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Year : 2020  |  Volume : 13  |  Issue : 6  |  Page : 681-684  

Laugier–Hunziker syndrome: A rare cause of diffuse orofacial pigmentation

1 Department of Oral Medicine and Radiology, GSL Dental College and Hospital, Rajahmundry, Andhra Pradesh, India
2 Department of Dentistry, Ananta Institute of Medical Sciences and Research Centre, Rajsamand, Rajasthan, India

Date of Submission25-Jul-2019
Date of Decision11-Mar-2020
Date of Acceptance11-Mar-2020
Date of Web Publication6-Nov-2020

Correspondence Address:
Silky R Punyani
903, Anant Sneh Heights, New Navratan Complex, Udaipur - 313 003, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_217_19

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Laugier–Hunziker syndrome (LHS) is lesser known and important differential of the multifocal mucocutaneous pigmentation. Essentially idiopathic in nature, this syndrome is characterized by multiple melanotic macules on the oral mucosa, lips, perioral region, and melanonychia. A young Indian male reported to us with a complaint of multiple brown-black spots on the lips and inner surface of the cheeks accompanied by the pigmentation of fingernail. The patient was evaluated for the possible causes of multifocal oral pigmentation. After taking an exhaustive clinical history and thorough investigations, no exact cause for this condition could be definitively ascertained. Eventually, he was diagnosed as a case of LHS, a rare cause of idiopathic oral pigmentation. The various differential diagnoses for the condition are also discussed.

Keywords: Oral diagnosis, oral hyperpigmentation, oral melanosis, oral pigmentation disorders

How to cite this article:
Deshpande A, Punyani SR. Laugier–Hunziker syndrome: A rare cause of diffuse orofacial pigmentation. Med J DY Patil Vidyapeeth 2020;13:681-4

How to cite this URL:
Deshpande A, Punyani SR. Laugier–Hunziker syndrome: A rare cause of diffuse orofacial pigmentation. Med J DY Patil Vidyapeeth [serial online] 2020 [cited 2021 Sep 20];13:681-4. Available from: https://www.mjdrdypv.org/text.asp?2020/13/6/681/300131

  Introduction Top

Laugier–Hunziker syndrome (LHS) is a rare disease, which is a benign pigmentary disorder characterized by multiple discrete, grey-to-brown-black macules on the skin and mucosae.[1] This interesting entity is a disease of exclusion as this diagnosis is made after ruling out all other possible causes of diffused oral and perioral pigmentation. We report a compelling case of LHS in a young Indian male.

  Case Report Top

A 25-year-old male patient reported to the department with a chief complaint of blackish spots on his lips and the inner surface of the cheeks that had gradually increased over 5 years. They were of the same size and shape ever since with no evident history of trauma and were not associated with tenderness or pain. No similar lesions were noted elsewhere. The patient had consulted multiple general practitioners and dentists with no improvement in the condition. A thorough systemic clinical history revealed no previous complaints and symptoms related to cardiovascular, respiratory, gastrointestinal, and endocrinal systems. There was no history of any medications and increased exposure to sunlight. Dental and family histories were unremarkable. He denied the smoking habit. On examination, the patient was moderately built. There was evidence of multiple blackish macules along the vermilion border of the lower and upper lip [Figure 1]a and [Figure 1]b. Intraoral examination revealed similar lesions on the upper and lower labial mucosa [Figure 1]b. Likewise, distantly placed lesions were seen on the buccal mucosae [Figure 1]c and [Figure 1]d.
Figure 1: (a and b) Multiple blackish macules on the vermilion borders of the lips and upper and lower labial mucosa. (c and d) Brownish macules present on bilateral buccal mucosae

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The lesions measured between 1 and 5 mm and were flat. They were nontender, with no remarkable alteration in consistency as compared to surrounding mucosa. On examination of extremities, a linear melanotic streak was noticed in one of the fingernails [Figure 2] although the toenails were spared. We proceeded to investigate the patient to rule out systemic causes of multifocal oral pigmentation, which included Cushing's disease, hypothyroidism, Peutz–Jeghers syndrome (PJS), and healing lesions of oral lichen planus among others.
Figure 2: A linear melanotic streak present on the fingernail

