An elderly man presents with asymptomatic hyperpigmented, confluent, brown macules on his upper and lower lips, along with hyperpigmented macules on his hands. He also has diffuse blue-black bilateral melanonychia with nail dystrophy on his toenails, and his fingernails contain longitudinal melanonychia with hyperpigmentation of the proximal nail beds. He denies any family history of mucocutaneous pigmentation or gastrointestinal or systemic symptoms. In the last 5 years, results of a colonoscopy, upper gastrointestinal endoscopy, and blood work have all been within normal limits.
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Laugier-Hunziker syndrome (LHS) is a rare, benign, acquired disease with unknown pathogenesis that was first described in the 1970s by Laugier and Hunziker.1 The asymptomatic hyperpigmentation of LHS presents spontaneously on the skin, lips, or mucosa as gray, dark brown, or blue-black lenticulate or linear macules that are typically less than 5 mm in diameter.2 Histologically, the macules have a collection of pigmentation in the basal layer of the epidermis due to increased synthesis of melanosomes.
Approximately 50% of patients have melanonychia with a variety of longitudinal striata that typically occur in the fingernails.2 Partial or complete nail pigmentation may also be present. Hutchinson sign, a pigmentation of the proximal nail folds, is also seen in some patients.3 Typically, LHS develops in middle-aged adults with mean onset at age 50; it is more prevalent in women and individuals of French or Italian origin.4
LHS is a rare disorder; diagnosis is based on clinical presentation with the exclusion of other systemic disease. Although LHS is a benign disease with no risk of malignant transformation, it is important to rule out other diseases of diffuse mucocutaneous and nail pigmentation that warrant further investigation or action by conducting a thorough medical history.
Symptoms such as fatigue, weight loss, gastrointestinal or cardiovascular diseases, history of drug use, and abnormal laboratory values could suggest systemic conditions, including Peutz-Jeghers syndrome, Bandler syndrome, McCune-Albright syndrome, subungual melanoma, racial pigmentation, acquired immunodeficiency syndrome, and primary adrenal insufficiency.2 Peutz-Jeghers syndrome often presents with pigmentation of the lips and oral mucosa. However, intestinal polyps are also seen with Peutz-Jeghers syndrome; therefore, this disease can be ruled out with a normal colonoscopy and upper gastrointestinal endoscopy. Bandler syndrome includes hyperpigmented macules of the hands, fingernails, and oral mucosa that occur during infancy. This syndrome also includes vascular malformations in the intestine, which can cause significant bleeding. McCune-Albright syndrome includes precocious puberty and fibrous dysplasia. The condition may present with labial or genital pigmentation, but does not affect the nails.
Subungual melanoma or skin cancer should be ruled out with a punch biopsy. Racial pigmentation occurs more commonly in the gingiva and is seen in populations such as Asians and African Americans. Drug-induced pigmentation can occur with the use of certain medications, but this condition reverses after the patient discontinues the offending drugs.2
The patient in our case had an atypical presentation with involvement of not only his fingernails but also his toenails. There have been previous case reports of longitudinal striate on the toenails, including one report of a 77-year-old man with bilateral fingernail and toenail striate, macules on the proximal nail folds, and confluent brown hyperpigmented macules on the lips and mucosa that developed asymptomatically over 4 years.2,5,6 This patient had complete melanonychia of some of his toenails, along with longitudinal striate, demonstrating the importance of considering the breadth and variety of nail pigmentation in LHS.2
Some patients with LHS may seek cosmetic treatment options, although there is no requirement for further medical treatment. Cosmetic treatment options include laser or cryotherapy. In one report, 22 Chinese patients received Q-switched alexandrite laser treatment; approximately 81% had excellent clearing of oral pigmentation after one treatment session.7 Although positive response to laser treatment has been reported, there is one reported case of the reoccurrence of lesions on the lips of a patient with LHS 12 months after erbium:yttrium-aluminum-garnet (Er:YAG) laser treatment.8 Further studies are needed to evaluate the efficacy of lasers in the treatment of LHS.
In our case, once testing ruled out other disorders, the patient was diagnosed with LHS. He was not concerned with the appearance of the hyperpigmentation and therefore elected not to pursue treatment.
Amelia Bush, BS, is a medical student and Maura Holcomb, MD, is a dermatology resident at Baylor College of Medicine in Houston.
- Laugier P, Hunziker N. Essential lenticular melanic pigmentation of the lips and cheek mucosa [in French]. Arch Belg Dermatol Syphiligr. 1970;26(3):391-399.
- Rangwala S, Doherty CB, Khatta R. Laugier-Hunziker syndrome: a case report and review of the literature. Dermatol Online J. 2010;16(12):9.
- 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;96(3):288-292.
- Pereira PM, Rodrigues CA, Lima LL, et al. Do you know this syndrome? An Bras Dermatol. 2010;85(5):751-753.
- Lampe AK, Hampton PJ, Woodford-Richens K, et al. Laugier-Hunziker syndrome: an important differential diagnosis for Peutz-Jeghers syndrome. J Med Genet. 2003;40(6):e77.
- Wang WM, Wang X, Duan N, et al. Laugier-Hunziker syndrome: a report of three cases and literature review. Int J Oral Sci. 2012;4(4):226-230.
- Zuo YG, Ma DL, Jin HZ, et al. Treatment of Laugier-Hunziker syndrome with the Q-switched alexandrite laser in 22 Chinese patients. Arch Dermatol Res. 2010;302(2):125-130.
- Ergun S, Saruhanoğlu A, Migliari DA, et al. Refractory pigmentation associated with Laugier-Hunziker syndrome following Er:YAG laser treatment. Case Rep Dent. 2013;2013:561040.