Laugier-Hunziker Syndrome Revisited

Sir,

Laugier–Hunziker syndrome (LHS) is an acquired, benign pigmentary disease of oral cavity and lips along with longitudinal melanonychia.[1] We hereby report a case of a young female with multiple melanotic macules over the tongue and palms, along with multiple streaks of longitudinal melanonychia.

A 24-year-old, otherwise healthy, female was referred for the evaluation of progressively increasing dark-colored lesions on the lips, palms, and nails, present over the preceding 10 years. There was no history of abdominal pain, vomiting, melena, palpitation, weakness, etc. Cutaneous examination showed multiple hyperpigmented macules coalescing to form patches over the palms, neck, and upper chest. Besides, a diffuse macular pigmentation was noted over the dorsum of the tongue. In addition, there were multiple streaks of longitudinal pigmentation on the fingernails, with the pigmentation extending proximally to involve the nail folds as well. Pseudo-Hutchinson sign was positive [Figures 1 and 2]. Ocular mucosa, genitalia, and toenails were spared. Histopathological examination showed hyperkeratosis, mild acanthosis, interwoven rete ridges, increased basal layer pigmentation with an increased number of normal melanocytes [Figure 3]. Routine laboratory investigations including complete hemogram, blood smear, renal profile, and hepatic profile were within normal limits. Serum, urine cortisol, adrenocorticotropic hormone (ACTH), and serum Vitamin B12 levels were absolutely normal. The patient was not on any drugs which could have caused the pigmentation. Upper gastrointestinal endoscopy and colonoscopy did not reveal any abnormality. Chest X-ray and abdominal ultrasound showed no pathological findings. A diagnosis of LHS was made, and the patient was counseled about the benign nature of the disease.

Figure 1.

(a) Multiple melanotic macules coalescing to form patches over the palms. (b) Note the presence of longitudinal melanonychia. Pseudo-Hutchinson sign is positive

Figure 2.

(a) Diffuse macular pigmentation over the dorsum of the tongue. (b) Note the hyperpigmentation over the neck and upper chest

Figure 3.

Photomicrograph showing hyperkeratosis, mild acanthosis, interwoven rete ridges, increased basal layer pigmentation with an increased number of normal melanocytes (H and E, ×10)

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. As in our case, this longitudinal melanonychia may be associated with pigmentation of the proximal nail fold which is termed as pseudo-Hutchinson sign. Histology shows increased basal layer pigmentation with or without an increased number of normal melanocytes. Gencoglan et al. described the dermoscopic findings in a case of LHS. Lips and genitalia revealed a parallel pattern. Longitudinal homogeneous pigmentation was present on the toenails. However, the melanotic macules on the palms and the soles showed a parallel furrow pattern.[5]

The differential diagnoses include lichen planus, Addison disease, Peutz–Jegher syndrome (PJS), Cronkhite–Canada syndrome, smoking, drugs, heavy metal exposure, acquired immune deficiency syndrome, LAMB syndrome, and LEOPARD syndrome. Smoking usually leads to pigmentation of the anterior part of the oral cavity, and this is not associated with pigmentary changes of nails. Addison disease is associated with raised levels of ACTH. Bandler syndrome is characterized by pigmentation of lips, nails, and oral mucosa and gastrointestinal bleeding due to intestinal vascular malformations. PJS has a tendency to run in families and the presence of multiple perioral melanotic macules with gastrointestinal bleed, is characteristic. Besides, there are cases which show overlapping features of both LHS and PJS. We could not perform a genetic analysis due to the lack of infrastructure. LHS can be associated with esophageal melanocytosis, actinic lichen planus, hypocellular marrow, thrombocytopenia, pancreatic cancer, etc.

Therapeutic options include cryosurgery, neodymium-doped: Yttrium aluminum garnet laser, Q-switched alexandrite laser, and erbium: Yttrium aluminum garnet laser.[4] The interesting features of our case are sparing of lips, multiple streaks of longitudinal melanonychia, and presence of pseudo-Hutchinson sign, which prompted the present documentation.

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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.