Laugier-Hunziker Syndrome Research Articles

Pigmented Fungiform Papillae of the Tongue: A Single-center Experience and Review of Literature.

Pigmented fungiform papillae of the tongue (PFPT) is a rare benign pigmentary disorder of the tongue. In dark-skinned individuals, PFPT appears to be relatively common. However, limited data exist on PFPT in Korean patients. We aimed to investigate the clinical characteristics of PFPT in Korean patients. Patients diagnosed with PFPT between 1995 and 2021 at the Pusan National University Hospital were included. Clinical characteristics of PFPT, dermoscopic findings, and comorbidities were reviewed. A total of 19 patients diagnosed with PFPT were enrolled. The male to female ratio was approximately 1:5. The mean age at diagnosis was 41.1 years (range, 8~67 years). According to Holzwanger's classification, Type I was the most common (89.5%). PFPT was commonly concomitant with pigmentary disorders, including mucosal melanotic macules, Laugier-Hunziker syndrome, melasma, and melanonychia (6/19, 31.6%). Preceding oral infection or inflammatory lesions were found in four patients (21.1%), and systemic diseases and infectious diseases existed in two patients (10.5%). Dermoscopic examination was performed in seven patients; pigmented border with dichotomized vessels (rose petal pattern, 71.4%) and diffuse pigmentation (cobblestone pattern, 71.4%) were common findings. Our study shows PFPT can coexist with pigmentary disorders. Concomitant pigmentary disorder shows an association with sex hormone or susceptibility to abnormal pigmentation may be a possible cause of PFPT.

Laugier–Hunziker syndrome: A case report

Laugier–Hunziker syndrome (LHS) is a rare, benign pigmentary disorder characterized by unique pigmentation over mucosa, nails, and acral sites, arising spontaneously during adulthood. It has no malignant potential or systemic involvement. It has a female preponderance (2:1 ratio). The pigmentary lesions in LHS respond poorly to therapy, and has high recurrence. Since LHS is a diagnosis based on exclusion, we discuss the differential diagnosis of mucocutaneous hyperpigmentation. We present a case of a 66-year-old man with LHS.

DIAGNOSTIC QUANDARY WITH LAUGIER-HUNZIKER SYNDROME

Laugier-Hunziker syndrome being a rare pigmentation disorder chiefly involving lips and nails has so far been coincidentally diagnosed. In Nepal, Laugier-Hunziker syndrome has not been reviewed very well, and no case has been reported in the literature till date. To the best of our knowledge, the current report is the only one in the Nepalese scientific community but addressing the age of the patient it stands with a diagnostic quandary.

Laugier-Hunziker syndrome: a rare case report and review of the literature

Laugier-Hunziker syndrome is a rare, hereditary pigmentary disorder characterized by mucocutaneous lentiginous lesions and melanonychia. The condition is regarded as benign since underlying malignancies or systemic disorders were not reported. Since various malignancy syndromes including Peutz-Jeghers and Cronkhite-Canada syndrome and systemic cause of hyperpigmentation such as Addison disease and drugs are presented with similar mucocutaneous findings, it is of paramount importance to assess the patients to exclude aforementioned conditions.

Nail changes associated with pigmentary disorders

Salient nail unit changes serve as pointers toward systemic disease. On similar lines, salient nail changes are associated with pigmentary disorders, and can help toward diagnosis. Nail changes form a part of various physiologic pigmentary changes including racial pigmentation, pregnancy, or neonatal period. At the same time, a number of nail changes can be valuable clues toward disorders of pigmentation associated with genetic syndromes (Laugier–Hunziker syndrome, incontinentia pigmenti, and tuberous sclerosis), endocrine disorders, disorders of metabolism (alkaptonuria), infections (HIV-AIDS), internal malignancies, etc. Nail changes are especially useful if cutaneous pigmentary changes are suspected to be drug-induced based or associated mucosal and/or cutaneous hyperpigmentation. Various drugs that can cause cutaneous pigmentary alterations along with nail changes include cyclophosphamide, adriamycin, vincristine, docetaxel, paclitaxel, carboplatin, psoralens, hydroxyurea, bleomycin, etc. Nail changes are also associated with disorders of hypomelanosis, for example, vitiligo. Pigmentary changes due to endogenous and exogenous pigments are again diagnosable based on the nail changes they induce. This narrative review highlights the importance of nail changes in suspecting and diagnosing various disorders of pigmentation. It also points toward areas which need future research.

