Laugier-Hunziker syndrome is a rare and benign disorder characterized by hyperpigmentation of the lips and buccal mucosae with associated longitudinal melanonychia of nails. Clinical correlation is needed to rule out other pigmentary disorders.

Labial and oral melanotic macules are commonly encountered in a broad range of conditions ranging from physiologic pigmentation to a sign of an underlying life-threatening disease. Although Laugier-Hunziker syndrome (LHS) shares some features of labial and oral pigmentation with a variety of conditions, it is a benign and acquired condition, frequently associated with longitudinal melanonychia. Herein, the demographic, clinical, dermoscopic, and pathological aspects of LHS were reviewed comprehensively. The important differential diagnoses of mucocutaneous and nail pigmentation are provided. An accurate diagnosis is crucial to design a reasonable medical strategy, including management options, malignant transformation surveillance, and psychological support. It is important that clinicians conduct long-term follow-up and surveillance due to the potential risks of malignant transformation and local severe complications in some conditions.

Laugier-Hunziker syndrome (LHS) is a rare acquired disorder characterized by macu-lar pigmentation of the lips and oral mucosa, with frequent longitudinal melanonychia. Involvement of other areas, such as the genitalia and fingers, has rarely been described. LHS is a benign condition with no known systemic manifestations. We report the case of a woman who developed melanotic macules on her fingers and elbow 16 years after the onset of pigmentation of her lips. This unusual feature of LHS in our patient was associated with irritant contact dermatitis and viral warts. Only two cases of an association with an inflammatory dermatosis have been reported previously in the literature.

Laugier-Hunziker syndrome is a rare acquired disorder characterized by diffuse pigmented macules of the oral mucosa. Other mucosal and cutaneous surfaces may be affected, and nail involvement is frequent. A case of Laugier-Hunziker syndrome in a Greek female with multiple buccal and labial mucosal lesions and unusual conjunctival involvement is presented here. In addition, a thorough review of the English language literature of this rather under-recognized syndrome is included and the main differential diagnostic possibilities are discussed.

Laugier-Hunziker syndrome is a rare acquired disorder characterized by diffuse hyperpigmentation of the oral mucosa and longitudinal melanonychia in adults. They appear as macular lesions less than 5 mm in diameter. Laugier-Hunziker syndrome is considered to be a benign disease with no systemic manifestation or malignant potential. Therefore, it is important to rule out other mucocutaneous pigmentary disorders that do require medical management. Prompt clinical recognition also averts the need for excessive and invasive procedures and treatments. In India, the reported cases of this syndrome are very few. We provide a review of literature on Laugier-Hunziker syndrome with its differential diagnosis.

Congenital hyperpigmentation in the acro-genital area and simultaneous occurrence of ganglioneuroma in the esophagus have yet to be reported. Herein, we report a 4-month-old girl presenting with feeding difficulty by esophageal ganglioneuroma and symmetrically distributed brown pigmented patches on the lips, axillae, dorsa of fingers and toes, and genital area. Although the esophageal stricture was resolved by surgical removal of ganglioneuroma, her skin manifestations remained for over 2 years.

Laugier-Hunziker-Baran syndrome represents a rare acquired pigmentary disorder which has no relevance to internal disorders and has no familial association. There are few reports on histopathologic studies of this syndrome concerning Japanese individuals. The differential diagnosis of oral and pigmented lesions between Laugier-Hunziker-Baran syndrome and other disorders, Peutz-Jeghers syndrome in particular, requires our utmost consideration.

Biopsy specimens of 2 cases were taken from pigmented maculae on the lower lips, buccal mucosa, tongue, and palate.

Similar histopathologic findings were observed for all locations. The histopathologic examination showed that there was an accumulation of melanin in the basal layer as well as an increase in the number of melanophages in the subepithelial area.

Oral scientists and clinicians must be familiar with Laugier-Hunziker-Baran syndrome, because this syndrome is probably more common than is generally recognized.

Laugier-Hunziker syndrome is a rare acquired hyperpigmentation of the oral mucosa and lips which is often associated with longitudinal melanonychia. We report two patients exhibiting the classical features of Laugier-Hunziker syndrome with additional and previously unreported conjunctival and penile pigmentation.

Two patients presented with a history of progressive acquired macular pigmentation of the oral mucosa, the lips, the ocular conjunctiva and the penis. Longitudinal melanonychia was found in one patient. Microscopic features (basal epithelial melanosis, moderate acanthosis and superficial pigmentary incontinence) and ultrastructural details (increased number of normal-appearing melanosomes inside basal keratinocytes and dermal melanophages) were characteristic of Laugier-Hunziker syndrome.

