Laugier-Hunziker syndrome - Case report

Abstract

Laugier-Hunziker syndrome is a rare, acquired disorder characterized by lenticular hyperpigmentation of the oral mucosa and longitudinal melanonychia. We present the case of a 63-year-old female with progressive, asymptomatic hyperpigmentation of buccal mucosa and a 7-year history of hyperpigmentation in several fingernails. Laugier-Hunziker syndrome was diagnosed based on the clinical features presented, dermoscopic findings and exclusion of underlying systemic diseases. Laugier-Hunziker syndrome is regarded as a diagnosis of exclusion. By identifying Laugier-Hunziker syndrome, other, more severe syndromes associated with hyperpigmentations can be excluded, namely Addison’s disease and Peutz-Jeghers syndrome.

Posible asociación autoinmune entre síndrome de Laugier-Hunziker y síndrome de Sjögren: reporte de un caso y revisión de la literatura

“…Si bien la asociación entre SLH y síndrome de Sjögren podría ser fortuita, existen reportes de esta patología asociada a diversas enfermedades autoinmunes en otros países, tales como lupus eritematoso cutáneo y sistémico, artritis reuma-toide, síndrome de Sjögren, anemia hemolítica con Coombs positivo, médula ósea hipocelular con anticuerpos antinucleares positivos y penfigoide bulloso [10][11][12][13][14] . La existencia de estos casos, por si solos, no son prueba suficiente para establecer una relación de causalidad, sin embargo, podrían sugerir un estímulo autoinmune para la síntesis de melanina en el SLH.…”

“…Se requiere de mayor información para determinar si el SLH es efectivamente de etiología autoinmune. Se desconoce si el hallazgo de estas patologías con SLH corresponden a hallazgos fortuitos 12 .…”

“…Revisando la literatura internacional disponible, existen al menos cinco publicaciones que relacionan SLH a autoinmunidad [10][11][12][13][14] . Lo anterior nos permite sospechar que un mecanismo autoinmune podría participar en la patogénesis de esta entidad y, por ende, requiere formular un enfrentamiento inicial acorde.…”

Laugier-Hunziker syndrome: a rare disease entity

“…There were also some depigmented macules over the glans which the patient ascribed to some painful vesicular eruption, possibly herpes genitalis in the past (Figure 2 Laugier and Hunziker first described this disease as an acquired, benign skin condition characterized by buccal mucosal pigmentation with longitudinal melanonychia [2]. The condition is also referred to as idiopathic lenticular mucocutaneous pigmentation [3]. The disease has a mean age of onset at 50 years.…”

Laugier–Hunziker Syndrome Revisited

“…LHS is also referred to as idiopathic lenticular mucocutaneous pigmentation. [ 2 3 ] Clinically, it is manifested with progressively increasing dark-brown macules over the lower lip, buccal mucosa, hard palate, tongue, gingiva, genitalia, fingers, sclera, perianal region, etc. [ 4 ] Nails are characterized by multiple pigmented streaks, most commonly in fingernails.…”