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The complete blood counts were within normal limits. Other blood parameters including thyroid function tests and serum cortisol were also within normal limits. ELISA for anti-HIV was negative. Endoscopy was then performed, and it did not reveal any polyposis of the intestines. The findings of the abdominal ultrasound were normal. Excisional biopsy was performed on the lesion of the left buccal mucosa which revealed nonpathognomonic findings of basilar melanin incontinence without a concomitant increase in the number of melanocytes [Figure 3].
Figure 3: Photomicrograph of the biopsy specimen showing an increase in the melanin production in the basal layer of the stratified squamous epithelium. Some amount of melanin pigmentation can be seen in some regions of the lamina propria. There is no increase in the number of melanocytes per se or melanocytic atypia. Features corroborative of oral melanotic macule (H and E, ×40)

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Based on the above findings and after ruling out all the other causes of multifocal oral pigmentation, the patient was diagnosed as a case of LHS. Although the patient was initially apprehensive regarding the pigmentation, once reassured and explained the benign nature of this entity, he opted to quit any therapeutic measures.

This case report has the patient's consent and the approval of the institutional ethics committee for publication.

  Discussion Top

LHS is a rare cause of multifocal oral and mucosal pigmentation. It is an acquired, benign condition characterized by brown-black macules on the lips and buccal mucosa accompanied with longitudinal melanonychia of unknown etiopathogenesis.[2],[3] It has been variably reported as sporadic and has been shown to have an autosomal dominant inheritance, although others have suggested that no genetic factors are associated.[4],[5] LHS is characterized by pigmentations which usually develop before the middle adulthood. Females are more commonly affected with most patients usually in the third to fifth decade.[6],[7] Clinical features include multiple, painless, lenticular (lens-shaped), or linear, brown-to-black mucocutaneous macules, usually <5 mm in diameter. The lesions may appear single or confluent with well-defined or diffuse margins. The hyperpigmentation is spontaneous and insidious in onset but stays permanently unless cosmetic intervention is done.[8] The lesions are most common on the lips, buccal mucosa, and hard palate. Another important finding is the pigmentation of the nails which appears as thick vertical melanotic bands. Single or multiple bands may form on nails either partially or completely.[9] Hence, further, single or multiple nails may be affected as the report by Wang et al. in their three cases of LHS.[10] In the first case along with multiple oral macules, a double longitudinal pigmented band was present on the fingernail of the right thumb only. The second case also reported irregular pigmentation only on the index finger of the right hand. Their third case had the involvement of multiple nails.

Singular nail involvement in LHS was also reported in the case series by Baran.[11] In his series of nine patients, only four patients had pigmentation of the fingernails (involving one finger only in one patient and three to six in the other three cases).

Duan et al. in their systematic review of LHS have reported the distribution of involved locations of pigmentary lesions in LHS [Table 1].[12]
Table 1: Distribution of locations involved by pigmentary lesions in LHS

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Thus, the oral cavity and lips remain the most frequently involved sites, and the involvement of other sites may vary from case to case.

This syndrome has no reported malignant potential.[8] As this condition is basically a diagnosis of exclusion, investigations must be done to rule out hypoadrenocorticism and Cushing disease, Peutz Jeghers Syndrome, HIV infection, Graves' disease, primary biliary cirrhosis, and Vitamin B12 insufficiency, which cause a similar pattern of pigmentation.[13],[14] Histopathological picture is often nonspecific and nonpathognomonic showing basilar melanin incontinence.[15] The key clinical differential diagnoses of LHS include PJS and Addison's disease apart from more common drug-induced pigmentation. PJS is characterized by oral and perioral pigmentation, pigmentation on the palms/soles, and associated with hamartomatous gastrointestinal polyps, which carry high malignant potential.[16] This condition was ruled out in the present case by performing an abdominal ultrasound and endoscopic examination. Addison's disease is characterized by hyperpigmentation of the skin in areas subjected to increased pressure, such as knuckles, skin creases, and mucous membranes. Accompanying features include sparse pubic and axillary hair in women, dehydration, hypotension, and abdominal pain. Such signs and symptoms were absent in the present case, and serum cortisol levels were within normal limits. Drug-induced pigmentation was ruled out in the present case as the patient had not been on medication. HIV-associated pigmentation also presents as diffuse pigmentation in the oral cavity. This infection was ruled out after the ELISA for HIV identification turned out to be negative. Less probable differentials including  McCune-Albright syndrome More Details, Gardener syndrome, and heavy metal exposure were also excluded. Treatment is required in these patients for esthetics. It includes Nd-YAG lasers, usually used in the treatment of melanosis of the orofacial region.[17],[18]