These two observations illustrate the topographical variations of Laugier-Hunziker syndrome and highlight the need to expand the originally described features to include more widespread areas of hyperpigmentation.

Macular pigmentation of Laugier and Hunziker is an acquired disorder involving lips, oral mucosa, acral surfaces, nail apparatus, or a combination of these in the absence of systemic disease, and is reported to show intraepidermal melanosis without melanocytosis. We present a 69-year-old white man with typical features of this disorder, involving lips and oral mucosa, first appearing 4 years before presentation. A pigmented macule on sun-exposed lip vermilion revealed increased numbers of slightly atypical melanocytes in a lentiginous epidermal pattern. A buccal mucosa pigmented macule and adjacent nonpigmented mucosa were studied using hematoxylin and eosin staining, and S-100 and L-3,4 dihydroxyphenylalanine histochemistry. The buccal mucosa macule revealed markedly increased numbers of dendritic, L-3,4 dihydroxyphenylalanine-reactive intraepithelial melanocytes. Further studies are warranted to determine if this pigmentation disorder represents a spectrum of histopathologic change or, in fact, should be renamed mucocutaneous lentiginosis of Laugier and Hunziker.

Laugier-Hunziker syndrome (LHS) is an acquired, benign, macular hyperpigmentation of the lips and oral mucosa, often associated with pigmentation of the nails. It is a rare disorder thought to be more common than the number of reported cases would suggest. It is important to include this condition in the differential diagnosis of diffuse oral pigmentation. Here we report the first case of the Laugier-Hunziker syndrome in Scandinavia. Other conditions causing diffuse or multifocal pigmented oral lesions are discussed.

We report the unusual occurrence of 3 cases of Laugier-Hunziker syndrome in the same family.

The Laugier-Hunziker syndrome is an acquired, idiopathic, benign mucocutaneous hypermelanosis that usually occurs on the lips and oral mucosa, although it may appear at other sites. Nails are frequently involved, mainly forming longitudinal hyperpigmented bands. We report the case of a patient that presented a typical picture of this entity, nearly 1 year after the beginning of treatment with levodopa. Two years after the first lesions occurred, she developed Addison's disease. The patient suffered from a diffuse discrete hyperpigmentation (it was more remarkable on exposed areas) and an intensification of the melanotic macules that were previously noticeable before in oral and genital mucosa, fingers, toes and nails. Hormonal replacement treatment enabled the control of laboratory and general manifestations and to decrease the degree of mucocutaneous hyperpigmentation considerably, despite initial hyperpigmented lesions persisting in described areas.

Laugier-Hunziker syndrome is a benign pigmentary disorder which manifests as macular hyperpigmentation of the lips and buccal mucosa. Some patients have longitudinal pigmented bands of nails. The syndrome has no systemic associations. Two patients of this rare syndrome are reported. Disorders producing similar pigmentary changes which must be differentiated are discussed.

Oral pigmentation may be physiological or pathological in nature. It may represent a localized anomaly of limited significance or the presentation of potentially life-threatening multisystem disease. Evaluation of a patient with oral pigmentation requires a systematic approach with resource to appropriate investigations in certain circumstances. A full history of evolution of the pigmentary changes, as well as inquiring into family history, drug ingestion and systemic symptoms of concurrent disease are clearly important in the assessment. The duration, pattern, hue and distribution of colour changes can provide useful diagnostic clues. Special attention is given to newly appearing lesions, or those that have changed significantly in appearance, and biopsy may be needed to validate the clinical impression. This review should enable the reader to increase their familiarity with the assessment of oral pigmentation, the common causes of oral pigmentary change and the rarer disorders of pigmentation seen in this area. The systemic diseases that may give rise to oral pigmentation are detailed and the early signs of oral melanoma are highlighted, as well as the drugs which may cause pigmentary changes in this area and the different pattern of pigmentation they may induce.

We report the case of a 46-year-old Caucasian female presenting with mucocutaneous pigmentation on the lips, oral mucosa, hands, feet and nails, as well as on a psoriatic plaque. She was successfully treated with Q-switched Nd-Yag laser, with double frequency, for both the mucosal and cutaneous lesions.

To present a clinical, histopathological and ultrastructural study on a group of patients affected by idiopathic mucocutaneous pigmentation (Laugier-Hunziker syndrome: LHS).

