eMedicine Specialties > Dermatology > Diseases of Pigmentation

Laugier-Hunziker Syndrome

Author: Christen M Mowad, MD, Assistant Professor, Department of Dermatology, Geisinger Medical Center
Coauthor(s): Lindsay Dane Sewell, MD, Staff Physician, Department of Dermatology, Geisinger Medical Center; Michelle Pelle, MD, Clinical Assistant Professor, Division of Dermatology, Department of Medicine, University of California at San Diego
Contributor Information and Disclosures

Updated: Mar 10, 2008

Introduction

Background

Laugier-Hunziker syndrome (LHS) was initially described in 1970 as acquired, benign hyperpigmentated 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 LHS, 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

Pathophysiology

The etiology of melanosis in LHS is unknown. A lack of family members with LHS is characteristic in most cases. To date, only one case of familial LHS has been described, which involved a mother and 2 daughters.3 Environmental risk factors have not been identified.

Frequency

United States

The incidence is rare, but it is likely underreported. Three patients, including 1 white female and 2 Hispanic females, 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

Mortality/Morbidity

Systemic illness and malignancy are not features of LHS. Such findings in association with mucocutaneous melanotic hyperpigmentation exclude the diagnosis of LHS.

Race

  • LHS mainly affects whites; however, persons of Hispanic, Arabic, or Asian5 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 LHS 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.

Sex

  • LHS was initially predominantly thought to affect females, with an estimated female-to-male ratio of 2:1. However, the idea that LHS is equally distributed between the sexes is gaining popularity.4

Age

  • 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 years6 and a median age of 42 years7 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 LHS, 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. Additionally, the Medscape CME course Hamartomatous Polyposis Syndromes may be of interest.

Clinical

Physical

  • Oral pigmentation8,9,10 is most commonly present on the buccal mucosa and the lips (usually the lower lip), but it can also occur on the gingiva, the tongue, the soft palate, and the hard palate. Macules are brown, black, or slate with a smooth surface. They may be solitary or confluent. Round, lenticular, and linear lesions have been described, and they may be well defined or indistinct. On average, the macules are 5 mm or smaller; however, buccal lesions as large as 1 cm have been described. Oral hyperpigmentation may exist alone or in combination with nail and/or skin pigmentation. Of the 5 cases originally described by Laugier and Hunziker, 40% had oral involvement only. Subsequent reports have also described the onset of oral pigmentation following melanonychia.
  • Nail hyperpigmentation11,12,13 occurs in an estimated 60% of cases, and it is permanent. Typically, multiple nails from both the fingers and the toes are involved. The degree of pigmentation may vary between streaks on the same patient. Streaks are smooth and not associated with dystrophic changes. The following 4 pigmentary presentations have been described:
    • One longitudinal band per nail, 1-8 mm in thickness
    • Two longitudinal bands per nail, 1-8 mm in thickness, which tend to occur along the lateral aspects of the nail plate
    • Half nail pigmentation
    • Complete nail pigmentation
  • The Hutchinson sign, defined as the extension of pigment onto the proximal nail fold, is characteristically believed to be an ominous finding associated with spreading malignant melanoma. However, a pseudo-Hutchinson sign has been reported in multiple patients with LHS,14,15 sometimes on various nails of the same patient. Pigment may also involve the lateral nail folds.
  • Extended mucocutaneous pigmentation has become a recognized feature of LHS.16 Melanotic macular hyperpigmentation has been observed on the neck, the thorax, the abdomen, the dorsal and lateral aspects of the fingers, the palms and soles, the genitalia, the perineum, the perianal skin, and the anal mucosa of patients with LHS. Patients with LHS involving the conjunctiva, sclera, and esophageal mucosa have also been reported.1,3,17

Causes

The etiology of melanosis in LHS is unknown.

More on Laugier-Hunziker Syndrome

Overview: Laugier-Hunziker Syndrome
Differential Diagnoses & Workup: Laugier-Hunziker Syndrome
Treatment & Medication: Laugier-Hunziker Syndrome
Follow-up: Laugier-Hunziker Syndrome
References
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References

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  2. Gerbig AW, Hunziker T. Idiopathic lenticular mucocutaneous pigmentation or Laugier-Hunziker syndrome with atypical features. Arch Dermatol. Jul 1996;132(7):844-5. [Medline].

  3. Makhoul EN, Ayoub NM, Helou JF, Abadjian GA. Familial Laugier-Hunziker syndrome. J Am Acad Dermatol. Aug 2003;49(2 Suppl Case Reports):S143-5. [Medline].

  4. 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. Jun 2003;40(6):e77. [Medline].

  5. Tan J, Greaves MW, Lee LH. Laugier-Hunziker syndrome and hypocellular marrow: a fortuitous association?. Clin Exp Dermatol. Sep 2007;32(5):584-5. [Medline].

  6. 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. Sep 2003;96(3):288-92. [Medline].