Proposed diagnostic criteria and classification for Laugier-Hunziker Syndrome

Laugier-Hunziker syndrome is one of those rare syndromes which has yet not been reviewed and reported much in literature. So far, there is no diagnostic criteria and classification that can help with prompt diagnosis of this syndrome. In this article an attempt has been made to put forward a simple criterion along with a classification that can assist the practitioners with early detection and also will have substantial contribution in medical curriculum.

PROPOSED DIAGNOSTIC CRITERIA AND CLASSIFICATION FOR LAUGIER-HUNZIKER SYNDROME

Laugier-Hunziker syndrome is one of those rare syndromes which has yet not been reviewed and reported much in literature. So far, there is no diagnostic criteria and classification that can help with prompt diagnosis of this syndrome. I have attempted to device simple criteria along with a classification that can assist the practitioners with early detection and also will have substantial contribution in medical curriculum.

The dermoscopic manifestation of nail pigmentation

Background and objectives: Nail pigmentations are of diagnostic challenging for dermatologists, as they may be involved in many local and systemic diseases that difficult to diagnose clinically. Our aim is to assess the specific dermoscopic features of different pigmented nail lesions. Methods: In our cross-sectional descriptive study; the total number of 46 patients with different types of nail pigmentation from all age groups and both sexes were included. Results: A total number of 46 patients, fungal melanonychia (n=14) was the commonest nail pigmentation found. In which the most common dermoscopic features were wide yellow streaks (100%). In drug-induced pigmentation (n=9) fine regular grey lines on a homogenous grey background were found. In Subungual hemorrhage (n=7) blood spots was the most common feature (100%). Lentigo (n=4) were associated with thin regular longitudinal grey lines on homogenous brown background. Nail melanocytic nevus (n=3) multiple, regular brown lines on the homogenous brown background was commonly found. In pseudomonal infection (n=5) bright green color mixed with dark green the most obvious feature. In nail-biting and in Laugier-Hunziker syndrome greyish coloration of background with thin longitudinal grey lines was the common feature. In melanoma (n=1) the dermoscopy showed the brown coloration of the nail plate background with irregular brown-black lines. Conclusions: Onychoscopy is a non-invasive device that collects much information that helping in the diagnosis of nail pigmentation.

Orofacial pigmentary lesions in the pediatric age group: A descriptive observational study

Background: Pigmentary disorders on the face are frequently encountered in all age groups and both genders but are more common in pediatric patients. These lesions can be isolated incidental findings or indicative of a congenital or acquired systemic or genetic disorder. Aims and Objectives: The aim and objective of this study is to study the frequency and pattern of pigmented orofacial lesions in the pediatric age group and to describe the underlying associated condition if any. Materials and Methods: A descriptive study was conducted over a period of 6 months (June–November 2018) in the dermatology department of a tertiary care hospital in Eastern Uttar Pradesh. All the children from 2 to 14 years of age who reported to us with complaints of pigmentary lesions on the face or oral cavity were included, and attempt was made to find out if any underlying conditions. Results: A total of 207 children were included in the study. Pityriasis alba was the most common pigmentary disorder on the face in the study population in 146 (70.5%) children, while pityriasis versicolor was noted in 21 (10.1%). Other facial dermatoses noted were vitiligo, congenital melanocytic nevus, and postinflammatory hypopigmentation and hyperpigmentation. Eight (3.8%) children had associated underlying diseases such as xeroderma pigmentosum, dyskeratosis congenita, Vitamin B12 deficiency, Laugier–Hunziker syndrome, and juvenile dermatomyositis. Conclusions: Pigmentary disorders of the face and oral cavity are frequently seen in children which forms a major part of dermatology consultation. Although most of these disorders are of cosmetic concerns, few conditions are associated with systemic conditions. A thorough investigation and management are the keys to manage such cases.

