Laugier-Hunziker Syndrome Workup

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

Laboratory Studies

Laugier-Hunziker syndrome (LHS) is a diagnosis of exclusion. When the diagnosis of Laugier-Hunziker syndrome is suspected, the following tests should be performed to rule out other differential diagnoses:

  • The corticotropin level is elevated in patients with Addison disease associated with primary adrenal failure. The morning cortisol level is low, and corticotropin stimulation results are abnormal. In addition, electrolyte abnormalities, including hyperkalemia and hyponatremia, may be observed. Significant electrolyte abnormalities may be absent, and the serum potassium level is not sensitive enough to use as a screening test for Addison disease. Patients with Addison disease often report salt cravings, weight loss, and fatigue. Physical examination may reveal hypotension, pigmentation of the buccal mucosa and nails, generalized hyperpigmentation, or darkening of nevi and scars.
  • The antinuclear antibody (ANA) test is useful to screen for connective tissue diseases, including lupus erythematosus, which may cause buccal and lip hyperpigmentation. Nail streaks have not been reported in patients with lupus. Sjögren syndrome, subacute cutaneous lupus erythematosus, Coombs positive–autoimmune hemolytic anemia, and inflammatory arthritis have occurred in some patients with Laugier-Hunziker syndrome. The significance of comorbid connective tissue disease and Laugier-Hunziker syndrome is currently unknown.
  • Liver function test results are abnormal in hemochromatosis.
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Imaging Studies

Radiographic barium contrast studies are performed to rule out gastrointestinal polyposis in association with PJS. Most commonly, hamartomas of the jejunum are present in PJS, but they may be present anywhere throughout the gastric, intestinal, and colonic mucosae.

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Other Tests

Dermatoscopic findings have been reported and include the following[20, 21] :

  • Mucosa - Parallel patterns of pigmentation
  • Nails - Longitudinal, homogeneous, regular, bandlike pigmentation with indistinct borders
  • Palmar/plantar skin - Parallel, furrowed hyperpigmentation

Dermatoscopic findings vary widely given the different locations involved. Further evaluation of these findings is needed to better establish dermatoscopic criteria for this condition.

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Procedures

Endoscopy may be performed in lieu of, or in addition to, radiographic barium contrast studies to rule out PJS.

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Histologic Findings

Biopsy specimens from the oral and esophageal mucosa and from keratinized skin have been studied. Nail biopsies are rarely performed in the setting of multiple pigmented bands; therefore, nail histology has not been described.

The pigmented macules of Laugier-Hunziker syndrome are not lentigines. They demonstrate mild-to-moderate acanthosis in most cases.[3] The predominant finding is basal cell hypermelanosis. The melanin deposition in the basal layer is dense and uniform. Rete ridges may be normal in size, or they may be elongated. Numerous melanophages are often present in the papillary dermis. The basement membrane has been found to be intact. Pigment incontinence may also be present.[3]

Although the absence of melanocytic proliferation has been a typical pathologic feature, one study recently noted an increase of nonnested intraepidermal melanocytes in the areas of clinical hyperpigmentation using S100 and L-3,4 dihydroxyphenylalanine immunohistochemistry studies.[1]

Electron microscopy reveals normal melanosome transfer to keratinocytes. Some studies suggest that melanosomes within basal keratinocytes are enlarged, whereas the melanosomes within papillary dermal melanophages are normal in size and in number.

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

  17. 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].

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

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  20. 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].

  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].

  22. 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].

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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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