Laugier–Hunziker syndrome: a report of three cases and literature review

Abstract

Laugier–Hunziker syndrome (LHS) is an acquired pigmentary condition affecting lips, oral mucosa and acral area, frequently associated with longitudinal melanonychia. There is neither malignant predisposition nor underlying systemic abnormality associated with LHS. Herein, we present three uncommon cases of LHS with possibly new feature of nail pigmentation, which were diagnosed during the past 2 years. We also review the clinical and histological findings, differential diagnosis, and treatment of the syndrome in published literature.

The Laugier-Hunziker Syndrome

“…Diagnosis of PJS is made based on the presence of polyps in the gastrointestinal tract and a family history of the disorder, neither of which was present in our patient. Addison's disease, an endocrine disorder caused by insuffi cient production of cortisol and aldosterone, is characterized by increased pigmentation of the knuckles, skin creases, and mucous membranes and should also be considered in the diff erential (5). Other systemic fi ndings include hypotension, dehydration, and abdominal pain, which were all absent in our patient.…”

“…With the lack of systemic symptoms and a prior colonoscopy showing no evidence of polyps, PJS and Addison's disease were ruled out. LHS is known to be a benign disorder with no systemic manifestations and no increased risk for malignancy (5). Treatment for LHS is usually sought for cosmetic reasons, and possible therapeutic options include laser therapy and cryosurgery (2).…”

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

“…LHS perioral macules are also dark brown to black in colour; the condition is predominant in women, and often begins in the third to fifth decade of life . Approximately 180 cases have been reported since it was first described in 1970, and it is mostly a diagnosis of exclusion . Around 60% of individuals with LHS also have longitudinal melanonychia .…”

Pigmented lesions on the mucosa: a wide range of diagnoses

“…22,23 Men tend to be more commonly affected than females. The oral melanoma develops commonly between the fourth and seventh decades 24 Our patient presented with multiple focal pigmented lesions in the oral mucosa and a single longitudinal melanonychia.…”