Laugier–Hunziker syndrome: A rare cause of diffuse orofacial pigmentation

Laugier–Hunziker syndrome (LHS) is lesser known and important differential of the multifocal mucocutaneous pigmentation. Essentially idiopathic in nature, this syndrome is characterized by multiple melanotic macules on the oral mucosa, lips, perioral region, and melanonychia. A young Indian male reported to us with a complaint of multiple brown-black spots on the lips and inner surface of the cheeks accompanied by the pigmentation of fingernail. The patient was evaluated for the possible causes of multifocal oral pigmentation. After taking an exhaustive clinical history and thorough investigations, no exact cause for this condition could be definitively ascertained. Eventually, he was diagnosed as a case of LHS, a rare cause of idiopathic oral pigmentation. The various differential diagnoses for the condition are also discussed.

Black Maculae between the Toes: A Rare Form of Laugier-Hunziker Syndrome?

Black Maculae between the Toes: A Rare Form of Laugier-Hunziker Syndrome?

MULTIPLE ORAL MUCOSA PIGMENTATION: IMPORTANCE OF CLINICAL ASPECTS AND MEDICAL INVESTIGATION

Solitary and multiple macules of the oral mucosa are associated with various causes. These lesions may represent different pathologic entities, thus requiring different treatments and follow-up. We present 2 clinical cases of patients with multiple pigmentations on oral mucosa. Case 1: A 31-year-old woman exhibited brown hyperpigmentation macules less than 5 mm in diameter affecting buccal mucosa and tongue borders. Homogeneous hyperpigmentation stripes on nails were also identified. Based on these characteristics and the absence of gastrointestinal signs and symptoms, the diagnosis of Laugier-Hunziker syndrome was established. Case 2: A 68-year-old woman showed multiple pigmented lesions on the tongue. Clinical hypotheses were Peutz-Jeghers syndrome and Laugier-Hunziker syndrome. Medical investigation revealed chronic abdominal pain history, gastrointestinal bleeding, and previous surgery for excision of polyps. In addition, multiple pigmented skin lesions were detected except on the nails, leading to the final diagnosis of Peutz-Jeghers Syndrome. Both patients remain under follow-up. Solitary and multiple macules of the oral mucosa are associated with various causes. These lesions may represent different pathologic entities, thus requiring different treatments and follow-up. We present 2 clinical cases of patients with multiple pigmentations on oral mucosa. Case 1: A 31-year-old woman exhibited brown hyperpigmentation macules less than 5 mm in diameter affecting buccal mucosa and tongue borders. Homogeneous hyperpigmentation stripes on nails were also identified. Based on these characteristics and the absence of gastrointestinal signs and symptoms, the diagnosis of Laugier-Hunziker syndrome was established. Case 2: A 68-year-old woman showed multiple pigmented lesions on the tongue. Clinical hypotheses were Peutz-Jeghers syndrome and Laugier-Hunziker syndrome. Medical investigation revealed chronic abdominal pain history, gastrointestinal bleeding, and previous surgery for excision of polyps. In addition, multiple pigmented skin lesions were detected except on the nails, leading to the final diagnosis of Peutz-Jeghers Syndrome. Both patients remain under follow-up.

Clinical manifestations and management of Laugier-Hunziker syndrome

Clinical manifestations and management of Laugier-Hunziker syndrome

Levodopa-induced skin disorders in patients with Parkinson disease: a systematic literature review approach.

The use of levodopa for treatment of Parkinson's disease is a well-established clinical practice. Data about the true incidence and severity of cutaneous complications associated with the use of levodopa are largely lacking. Aim of this review was to evaluate the quality of evidence referring to the skin disorders caused by levodopa treatment for Parkinson's disease. Thirty of 1084 studies were included; 8 randomized controlled trials and 22 case reports in a total of 2749 patients. Malignant melanoma was the most frequent oral levodopa-related skin disorder followed by allergic cutaneous reactions, alopecia, vitiligo, skin hyperpigmentation, Laugier-Hunziker syndrome, Henoch-Schönlein syndrome, pseudobullous morphea and scleroderma-like illness. Naranjo scores ranged from 2 to 8. Regarding levodopa clinical trials, the most frequent skin complication was peripheral edema, followed by malignant melanoma. Although evidence is not robust, melanoma is the most frequent and possible fatal levodopa-associated skin disorder, while other skin allergic or immunological reactions are less common and reversible. Although levodopa treatment may induce melanogenesis and promote melanomagenesis, existing evidence does not support an association between levodopa therapy and induction or progression of malignant melanoma. The suggested association with melanoma may reflect the well-documented association of Parkinson's disease with melanoma rather than the exposure to the drug. Nevertheless, until a solid conclusion can be drawn, the use of levodopa in the context of malignant melanoma should be considered with caution. Well-designed prospective studies are needed to determine the cause and effect relationship between levodopa and skin disorders.

