Skip to main content
Open Access Publications from the University of California

Dermatology Online Journal

Dermatology Online Journal bannerUC Davis

Laugier Hunziker syndrome

Main Content

Laugier Hunziker syndrome
Ali Jabbari MD PhD, Mercedes E Gonzalez MD, Andrew G Franks Jr MD, Miguel Sanchez MD
Dermatology Online Journal 16 (11): 23

Department of Dermatology, New York University, New York, New York


Laugier Hunziker syndrome is a rare disorder that is characterized by adult-onset hyperpigmented macules of the lips, oral cavity, and fingertips. Longitudinal melanonychia is present in the majority of cases. We present a 45-year-old woman with adult-onset hyperpigmented macules of the oral cavity as well as linear melanonychia that involved multiple fingernails. The history, clinical examination, and paucity of laboratory abnormalities or systemic findings support a diagnosis of Laugier Hunziker syndrome.


Figure 1Figure 2

A 45-year-old woman was referred to the Dermatology Clinic at Bellevue Hospital Center in September, 2009, for evaluation of hyperpigmented macules of the fingernails and tongue. The lesions first appeared approximately four years prior to presentation and they had been getting darker for the last five months. Additionally, she presented with her brother and daughter, both of whom had similar findings of the tongue.

Past medical history included gastroesophageal reflux disease, type II diabetes mellitus, and hyperlipidemia. Medications included atorvastatin, metformin, and esomeprazole. She reported no known drug allergies.

Physical examination

On the lateral aspects of the tongue, numerous brown and dark brown macules were present. On the first, second, and third digits of the left hand, linear melanonychia was noted.

Laboratory data

Hemoglobin was 11.5 g/dL, hematocrit 25.3 percent, and mean corpuscular volume 77.5 fL. A basic metabolic panel was normal.




Laugier and Hunziker first reported a series of five patients with hyperpigmented macules of the mouth and lips; two of them had pigment changes of the fingernails [1]. Since that time, about 100 cases have been described in the literature. Affected patients have presented between the second to the ninth decades of life. Both men and women are affected. Case reports have included related family members [2]; the condition has been variably reported as sporadic [3, 4, 5], and inherited in an autosomal dominant fashion [2].

The clinical features of the syndrome include melanotic hyperpigmented macules of the oral cavity and lips [6], linear, longitudinal melanonychia of the fingernails [7], and hyperpigmented macules of the distal aspects of the fingers. Onset is usually early or in mid adult life. Hutchinson sign, which is defined as the extension of pigment onto the proximal nail fold, has been associated with Laugier Hunziker syndrome [8]. Additionally, cases have been described that include involvement of the interdigital areas [5], vulva [9], penis [10, 11], anal mucosa [12], pretibial area [13], and conjunctiva [2, 10].

Case reports have described Laugier Hunziker syndrome with esophageal melanocytosis [14], actinic lichen planus [15], hypocellular marrow and thrombocytopenia [16], invasive melanoma [17], and lupus erythematous [18]. The relationships with these conditions have not been well established.

The histopathologic description of the mucosal macules of Laugier Hunziker syndrome includes normal numbers and appearance of melanocytes although some case reports have described increases in the numbers of melanocytes [6]. There is accumulation of pigment in the basal epidermal layer. Melanophages can be observed in the reticular dermis. Nevus cells have not been reported. Electron-microscope examination has confirmed the presence of large numbers of mature melanosomes in the stratum basale [19].

The differential diagnosis for Laugier Hunziker syndrome includes drug ingestion (phenytion, antimalarials, clofazimine, zidovudine, and phenothiazine), Albright syndrome, Addison disease [20], and Peutz Jeghers syndrome (PJS). In PJS, hyperpigmented macules of the oral mucosa and acral skin are either congenital or appear in early life. This fact contrasts with the acquisition of characteristic skin lesions in Laugier Hunziker syndrome, which usually occur after puberty. Furthermore, PJS is associated with hamartomatous polyps of the gastrointestinal tract whereas Laugier Hunziker syndrome is not. Lastly, the distribution of skin lesions may be different between these diseases; PJS rarely involves the tongue or palate. Fingernail involvement in PJS has been reported in a few cases whereas it is observed in approximately 60 percent of cases of Laugier Hunziker syndrome [21]. Although the patient presented here did not undergo colonoscopic or esophagogastroduodenoscopic evaluation, the appearance of her skin lesions in mid adulthood and the fingernail involvement support a diagnosis of Laugier Hunziker syndrome over PJS.