  Conclusion Top

The present case qualifies as a case of LHS after taking a detailed case history and relevant investigations to rule out the known causes of mucocutaneous pigmentation. The presence of melanonychia is an essential clinical finding for a diagnosis of LHS. LHS should always be kept in mind when no obvious pathology for hyperpigmentation can be accounted for. It is necessary for an oral physician as well as a general dentist to be aware of this lesser known entity, its benign nature, and to reassure the patient accordingly.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Moore RT, Chae KA, Rhodes AR. Laugier and Hunziker pigmentation: A lentiginous proliferation of melanocytes. J Am Acad Dermatol 2004;50:S70-4.  Back to cited text no. 1
Koch SE, LeBoit PE, Odom RB. Laugier-Hunziker syndrome. J Am Acad Dermatol 1987;16:431-4.  Back to cited text no. 2
Makhoul EN, Ayoub NM, Helou JF, Abadjian GA. Familial Laugier-Hunziker syndrome. J Am Acad Dermatol 2003;49:S143-5.  Back to cited text no. 3
Pereira PM, Rodrigues CA, Lima LL, Reyes SA, Mariano AV. Do you know this syndrome? An Bras Dermatol 2010;85:751-3.  Back to cited text no. 4
Jabbari A, Gonzalez ME, Franks AG Jr., Sanchez M. Laugier Hunziker syndrome. Dermatol Online J 2010;16:23.  Back to cited text no. 5
Nikitakis NG, Koumaki D. Laugier-Hunziker syndrome: Case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol 2013;116:e52-8.  Back to cited text no. 6
Lalosevic J, Zivanovic D, Skiljevic D, Medenica L. Laugier-Hunziker syndrome – Case report. An Bras Dermatol 2015;90:223-5.  Back to cited text no. 7
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;96:288-92.  Back to cited text no. 8
Gerbig AW, Hunziker T. Idiopathic lenticular mucocutaneous pigmentation or Laugier-Hunziker syndrome with atypical features. Arch Dermatol 1996;132:844-5.  Back to cited text no. 9
Wang WM, Wang X, Duan N, Jiang HL, Huang XF. Laugier-Hunziker syndrome: A report of three cases and literature review. Int J Oral Sci 2012;4:226-30.  Back to cited text no. 10
Baran R. Longitudinal melanotic streaks as a clue to Laugier-Hunziker syndrome. Arch Dermatol 1979;115:1448-9.  Back to cited text no. 11
Duan N, Zhang YH, Wang WM, Wang X. Mystery behind labial and oral melanotic macules: Clinical, dermoscopic and pathological aspects of Laugier-Hunziker syndrome. World J Clin Cases 2018;6:322-34.  Back to cited text no. 12
Nayak RS, Kotrashetti VS, Hosmani JV. Laugier-Hunziker syndrome. J Oral Maxillofac Pathol 2012;16:245-50.  Back to cited text no. 13
  [Full text]  
Alawi F. Pigmented lesions of the oral cavity: An update. Dent Clin North Am 2013;57:699-710.  Back to cited text no. 14
Gencoglan G, Gerceker-Turk B, Kilinc-Karaarslan I, Akalin T, Ozdemir F. Dermoscopic findings in Laugier-Hunziker syndrome. Arch Dermatol 2007;143:631-3.  Back to cited text no. 15
Lampe AK, Hampton PJ, Woodford-Richens K, Tomlinson I, Lawrence CM, Douglas FS. Laugier-Hunziker syndrome: An important differential diagnosis for Peutz-Jeghers syndrome. J Med Genet 2003;40:e77.  Back to cited text no. 16
Rangwala S, Doherty CB, Katta R. Laugier-Hunziker syndrome: A case report and review of the literature. Dermatol Online J 2010;16:9.  Back to cited text no. 17
Sheridan AT, Dawber RP. Laugier-Hunziker syndrome: Treatment with cryosurgery. J Eur Acad Dermatol Venereol 1999;13:146-8.  Back to cited text no. 18


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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