Melanonychia striata: clarifying behind the Black Curtain. A review on clinical evaluation and management of the 21st century.

Melanonychia striata is characterized by a tan, brown, or black longitudinal streak within the nail plate that runs from the proximal nail fold to the distal part of the nail plate. Melanonychia striata is due to increased activity of melanocytes or melanocytic hyperplasia in the nail matrix with subsequently increased melanin deposition in the nail plate. The most common cause of melanonychia striata associated with melanocytic activation is ethnic melanonychia which occurs in dark-skinned individuals. Other causes of melanonychia striata related to melanocytic activation include pregnancy, chronic local trauma, infections, medications, dermatological disorders, endocrine disorders, alkaptonuria, hemochromatosis, porphyria, graft-vs-host disease, Peutz-Jeghers syndrome, and Laugier-Hunziker syndrome. Causes of melanonychia striata associated with melanocytic hyperplasia include nail matrix melanocytic nevus, nail lentigo, and nail apparatus/subungual insitu and invasive melanoma. In most cases, melanonychia striata is a benign condition, especially in children. Consequently, most investigators advocate a wait-and-see approach. Nail apparatus/subungual melanoma should be suspected if there is an abrupt onset after middle age, personal or family history of melanoma, rapid growth, darkening of a melanonychia band, pigment variegation, blurry lateral borders, irregular elevation of the surface, a bandwidth >3mm, proximal widening, associated nail plate dystrophy, single rather than multiple digit involvement, and periungual spread of pigmentation onto the adjacent cuticle and/or proximal and/or lateral nail folds (Hutchinson sign). Prolonged follow-up is mandatory for early detection of possible malignant changes.

Mystery behind labial and oral melanotic macules: Clinical, dermoscopic and pathological aspects of Laugier-Hunziker syndrome.

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 in endocrine clinical practice.

SummaryLaugier–Hunziker syndrome (LHS) is a rare, benign and acquired disorder characterized by hyperpigmentation of the oral cavity and lips along with longitudinal melanonychia. No underlying systemic abnormalities or malignant predisposition is associated with it. In everyday clinical practice, an endocrinologist encounters certain endocrine conditions (e.g. Addison’s disease, McCune–Albright syndrome) that present with, inter alia, mucocutaneous hyperpigmentation. Even though LHS is easily distinguished from endocrine entities mentioned earlier, diagnostic evaluation usually requires skilled and thorough practitioner. Since it is the diagnosis of exclusion, a number of systemic conditions must be ruled out prior to making the final diagnosis. However, its major differential diagnosis is primarily Peutz-Jeghers syndrome, which carries an increased risk of cancer. Here, we report a case of a young woman who was referred to the endocrinologist for diagnostic evaluation of dark-colored lesions of the oral cavity and nails. All performed laboratory tests were within reference range. Endoscopic gastrointestinal evaluation did not reveal neoplastic formations. Owing to an adult-onset, asymptomatic clinical course and negative diagnostic findings, we made a final diagnosis. In this case, target diagnostic evaluation notably reduced the need for additional expensive and invasive procedures and treatments.Learning points:Laugier–Hunziker syndrome is a rare, acquired cause of asymptomatic, benign mucocutaneous hyperpigmentation.Prior to making a final diagnosis, certain medical entities with overlapping clinical features must be excluded.Endocrine conditions that usually present with the hyperpigmentation of the skin and mucous membranes (e.g. Addison’s disease, McCune–Albright syndrome) can be easily ruled out based on clinical and laboratory findings.Its major differential diagnosis, Peutz-Jeghers syndrome is characterized by melanotic macules of the face and mouth, intestinal polyposis and significantly increased risk of different types of cancer, especially gastrointestinal.Anamnesis, physical examination and target diagnostic evaluation reduce the need for additional invasive and expensive procedures and treatment.