  7. Lenane P, Sullivan DO, Keane CO, Loughlint SO. The Laugier-Hunziker syndrome. J Eur Acad Dermatol Venereol. Nov 2001;15(6):574-7. [Medline].

  8. Buchner A, Hansen LS. Melanotic macule of the oral mucosa. A clinicopathologic study of 105 cases. Oral Surg Oral Med Oral Pathol. Sep 1979;48(3):244-9. [Medline].

  9. Lamey PJ, Nolan A, Thomson E, Lewis MA, Rademaker M. Oral presentation of the Laugier-Hunziker syndrome. Br Dent J. Jul 20 1991;171(2):59-60. [Medline].

  10. Mowad CM, Shrager J, Elenitsas R. Oral pigmentation representing Laugier-Hunziker syndrome. Cutis. Jul 1997;60(1):37-9. [Medline].

  11. Baran R. Longitudinal melanotic streaks as a clue to Laugier-Hunziker syndrome. Arch Dermatol. Dec 1979;115(12):1448-9. [Medline].

  12. Baran R, Barrière H. Longitudinal melanonychia with spreading pigmentation in Laugier-Hunziker syndrome: a report of two cases. Br J Dermatol. Dec 1986;115(6):707-10. [Medline].

  13. Sterling GB, Libow LF, Grossman ME. Pigmented nail streaks may indicate Laugier-Hunziker syndrome. Cutis. Oct 1988;42(4):325-6. [Medline].

  14. 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. Mar 1999;12(2):171-3. [Medline].

  15. Porneuf M, Dandurand M. Pseudo-melanoma revealing Laugier-Hunziker syndrome. Int J Dermatol. Feb 1997;36(2):138-41. [Medline].

  16. Sabesan T, Ramchandani PL, Peters WJ. Laugier-Hunziker syndrome: a rare cause of mucocutaneous pigmentation. Br J Oral Maxillofac Surg. Aug 2006;44(4):320-1. [Medline].

  17. Yamamoto O, Yoshinaga K, Asahi M, Murata I. A Laugier-Hunziker syndrome associated with esophageal melanocytosis. Dermatology. 1999;199(2):162-4. [Medline].

  18. Kanwar AJ, Kaur S, Kaur C, Thami GP. Laugier-Hunziker syndrome. J Dermatol. Jan 2001;28(1):54-7. [Medline].

  19. Gencoglan G, Gerceker-Turk B, Kilinc-Karaarslan I, Akalin T, Ozdemir F. Dermoscopic findings in Laugier-Hunziker syndrome. Arch Dermatol. May 2007;143(5):631-3. [Medline].

  20. 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. Jun 2005;31(6):709-12. [Medline].

  21. Papadavid E, Walker NP. Q-switched Alexandrite laser in the treatment of pigmented macules in Laugier-Hunziker syndrome. J Eur Acad Dermatol Venereol. Sep 2001;15(5):468-9. [Medline].

  22. Sheridan AT, Dawber RP. Laugier-Hunziker syndrome: treatment with cryosurgery. J Eur Acad Dermatol Venereol. Sep 1999;13(2):146-8. [Medline].

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  24. Kemmett D, Ellis J, Spencer MJ, Hunter JA. The Laugier-Hunziker syndrome--a clinical review of six cases. Clin Exp Dermatol. Mar 1990;15(2):111-4. [Medline].

  25. Koch SE, LeBoit PE, Odom RB. Laugier-Hunziker syndrome. J Am Acad Dermatol. Feb 1987;16(2 Pt 2):431-4. [Medline].

  26. Lenane P, O'Sullivan D, Keane CO. Extended Laugier-Hunziker Syndrome. Poster #48 presented at American Academy of Dermatology. New Orleans, La: March 1999.

  27. McKenna KE, Walsh MY, Burrows D. Pigmentation of Peutz-Jeghers syndrome occurring in psoriatic plaques. Dermatology. 1994;189(3):297-300. [Medline].

  28. Susser WS, Whitaker-Worth DL, Grant-Kels JM. Mucocutaneous reactions to chemotherapy. J Am Acad Dermatol. Mar 1999;40(3):367-98; quiz 399-400. [Medline].

  29. Veraldi S, Cavicchini S, Benelli C, Gasparini G. Laugier-Hunziker syndrome: a clinical, histopathologic, and ultrastructural study of four cases and review of the literature. J Am Acad Dermatol. Oct 1991;25(4):632-6. [Medline].

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Further Reading

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Keywords

Laugier disease, essential melanotic pigmentation, idiopathic lenticular mucocutaneous pigmentation, LHS

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Contributor Information and Disclosures

Author

Christen M Mowad, MD, Assistant Professor, Department of Dermatology, Geisinger Medical Center
Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Coauthor(s)

Lindsay Dane Sewell, MD, Staff 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.

Michelle Pelle, MD, Clinical Assistant Professor, Division of Dermatology, Department of Medicine, University of California at San Diego
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.

Medical Editor

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.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey P Callen, MD, Professor of Medicine, 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, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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