelle Pelle, MD Clinical Assistant Professor, Division of Dermatology, Department of Medicine, University of California, San Diego, School of Medicine
Michelle Pelle, MD is a member of the following medical societies: American Academy of Dermatology, California Medical Association, Medical Dermatology Society, and Pennsylvania Medical Society
Disclosure: Nothing to disclose.
Lindsay Dane Sewell, MD Resident Physician, Department of Dermatology, Geisinger Medical Center
Lindsay Dane Sewell, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.
Laugier-Hunziker Syndrome
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