Laugier-hunziker syndrome: A rare cause of oral and acral pigmentation

Abstract

Laugier–Hunziker syndrome (LHS) is an acquired, benign pigmentary skin condition involving oral cavity including lower lip in the form of brown black macules 1–5 mm in size, frequently associated with longitudinal melanonychia. There is no underlying systemic abnormality or malignant predisposition associated with LHS, and therefore the prognosis is good. Important differential diagnoses include Peutz Jeghers syndrome and Addison’s disease among other causes of oral and acral pigmentation. Treatment is sought mainly for cosmetic reasons and Q-switched Nd-Yag laser/ Q-switched alexandrite therapy and cryosurgery have been tried with varying success.

The Laugier-Hunziker Syndrome

“…Other systemic fi ndings include hypotension, dehydration, and abdominal pain, which were all absent in our patient. Other less common conditions that should be considered in the diff erential for hyperpigmentation include the McCune-Albright syndrome, drug-induced hyperpigmentation (tetracyclines, antimalarials, and chemotherapeutics), and the Gardener syndrome (2).…”

“…Lesions most commonly involve the buccal mucosa and lips, but the gums, tongue, palate, fi ngers, toes, neck, and abdomen can also be involved. Nails are aff ected in about 60% of cases, and fi ndings include longitudinal stripes aff ecting the nail plate (2). Th e diagnosis of LHS is frequently made clinically and is a diagnosis of exclusion (3).…”

“…Th e diagnosis of LHS is frequently made clinically and is a diagnosis of exclusion (3). Biopsy of the lesions can be performed to confi rm the diagnosis, and histopathologic changes associated with LHS show increased basal layer pigmentation with a normal number and morphologic appearance of melanocytes (2).…”

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

“…El aumento de ACTH produce una hiperpigmentación difusa de mucosas y zonas de presión (p. ej. nudillos y pliegues), así como aréolas mamarias y cicatrices, pudiendo ser esta la primera manifestación 1,6,8 . En nuestra paciente las máculas eran bien delimitadas, y se encontraba asintomática, con niveles de cortisol plasmático y ACTH dentro de rango normal.…”

“…Generalmente muestra un incremento significativo de melanina en epidermis, melanocitos de morfología normal en membrana basal (los cuales pueden estar aumentados en cantidad) e incontinencia pigmentaria en dermis papilar, con melanófa-gos [5][6][7] . El principal objetivo de una biopsia en el SLH con lesiones focales es descartar melanoma.…”