Laugier-hunziker syndrome L81.46

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 21.12.2020

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Synonym(s)

Idiopathic lenticular mucocutaneous pigmentation; Laugier-Hunziker-Baran syndrome; Pigmentation melanique lenticulaire essentielle de la muqueuse jugale et des lèvres

History
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Hunziker and Laugier, 1970; Baran, 1979

Definition
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Syndrome characterized by light to dark brown, lenticular, sometimes diffuse patches of the cheek and lip mucosa, with longitudinal nail pigmentation in 50-60% of cases.

Etiopathogenesis
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Unknown.

Histology
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Hyperpigmentation of the epithelium, melanophages in the papillary body, no nevus cells.

Differential diagnosis
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See Table 1 under Melanonychia striata longitudinalis.

Differential diagnoses of diffuse oral mucosal and lip hyperpigmentation may be congenital (in the context of systemic diseases such as Peutz-Jeghers syndrome, McCune-Albright syndrome, LEOPARD syndrome or Name syndrome) or acquired (smoking, drug use or inflammatory processes).

Note: Although Laugier-Hunziker syndrome is defined as a benign disease without malignant potential, malignant melanoma should always be considered in differential diagnosis (Lambertini M et al. 2018).

Literature
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  1. Baran R (1979) Longitudinal melanotic streaks as a clue to Laugier-Hunziker syndrome. Arch Dermatol 115: 1448-1449
  2. Haneke E (1991) Laugier-Hunziker-Baran syndrome. Dermatologist 42: 512-515
  3. Kanwar AJ et al (2001) Laugier-Hunziker syndrome. J Dermatol 28: 54-57
  4. Lambertini M et al (2018) Oral melanoma and other pigmentations: when to biopsy? J Eur Acad Dermatol Venereol 32: 209 - 214.
  5. Laugier P, Hunziker N (1970) Essential lenticular melanic pigmentation of the lip and cheek mucosa. Arch Belg Dermatol Syphiligr 26: 391-399.
  6. Lenane P et al (2001) The Laugier-Hunziker syndrome. J Eur Acad Dermatol Venereol 15: 574-577
  7. Makhoul EN et al (2003) Familial Laugier-Hunziker syndrome. J Am Acad Dermatol 49: S143-145.

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 21.12.2020