Laugier-Hunziker syndrome: a case of asymptomatic mucosal and acral hyperpigmentation
Keywords:
Laugier-Hunziker, mucosal and acral hyperpigmentation, mucocutaneous hyperpigmentationAbstract
Laugier-Hunziker syndrome (LHS) is a rare condition characterized by acquired hyperpigmentation involving the lips, oral mucosa, acral surfaces, nails and perineum. While patients with LHS may manifest pigmentation in all of the aforementioned areas, most present with pigmentation localized to only a few of these anatomical sites. We herein report a patient exhibiting the characteristic pigment distribution pattern associated with LHS. Since LHS is a diagnosis based on exclusion, we discuss the differential diagnosis of mucocutaneous hyperpigmentation. Due to the benign nature of the disease, it is critical to differentiate this disorder from conditions with similar mucocutaneous pigmentary changes with somatic abnormalities that require medical management. We also explore potential mechanisms that may explain the pathogenesis of LHS.
References
Laugier P, Hunziker N. Pigmentation melanique lenticulaire, essentielle de la muqueuse jugale et des levres. Arch Belges Dermatol Syphiligr. 1970;26(3):391-399.
Nikitakis NG, Koumaki D. Laugier–Hunziker syndrome: case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral. 2013;116(1):e52-8. PubMed CrossRef
Rangwala S, Doherty CB, Katta R. Laugier-Hunziker syndrome: A case report and review of the literature. Dermatol Online J. 2010;16(12). PubMed
Moore RT, Chae KM, Rhodes AR. Laugier and Hunziker pigmentation: a lentiginous proliferation of melanocytes. J Am Acad Dermatol. 2004;50(5):70-74. PubMed CrossRef
Koch SE, LeBoit PE, Odom RB. Laugier-Hunziker syndrome. J Am Acad Dermatol. 1987 Feb 28;16(2):431-434. PubMed
Simionescu O, Dumitrescu D, Costache M, Blum A. Dermatoscopy of an invasive melanoma on the upper lip shows possible association with Laugier–Hunziker syndrome. J Am Acad Dermatol. 2008;59(5):S105-S108. PubMed CrossRef
Tacastacas JD, Bray J, Cohen YK, et al. Update on primary mucosal melanoma. J Am Acad Dermatol. 2014;71(2):366-375. PubMed CrossRef
Posch C, Moslehi H, Sanlorenzo M, et al. Pharmacological inhibitors of c-KIT block mutant c-KIT mediated migration of melanocytes and melanoma cells in vitro and in vivo. Oncotarget. 2016;7(29):45916-45925. PubMed CrossRef
Alexis JB, Martinez AE, Lutzky J. An immunohistochemical evaluation of c-kit (CD-117) expression in malignant melanoma, and results of imatinib mesylate (Gleevec) therapy in three patients. Melanoma Res. 2005;15(4):283-285. PubMed
Shibusawa Y, Tamura A, Mochiki E, Kamisaka K, Kimura H, Ishikawa O. c-kit Mutation in generalized lentigines associated with gastrointestinal stromal tumor. Dermatology. 2004;208(3):217-220. PubMed CrossRef
Costa JJ, Demetri GD, Harrist T, Dvorak AM, Hayes DF, Merica EA, Menchaca DM, Gringeri AJ, Schwartz LB, Galli SJ. Recombinant human stem cell factor (kit ligand) promotes human mast cell and melanocyte hyperplasia and functional activation in vivo. J Exper Med. 1996;183(6):2681-2686. PubMed CrossRef
Vachiramon V, McMichael AJ. Approaches to the evaluation of lip hyperpigmentation. Int J Dermatol. 2012;51(7):761-770.
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