Laugier-Hunziker Syndrome Clinical Presentation

Updated: Jun 27, 2019
  • Author: Christen M Mowad, MD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

Physical Examination

Oral pigmentation [6, 10, 11, 12] 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. [13] 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. Cutaneous lesions may fade following puberty; however, oral hyperpigmentation occurs gradually and is considered permanent. Of the five cases originally described by Laugier and Hunziker, 40% had oral involvement only. Subsequent reports have also described the onset of oral pigmentation following melanonychia. Melanoacanthomas have also been reported.

See the image below.

Oral findings in Laugier-Hunziker syndrome. Courte Oral findings in Laugier-Hunziker syndrome. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/colour/laugier1.jpg).

Nail hyperpigmentation [6, 14, 15, 16] 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 four 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 (melanonychia)

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, [9, 17] sometimes on various nails of the same patient. Pigment may also involve the lateral nail folds.

See the image below.

Nail findings in Laugier-Hunziker syndrome. Courte Nail findings in Laugier-Hunziker syndrome. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/colour/laugier2.jpg).

Extended mucocutaneous pigmentation has become a recognized feature of Laugier-Hunziker syndrome. [18] 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, 19]

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Complications

Systemic illness and malignancy are not features of Laugier-Hunziker syndrome (LHS). Thrombocytopenia and anemia have been reported in association with Laugier-Hunziker syndrome; however, systemic illness is not typically seen. [20]

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