Dermatology Online Journal

Laugier Hunziker syndrome
Ali Jabbari MD PhD, Mercedes E Gonzalez MD, Andrew G Franks Jr MD, Miguel Sanchez MD
Dermatology Online Journal 16 (11): 23

Department of Dermatology, New York University, New York, New York

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Abstract

Laugier Hunziker syndrome is a rare disorder that is characterized by adult-onset hyperpigmented macules of the lips, oral cavity, and fingertips. Longitudinal melanonychia is present in the majority of cases. We present a 45-year-old woman with adult-onset hyperpigmented macules of the oral cavity as well as linear melanonychia that involved multiple fingernails. The history, clinical examination, and paucity of laboratory abnormalities or systemic findings support a diagnosis of Laugier Hunziker syndrome.

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History

Figure 1	Figure 2

A 45-year-old woman was referred to the Dermatology Clinic at Bellevue Hospital Center in September, 2009, for evaluation of hyperpigmented macules of the fingernails and tongue. The lesions first appeared approximately four years prior to presentation and they had been getting darker for the last five months. Additionally, she presented with her brother and daughter, both of whom had similar findings of the tongue.

Past medical history included gastroesophageal reflux disease, type II diabetes mellitus, and hyperlipidemia. Medications included atorvastatin, metformin, and esomeprazole. She reported no known drug allergies.

Physical examination

On the lateral aspects of the tongue, numerous brown and dark brown macules were present. On the first, second, and third digits of the left hand, linear melanonychia was noted.

Laboratory data

Hemoglobin was 11.5 g/dL, hematocrit 25.3 percent, and mean corpuscular volume 77.5 fL. A basic metabolic panel was normal.

Histopathology

None

Comment

Laugier and Hunziker first reported a series of five patients with hyperpigmented macules of the mouth and lips; two of them had pigment changes of the fingernails [1]. Since that time, about 100 cases have been described in the literature. Affected patients have presented between the second to the ninth decades of life. Both men and women are affected. Case reports have included related family members [2]; the condition has been variably reported as sporadic [3, 4, 5], and inherited in an autosomal dominant fashion [2].

The clinical features of the syndrome include melanotic hyperpigmented macules of the oral cavity and lips [6], linear, longitudinal melanonychia of the fingernails [7], and hyperpigmented macules of the distal aspects of the fingers. Onset is usually early or in mid adult life. Hutchinson sign, which is defined as the extension of pigment onto the proximal nail fold, has been associated with Laugier Hunziker syndrome [8]. Additionally, cases have been described that include involvement of the interdigital areas [5], vulva [9], penis [10, 11], anal mucosa [12], pretibial area [13], and conjunctiva [2, 10].

Case reports have described Laugier Hunziker syndrome with esophageal melanocytosis [14], actinic lichen planus [15], hypocellular marrow and thrombocytopenia [16], invasive melanoma [17], and lupus erythematous [18]. The relationships with these conditions have not been well established.

The histopathologic description of the mucosal macules of Laugier Hunziker syndrome includes normal numbers and appearance of melanocytes although some case reports have described increases in the numbers of melanocytes [6]. There is accumulation of pigment in the basal epidermal layer. Melanophages can be observed in the reticular dermis. Nevus cells have not been reported. Electron-microscope examination has confirmed the presence of large numbers of mature melanosomes in the stratum basale [19].

The differential diagnosis for Laugier Hunziker syndrome includes drug ingestion (phenytion, antimalarials, clofazimine, zidovudine, and phenothiazine), Albright syndrome, Addison disease [20], and Peutz Jeghers syndrome (PJS). In PJS, hyperpigmented macules of the oral mucosa and acral skin are either congenital or appear in early life. This fact contrasts with the acquisition of characteristic skin lesions in Laugier Hunziker syndrome, which usually occur after puberty. Furthermore, PJS is associated with hamartomatous polyps of the gastrointestinal tract whereas Laugier Hunziker syndrome is not. Lastly, the distribution of skin lesions may be different between these diseases; PJS rarely involves the tongue or palate. Fingernail involvement in PJS has been reported in a few cases whereas it is observed in approximately 60 percent of cases of Laugier Hunziker syndrome [21]. Although the patient presented here did not undergo colonoscopic or esophagogastroduodenoscopic evaluation, the appearance of her skin lesions in mid adulthood and the fingernail involvement support a diagnosis of Laugier Hunziker syndrome over PJS.

Treatment for the hyperpigmented macules in Laugier Hunziker syndrome include cryosurgery [22], Q-switched Nd:YAG [23], and Q-switched alexandrite lasers [24, 25].

References

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