|Year : 2014 | Volume
| Issue : 2 | Page : 103-105
Uncommon cause of common oral hyperpigmentation: Laugier-Hunziker syndrome
Sarabjit Kaur1, Nidhi Jindal1, Vijay Kumar Jain1, Richa Jindal2
1 Department of Dermatology, Venereology and Leprosy, Pt. B.D. Sharma PGIMS, Rohtak, Haryana, India
2 Department of Pathology, St Stevens Hospital, New Delhi, India
|Date of Web Publication||15-Dec-2014|
House No. 401, Sector 14, Rohtak - 124 001, Haryana
Source of Support: None, Conflict of Interest: None
Laugier– Hunziker syndrome (LHS) is a rare benign acquired disorder that is characterized by hyperpigmented macules of the oral cavity, lips and tips of the fingers and toes, often associated with longitudinal melanonychia. The case discussed here is a 50-year-old woman with clinical and histopathological features suggestive of LHS. We also elicit the various other causes of a similar presentation.
Keywords: Laugier– Hunziker syndrome, longitudinal melanonychia, oral hyperpigmentation
|How to cite this article:|
Kaur S, Jindal N, Jain VK, Jindal R. Uncommon cause of common oral hyperpigmentation: Laugier-Hunziker syndrome. Pigment Int 2014;1:103-5
| Introduction|| |
Literature implicates various causes of the oral hyperpigmentation, simultaneous detailed examination of nails and hair is always required to give a definite diagnosis. One such disorder, Laugier-Hunziker syndrome (LHS) is an uncommon cause of commonly seen hyperpigmented macules of the oral cavity, lips and longitudinal melanonychia. It usually begins in third to fifth decade of life with a female preponderance.  LHS is an idiopathic rare benign acquired disorder without systemic involvement. 
Here, we describe a middle age female patient with a diffuse oral hyperpigmentation associated with longitudinal pigmented bands on nails. These symptoms were correlated, and the diagnosis of LHS was made on the basis of clinical and histological findings.
| Case Report|| |
A 50-year-old female was referred by ENT department for the evaluation of hyperpigmented macules in the oral cavity. Asymptomatic mucosal pigmentation was present for the last 5 years. There was no history of drug intake, trauma, smoking or any other addiction prior to the onset of the lesions. There was no history of abdominal pain, diarrhea, vomiting, hematemesis, and rectal bleeding, hypotension or weight loss. No other family member had similar oral or cutaneous pigmentation. Clinical examination revealed diffuse hyperpigmentation over the dorsum of the tongue and discrete macular pigmentation involving lips. Further examination of nails revealed longitudinal pigmented bands involving middle and ring finger of the right hand and middle finger of the left hand [Figure 1]. There was no periungual extension of the brown-black pigmentation. Dermoscopic findings of the pigmented band showed thin longitudinal brown lines homogenous in thickness and color. Cutaneous and hair examination was normal. Probable differential diagnoses were LHS, lichen planus, Addison's disease, Peutz-Jeghers syndrome (PJS); and the patient was investigated accordingly. All routine hematological and biochemical investigations were within normal limits. Thyroid function test was within normal limits. Serum and urine cortisol along with adrenocorticotropin hormone levels were normal according to age. Colonoscopy to rule out hamartomatous polyposis was not performed as a patient did not present with any intestinal symptom. The patient's age and absence of family history ruled out the possibility of PJS. Histopathology examination of the biopsy from tongue lesions showed acanthotic epithelium covered soft tissue with an increase in basal layer pigmentation. Few melanophages are present in underlying soft tissue [Figure 2]a and b. A final diagnosis of LHS was made on the basis of clinical and histological features and absence of systemic involvement.
|Figure 1: Diffuse widespread pigmentation over dorsum of tongue and longitudinal melanonychia of fi nger nails|
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|Figure 2: (a) Microphotograph showing soft tissue with melanin incontinence (H and E; ×40) (b) microphotograph showing basal layer hyperpigmentation (H and E; ×40)|
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| Discussion|| |
In 1970, Laugier and Hunziker first reported a series of five patients with acquired mucocutaneous pigmentation; two of them had longitudinal melanonychia of fingernails. , Most of the cases reported from Asia including India are sporadic. However, Makhoul et al. reported three cases of LHS in the same family. No definite cause has been identified yet. , LHS, however, is an acquired benign pigmentary disorder of the lips, oral mucosa and fingers, without any systemic manifestation.  Clinical features include dark brown lenticular or linear, solitary or confluent macules of oral cavity and lips. In 50-60% of the patient, nails shows longitudinal melanonychia of varying width, with involvement of one or more of the fingernails and less often toenails.  When a single digit is affected, subungual malignant melanoma must be considered. The most common cause of longitudinal melanonychia is benign racial pigmentation. 
Histology of macules shows accumulation of melanin in the basal layer of epithelium.  Pigment - laden melanophages are evident in the papillary dermis with normal number and morphology of melanocytes while one report does describe increased non-nested intraepidermal melanocytes. , Histopathologically, similar disorders with basal hyperpigmentation (mild melanin incontinence is sometimes present also) includes generalized hyperpigmentary disorders; idiopathic eruptive macular pigmentation, dyschromatosis symmetrica hereditaria, dyschromatosis universalis, melasma, ephelis (freckle), cafι-au-lait spots, macules of Albright's syndrome, Bannayan-Riley-Ruvalcaba syndrome and PJS, and Becker's nevus.  Q-switched alexandrite laser has been reported to be effective and safe for treating pigmented lesions of LHS, however reassurance is all that required. 
Peutz-Jeghers syndrome and LHS share overlapping clinical and histological features. However, the onset of the lesions in early life and the presence of intestinal polyposis or pigmentation involving hands and feet suggest PJS while both oral and nail pigmentations favors LHS. Differential diagnosis of a similar presentation has been enumerated in [Table 1] and [Table 2]. ,, All patients presenting with an oral hyperpigmentation should undergo a complete evaluation. Diagnosis of LHS must be established only after excluding underlying systemic pathologic conditions.
|Table 1: Differential diagnosis of similar presentation/mucocutaneous pigmentation|
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| Conclusion|| |
As far as our knowledge goes, few cases of LHS have been reported in India. Though it has been reported worldwide, its entity is not known or rather to say an often missed case. A keen eye is needed to diagnose this acquired benign disorder and must not miss examination of the nails of the patients presenting with mucocutaneous pigmentation as it prevents unnecessary and potentially hazardous investigations. No treatment is required other than reassurance as these patients do not have any systemic complaints or complications.
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[Figure 1], [Figure 2]
[Table 1], [Table 2]