Twelve patients were investigated: clinical examination, laboratory tests, and X-ray studies together with light microscopy and electron microscopy were performed in order to diagnose LHS.

All cases showed acquired, benign, macular hyperpigmentation of buccal mucosa lips and nails. Histologically, pigmentations are due to an accumulation of melanin in the basal layer keratinocytes and an increase in the number of melanophages in the submucosa and/or papillary dermis. Ultrastructurally there were increased numbers of normal-appearing melanosomes in keratinocytes of the lower epithelium. No evidence of malignant changes were detected.

The importance of this condition relates to it being included in the differential diagnoses of pigmentary disorders of the oral mucosa with associated nail involvement. It is important to recognize this acquired benign disorder to avoid unnecessary investigations and treatments.

Hutchinson's sign, periungual extension of brown-black pigmentation from longitudinal melanonychia onto the proximal and lateral nailfolds, is an important indicator of subungual melanoma. However, experience has demonstrated that Hutchinson's sign, although valuable, is not an infallible predictor of melanoma. Periungual pigmentation is present in a variety of benign disorders and, therefore, may lead to overdiagnosis of subungual melanoma. Periungual hyperpigmentation occurs in at least one nonmelanoma skin cancer, Bowen's disease of the nail unit. Hyperigmentation of the nail bed and matrix may reflect through the "transparent" nailfolds simulating Hutchinson's sign. "Pseudo-Hutchinson's sign" is a phrase coined to encompass these three simulants of Hutchinson's sign. Each represents a misleading clue to the diagnosis of subungual melanoma. Total reliance on the (apparent) presence or absence of periungual pigmentation may lead to overdiagnosis or underdiagnosis of subungual melanoma. All relevant clinical and historical information, including the presence or absence of periungual pigmentation, must be carefully evaluated in a patient suspected of having subungual melanoma. Ultimately, the diagnosis of subungual melanoma is made histologically.

Longitudinal pigmented streaks--melanonychia striata longitudinalis--are discussed from the perspective of a difficult diagnostic problem. These must be differentiated as early as possible from melanoma. The history of this subject and evaluation of patients in the context of various populations are reviewed. Difficult decisions and technical aspects of the approach are outlined. Case presentations contrast multiple different subungual pigmented lesions that enter into the differential diagnosis of these tumors. A larger number of lesions that make up this differential diagnosis are also discussed in addition to the cases. Appropriate biopsy is reviewed. The potential deformities from the biopsy are contrasted with dangers that are associated with some subungual pigmented tumors.

Four cases of Laugier-Hunziker syndrome are described. In all patients (two men and two women between 39 and 57 years of age) pigmentation of the lower lip and hard palate was found. in addition, two patients had involvement of the buccal mucosa; another patient also had pigmentation of the upper lip, the gums, the soft palate, and the fingers of both hands. Histopathologic examination demonstrated an accumulation of melanin in the basal layer keratinocytes and an increase in the number of melanophages in the papillary dermis. Ultrastructural study showed the presence of numerous mature melanosomes in the cytoplasm of the keratinocytes of the basal layer and of the melanophages in the papillary dermis. Alterations of the melanocytes were not observed.

The Laugier-Hunziker syndrome is an acquired, benign, macular hyperpigmentation of the lips and buccal mucosa. The nails are often involved with the development of melanonychia. Twenty-two previous cases have been recorded in the literature. We present details of six Caucasian patients with the Laugier-Hunziker syndrome who are the first recorded from Britain. They all had acquired, macular hyperpigmentation of the lips and buccal mucosa. In five of these patients longitudinal pigmented bands were found on the nails. None had other family members affected. Although this is the first report of British patients with this syndrome, we believe that the condition is probably more common than is generally recognized.

Longitudinal melanonychia presents a difficult clinical challenge because subungual melanoma must always be included in the differential diagnosis and because the cause of longitudinal melanonychia is usually not apparent. Accordingly, biopsy is often necessary to establish the cause. This review attempts to expedite management by providing suggestions for the examination of patients with this disorder. The causes of longitudinal melanonychia are enumerated and clues to arrive at the various causes are discussed. Similarities between longitudinal melanonychia and subungual melanoma are discussed in an effort to clarify their differences and similarities; clues to the diagnosis of subungual melanoma are also discussed. Various biopsy techniques applicable to longitudinal melanonychia are considered and the indications for different surgical approaches are emphasized. The importance of the pathologist in interpreting nail biopsy specimens is emphasized.