Laugier-Hunziker Syndrome Clinical Presentation

  • Author: Christen M Mowad, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 18, 2012
 

Physical

Oral pigmentation[8, 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. The latter three may also help distinguish Laugier-Hunziker from Petz-Jeghers syndrome.[11] 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 hyperpigmentation[12, 13, 14] 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 Laugier-Hunziker syndrome,[15, 16] 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 Laugier-Hunziker syndrome.[17] 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 Laugier-Hunziker syndrome. Patients with Laugier-Hunziker syndrome involving the conjunctiva, sclera, and esophageal mucosa have also been reported.[1, 3, 18]

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Causes

The etiology of melanosis in Laugier-Hunziker syndrome is unknown.

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Contributor Information and Disclosures
Author

Christen M Mowad, MD  Associate 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, American Dermatological Association, Noah Worcester Dermatological Society, Pennsylvania Academy of Dermatology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

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.

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, 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.

Specialty Editor Board

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.

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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

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, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Celgene Honoraria Safety Monitoring Committee

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M 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, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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  21. 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. Nov 2010;37(11):980-4. [Medline].

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  24. 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. Mar 2010;302(2):125-30. [Medline].

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