Case Report: Dermoscopic features of oral lichen planus - the evolution of mucoscopy

Dermoscopy, a non-invasive technique for cutaneous diagnosis is being increasingly studied in various disorders of the skin, nails and scalp. However, it has been under-utilized for the diagnosis and characterization of mucosal disorders. The dermoscopic characterization of cutaneous lichen planus and its variants has been well documented with Wickham’s striae constituting the hallmark of the condition. However, the dermoscopic features of oral lichen planus with hand-held or videodermoscopy remain to be elucidated. We present the case of a young adult man who presented with asymptomatic white lacy lesions over a bluish-black background over the tongue, patchy hyperpigmentation of the buccal mucosae and gingivae, and longitudinal melanonychia involving some nails. History of intake of any drugs preceding the lesions, smoking, chewing of betel nut and dental implants was negative. Family history was non-contributory. There were no cutaneous lesions suggestive of lichen planus. Mucoscopy (dermoscopy of the mucosa, oral in this case) and onychoscopy were done followed by biopsy from the tongue that confirmed the diagnosis of lichen planus. Oral mucoscopy of the tongue revealed a tri-colored pattern with structureless veil-like grey-white areas (modified Wickham’s striae), well-demarcated red glossy erosions, and violaceous-to-brown clods. Additionally, vascular pattern of dotted and linear to curved vessels along the borders of leukoplakia-like areas and erosions were observed. Onychoscopy confirmed lichen planus-associated melanonychia. Dermoscopy also proved useful in conveniently ruling out other disorders typified by mucosal and nail pigmentation such as Laugier Hunziker syndrome and drug-induced changes. Although direct oral microscopy has been used in defining features of oral lichen planus, to the best of our knowledge this case is the first report on mucoscopy or dermoscopy of oral lichen planus

Case Report: Dermoscopic features of oral lichen planus - the evolution of mucoscopy.

Dermoscopy, a non-invasive technique for cutaneous diagnosis is being increasingly studied in various disorders of the skin, nails and scalp. However, it has been under-utilized for the diagnosis and characterization of mucosal disorders. The dermoscopic characterization of cutaneous lichen planus and its variants has been well documented with Wickham's striae constituting the hallmark of the condition. However, the dermoscopic features of oral lichen planus with hand-held or videodermoscopy remain to be elucidated. We present the case of a young adult man who presented with asymptomatic white lacy lesions over a bluish-black background over the tongue, patchy hyperpigmentation of the buccal mucosa and gingivae, and longitudinal melanonychia involving some nails. History of intake of any drugs preceding the lesions, smoking, chewing of betel nut and dental implants was negative. Family history was non-contributory. There were no cutaneous lesions suggestive of lichen planus. Mucoscopy (dermoscopy of the mucosa, oral in this case) and onychoscopy were done followed by biopsy from the tongue that confirmed the diagnosis of lichen planus. Oral mucoscopy of the tongue revealed a tri-colored pattern with structureless veil-like grey-white areas (modified Wickham's striae), well-demarcated red glossy erosions, and violaceous-to-brown clods. Additionally, vascular pattern of dotted and linear to curved vessels along the borders of leukoplakia-like areas and erosions were observed. Onychoscopy confirmed lichen planus-associated melanonychia. Dermoscopy also proved useful in conveniently ruling out other disorders typified by mucosal and nail pigmentation such as Laugier Hunziker syndrome and drug-induced changes. Although direct oral microscopy has been used in defining features of oral lichen planus, to the best of our knowledge this case is the first report on mucoscopy or dermoscopy of oral lichen planus.

Oral pigmentations in Laugier-Hunziker syndrome: a case report and review of diagnostic criteria

ABSTRACT Laugier-Hunziker syndrome (LHS) is a rare mucocutaneous disorder, of unknown etiology, characterized by multiple hyperpigmented macules, dispersed mostly on the oral mucosa, occasionally associated with longitudinal ridging of the nails. The diagnosis requires exclusion of other conditions, such as Addison's disease and Peutz-Jeghers syndrome. We report a case of a 34-year-old male patient, presenting with hyperpigmented macules on the lips, buccal mucosa and palate, as well as mild dark striations on toenails. After careful clinical and laboratorial investigations, the diagnosis of LHS was established. Given the lack of aesthetic complaints and symptoms, no treatment was necessary.