Treatment for the hyperpigmented macules in Laugier Hunziker syndrome include cryosurgery [22], Q-switched Nd:YAG [23], and Q-switched alexandrite lasers [24, 25].


1. Laugier P, Hunziker N. Pigmentation mélanique lenticulaire, essentielle, de la muqueuse jugale et des lèvres. Arch Belg Dermatol Syphiligr 1970;26:391

2. Makhoul EN, et al. Familial Laugier-Hunziker syndrome. J Am Acad Dermatol 2003;49:S143 [PubMed]

3. Lampe AK, et al. Laugier-Hunziker syndrome: an important differential diagnosis for Peutz-Jeghers syndrome. J Med Genet 2003;40:e77 [PubMed]

4. Fisher D, et al. Laugier-Hunziker Syndrome. Clin Exp Dermatol 2004;29:312 [PubMed]

5. Porneuf M, Dandurand M. Pseudo-melanoma revealing Laugier-Hunziker syndrome. Int J Dermatol 1997;36:138 [PubMed]

6. Moore RT, et al. Laugier and Hunziker pigmentation: a lentiginous proliferation of melanocytes. J Am Acad Dermatol 2004;50:S70 [PubMed]

7. Koch SE, et al. Laugier-Hunziker syndrome. J Am Acad Dermatol 1987;16:431 [PubMed]

8. Baran R, Barriere H. Longitudinal melanonychia with spreading pigmentation in Laugier-Hunziker syndrome: a report of two cases. Br J Dermatol 1986;115:707 [PubMed]

9. Lenane P, et al. The Laugier-Hunziker syndrome. J Eur Acad Dermatol Venereol 2001;15:574 [PubMed]

10. Ayoub N, et al. Additional conjunctival and penile pigmentation in Laugier-Hunziker syndrome: a report of two cases. Int J Dermatol 2004;43:571 [PubMed]

11. Revuz J, Clerici T. Penile melanosis. J Am Acad Dermatol 1989;20:567 [PubMed]

12. Gerbig AW, Hunziker T. Idiopathic lenticular mucocutaneous pigmentation or Laugier-Hunziker syndrome with atypical features. Arch Dermatol 1996; 132:844 [PubMed]

13. Aliagaoglu C, et al. Laugier-Hunziker syndrome: diffuse large hyperpigmentation on atypical localization. J Dermatol 2008;35:806 [PubMed]

14. Yamamoto O, et al. A Laugier-Hunziker syndrome associated with esophageal melanocytosis. Dermatology 1999;199:162 [PubMed]

15. Aytekin S, Alp S. Laugier-Hunziker syndrome associated with actinic lichen planus. J Eur Acad Dermatol Venereol 2004;18:221 [PubMed]

16. Tan J, et al. Laugier-Hunziker syndrome and hypocellular marrow: a fortuitous association? Clin Exp Dermatol 2007;32:584 [PubMed]

17. Simionescu O, et al. Dermatoscopy of an invasive melanoma on the upper lip shows possible association with Laugier-Hunziker syndrome. J Am Acad Dermatol 2008;59:S105 [PubMed]

18. Lamey P, et al. Oral presentation of the Laugier-Hunziker syndrome. Br Dent J 1991;171:59 [PubMed]

19. Veraldi S, et al. Laugier-Hunziker syndrome: a clinical, histopathologic, and ultrastructural study of four cases and review of the literature. J Am Acad Dermatol 1991;25:632 [PubMed]

20. Yesudian P, et al. Primary adrenocortical insufficiency masquerading as Laugier-Hunziker syndrome. Int J Dermatol 2008;47:596 [PubMed]

21. Yago K, et al. Laugier-Hunziker-Baran syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;106:e20 [PubMed]

22. Sheridan A T, Dawber R P. Laugier-Hunziker syndrome: treatment with cryosurgery. J Eur Acad Dermatol Venereol 1999;13:146 [PubMed]

23. Ferreira, M et al. Laugier-Hunziker syndrome: case report and treatment with the Q-switched Nd-Yag laser. J Eur Acad Dermatol Venereol 1999;12:171 [PubMed]

24. Ozawa T, et al. Q-switched alexandrite laser therapy for pigmentation of the lips owing to Laugier-Hunziker syndrome. Derm Surg 2005;31:709 [PubMed]

25. Papadavid E, Walker N P. Q-switched Alexandrite laser in the treatment of pigmented macules in Laugier-Hunziker syndrome. J Eur Acad Dermatol Venereol 2001;15:468 [PubMed]

© 2010 Dermatology Online Journal