Laugier-Hunziker syndrome: a rare disease entity

Dear Editors,
Laugier-Hunziker syndrome (LHS) is a rare, acquired, idiopathic disease of benign nature characterized by pigmentation of the oral mucosa, lips and longitudinal melanonychia [1]. Around 100 cases of the disease have been reported, mostly from Europe. We report a case of LHS from India with additional penile pigmentation, making our case a unique presentation.
A 20-year-old man presented with progressive darkening of the tongue, nails, palms, soles and genitals for the last 4 months. The patient gave no history of any long-term medications, smoking, radiation exposure, precocious puberty, dark colored stools, abdominal pain, breathlessness or weakness. There was no history of any gastrointestinal or mucosal pigmentary disease in other family members. Examination revealed well-defined, coalescent hyperpigmented macules on the palms, soles and dorsal aspects of the hands but sparing the palmar creases (Figures 1 A–C). Macular, slate gray pigmentation was present over the dorsal surface of the tongue (Figure 2 A). The rest of the oral mucosa was not involved. The glans penis showed multiple, slate gray to violaceous, hyperpigmented macules in a reticulate pattern (Figure 2 B). 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 B). The fingernails showed several longitudinal streaks of melanonychia with involvement of the proximal nail folds – a positive pseudo-Hutchinson sign (Figure 1 B). Toe nails were spared. Histopathology from a hyperpigmented macule from the palm showed hyperkeratosis, acanthosis and increased basal layer pigmentation. Systemic examination was unremarkable. Routine laboratory investigations including blood count and liver and renal function tests were normal. The adrenocorticotropic hormone (ACTH) levels, serum cortisol and serum vitamin B12 levels were within the normal range. No abnormality was detected on ultrasound of the abdomen, upper gastrointestinal endoscopy and colonoscopy. Based on the above findings, a diagnosis of Laugier-Hunziker syndrome was made and the benign nature of the disease was explained to the patient.
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...


View full text...