Laugier-Hunziker syndrome

Abstract

Laugier–Hunziker syndrome is a rare acquired disorder characterized by diffuse hyperpigmentation of the oral mucosa and longitudinal melanonychia in adults. They appear as macular lesions less than 5 mm in diameter. Laugier–Hunziker syndrome is considered to be a benign disease with no systemic manifestation or malignant potential. Therefore, it is important to rule out other mucocutaneous pigmentary disorders that do require medical management. Prompt clinical recognition also averts the need for excessive and invasive procedures and treatments. In India, the reported cases of this syndrome are very few. We provide a review of literature on Laugier–Hunziker syndrome with its differential diagnosis.

The role of STK 11 gene testing in individuals with oral pigmentation

“…Around 60% of individuals with LHS also have longitudinal melanonychia . In contrast to PJS, there are no known systemic complications of LHS and no evidence of an increased cancer risk; therefore a correct diagnosis avoids the requirement for surveillance . There does not appear to be a consistent familial pattern of LHS and to date a genetic basis for this dermatosis has not been elucidated.…”

Laugier-Hunziker syndrome: A diagnostic dilemma?

“…There was no history of tobacco addiction, drug intake, similar lesions in family members or systemic complaints. On examination, multiple well-defined light-brown-to-black macules up to 1 cm in size were observed over labial mucosa, buccal mucosa, hard palate, distal phalanges of hands, soles, and prepuce of penis, with varied patterns of nail pigmentation, as noted in 1970 under the term "idiopathic lenticular mucocutaneous syndrome" was light-to-dark-brown blotchy macules less than 5 mm, limited to fingertips, soles and mucosal surfaces, with striking involvement of nail folds exhibiting a pseudo-Hutchinson's sign [5,6] . The nail involvement is described as four types: single 1-2 mm longitudinal streaks, double 2-3 mm lateral longitudinal streaks, radial or ulnar half homogenous pigmentation, and complete pigmentation [7] .…”

Laugier-Hunziker syndrome: a rare disease entity

“…One case of familial LHS in a mother and two daughters has been reported [4]. The pigmentation occurs after an unknown trigger that leads to increased formation of melanosomes and their transport to the basal cells [5].…”

“…More than half of patients have involvement of nails in the form of longitudinal, partial or complete melanonychia [5]. Pseudo-Hutchinson sign is said to be present when melanonychia is accompanied by pigmentation of the proximal nail fold [7].…”