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Year : 2019  |  Volume : 64  |  Issue : 3  |  Page : 247-248
Multiple hyperpigmented macules on labial mucosa with longitudinal melanonychia


Department of Dermatology, University Hospital Dr. José Eleuterio González, Autonomous University of Nuevo Leon, Monterrey, México

Date of Web Publication20-May-2019

Correspondence Address:
Dr. Jorge Ocampo-Candiani
Av. Francisco I. Madero Pte. s/n y Av. Gonzalitos, Col. Mitras Centro CP: 64460, Monterrey
México
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_470_18

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How to cite this article:
Garcia-Lozano JA, Gonzalez-Benavides N, Ocampo-Candiani J. Multiple hyperpigmented macules on labial mucosa with longitudinal melanonychia. Indian J Dermatol 2019;64:247-8

How to cite this URL:
Garcia-Lozano JA, Gonzalez-Benavides N, Ocampo-Candiani J. Multiple hyperpigmented macules on labial mucosa with longitudinal melanonychia. Indian J Dermatol [serial online] 2019 [cited 2019 Nov 22];64:247-8. Available from: http://www.e-ijd.org/text.asp?2019/64/3/247/258626




A 58-year-old female with a medical history of type 2 diabetes mellitus and arterial hypertension, presented with 3 months history of multiple hyperpigmented macules in the labial mucosa, hard palate, and tongue without any associated symptoms [Figure 1]a and [Figure 1]b. The patient was a nonsmoker and denied the use of any new medication that could cause the pigmentation. Dermatological examination revealed longitudinal melanonychia on the first toenail of her right foot [Figure 2]. The patient denied the history of abdominal pain, bleeding, diarrhea, intussusception, or intestinal obstruction. The family history of gastrointestinal neoplasia or pigmentary disorders was negative. Laboratory tests revealed elevated blood glucose (206 mg/dL) and urinary glucose (250 mg/dL). Complete blood count was unremarkable, fecal immunochemical test resulted negative, and levels of adrenocorticotropic hormone and cortisol were all within normal limits. Labial mucosa biopsy revealed an epidermis with acanthosis and pigmentation of the basal layer [Figure 3]. Potassium hydroxide examination of the nail was negative for the presence of hyphae, pseudohyphae, or yeast cells.
Figure 1: (a and b) Presence of multiple hyperpigmented macules in labial mucosa, hard palate and tongue

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Figure 2: Longitudinal melanonychia in first right foot toe nail

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Figure 3: Histopathological findings: Epidermis with acanthosis and pigmentation of the basal layer (H and E, ×100)

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   Question Top


What is your diagnosis?


   Answer Top


Laugier-Hunziker syndrome (LHS).


   Diagnosis Top


Due to the clinical findings of multiple hyperpigmented macules in oral and labial mucosa associated with longitudinal melanonychia in the absence of systemic disease, the patient was diagnosed with LHS.


   Review Top


LHS is a rare, benign and acquired pigmentary disorder first described in 1970 by Laugier and Hunziker; characterized by the presence of multiple hyperpigmented macules in oral and labial mucosa.[1] In 1979, Baran emphasized the importance of nail involvement as an essential finding for its diagnosis, which is found in 60% of cases. Reported nail abnormalities include a homogenous involvement of one half of the nail, an isolated pigmented band and double striking of the nails.[2] This syndrome of unknown etiology, usually affects Caucasian middle-aged women, and has not been associated with any systemic diseases.

It is a diagnosis of exclusion and a thorough examination is warranted to rule out other pathologies. Differential diagnoses of this entity include Peutz-Jeghers syndrome (PJS), Addison's disease, Albright syndrome, Bandler syndrome, Cronkhite-Canada syndrome, melanoma, drug induced pigmentation (phenytoin, zidovudine, antimalarials, clofazimine and phenothiazine), hiperpigmentation due to smoking or heavy metal exposure and oral lichen planus.[3]

Typically histopathologic examination shows hyperpigmentation of the basal epithelial layer. Most reports suggest melanocytes are normal in number, morphology and distribution. In some cases melanophages in the papillary dermis may be observed.[4] These findings indicate that the condition is due to an increased melanocytic activity. No putative genetic defects have been identified, and this is generally thought to be an acquired condition; however, STK11 gene testing allows clinicians to differentiate between PJS and LHS.[5]

LHS is a benign condition and no treatment is needed. However, some patients may choose to remove the hyperpigmentation for aesthetic considerations. Cryotherapy, Q-switched Nd:YAG laser and Q-switched alexandrite laser have been successfully used to remove the hyperpigmented macules.[6]

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Laugier P, Hunziker N. Essential lenticular melanic pigmentation of the lip and cheek mucosa. Arch Belg Dermatol Syphiligr 1970;26:391-9.  Back to cited text no. 1
    
2.
Kemmett D, Ellis J, Spencer MJ, Hunter JA. The Laugier-Hunziker syndrome – A clinical review of six cases. Clin Exp Dermatol 1990;15:111-4.  Back to cited text no. 2
    
3.
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. 3
    
4.
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. 4
    
5.
Duong BT, Winship I. The role of STK 11 gene testing in individuals with oral pigmentation. Australas J Dermatol 2017;58:135-8.  Back to cited text no. 5
    
6.
Ozawa T, Fujiwara M, Harada T, Muraoka M, Ishii M. Q-switched alexandrite laser therapy for pigmentation of the lips owing to Laugier-Hunziker syndrome. Dermatol Surg 2005;31:709-12.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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