Surgical Treatment Outcome for Familial Melkersson-Rosenthal Syndrome.

BACKGROUND Recurrent facial nerve palsy, orofacial edema, and fissured tongue are a triad of manifestations that characterize a rare disorder named Melkersson-Rosenthal syndrome. It is important to consider this syndrome when diagnosing atypical, unilateral, or bilateral facial palsies with characteristics of familial prevalence. There is no established outcome prediction for this disease and the syndrome does not have a specific duration or prospective timeline. Recurrent facial paralysis can require surgery and a multidisciplinary approach with regular follow-up. CASE REPORT We describe a 38-year-old woman presenting with a third episode of facial paralysis and discuss her pedigree chart and the treatment course chosen. After conservative treatment with oral corticosteroids, antiviral therapy, and motor physical therapy with no significant improvements, the patient underwent facial nerve decompression surgery with outstanding results. Eight months after surgery and intense postoperative physical therapy, the patient improved from grade VI to grade II palsy on the House-Brackmann Scale. The patient's older brother also presented a fissured tongue and had a history of 2 episodes of facial paralysis. The patient's son, mother, and sister also presented tongue fissuring but did not have any other clinical signs of the syndrome. CONCLUSIONS Despite being rare, Melkersson-Rosenthal syndrome is associated with a family inheritance and its diagnosis has prognostic implications. Therefore, it is of the utmost importance to have suspicion of this disorder in order to improve quality of care and target the treatment accordingly. Surgical treatment in these cases seems to be an excellent choice to treat current facial paralysis and prevent further episodes.

Oral Lesions in Patients With Psoriasis: Prevalence and Association With Its Clinical and Epidemiological Characteristics. / Manifestaciones orales en pacientes con psoriasis. Prevalencia y asociación con sus características clínicas y epidemiológicas.

BACKGROUND AND OBJECTIVE: Psoriasis is a multisystem disease associated with an increased prevalence of oral lesions. The aim of this study was to determine the prevalence of oral lesions in patients with psoriasis and examine associations with clinical and patient characteristics. MATERIAL AND METHODS: We conducted a cross-sectional study of patients with psoriasis and healthy controls seen between December 2019 and February 2020. We recorded biometric data, comorbidities associated with psoriasis, oral examination findings, and clinical characteristics of psoriasis. RESULTS: We studied 100 patients with psoriasis and 100 controls. Oral lesions were more common in the psoriasis group (74% vs 46%, P<.001). The most common lesions were fissured tongue (39% vs 16%, P<.001) and periodontitis (28% vs 16%, P=.04). Geographic tongue was uncommon in both the study and the control group (4% vs 2%, P=.68). In the psoriasis group, patients with fissured tongue had a higher prevalence of cardiovascular disease (23.1% vs 4.9%), diabetes mellitus (28.2% vs 8.2%), and psoriatic arthritis (15.4% vs 1.6%) than those without this condition. Periodontitis was also associated with a higher prevalence of cardiovascular disease (28.6% vs 5.6%). Type of psoriasis, location, and time since onset were not significantly associated with oral lesions. Patients with oral lesions, however, had more severe disease (Psoriasis Area Severity Index [PASI], 3.9 vs 2.4; P=.05). Mean PASI was also higher in patients with fissured tongue (4.7 vs. 2.7, P=.03) and periodontitis (5.1 vs. 2.9, P=.04). CONCLUSIONS: The prevalence of oral lesions, especially fissured tongue and periodontitis, is higher in patients with psoriasis than in healthy controls. Oral lesions were associated with more severe psoriasis and a higher prevalence of associated comorbidities. We recommend examining the oral cavity of patients with psoriasis, especially those with more severe disease and comorbidities, irrespective of type of psoriasis, location, or time since onset.

Hidradenitis Suppurativa and Concomitant Down Syndrome: Literature Review of Other Associated Mucocutaneous Manifestations in Adults.

This contribution describes hidradenitis suppurativa (HS) occurring in Down disease that presented with morphology conforming to an overlap of stages 1 and 2 of the Hurley staging system, namely the formation of solitary or multiple isolated abscesses without scarring or sinus tracts, recurrent abscesses, and single or multiple widely separated lesions with sinus tract formation, occupying apocrine sweat gland-bearing areas: the inner thighs, groin, and buttocks. The lesions were bilateral and symmetrical, of rare occurrence. In addition, the clinical and pathognomonic features of several other concomitant diseases are defined and reviewed; these include elastosis perforans serpiginosa, fissured tongue/macroglossia, syringomas, palmoplantar keratodermas, cheilitis, xerosis, atopic dermatitis, seborrheic dermatitis, vitiligo, cutis marmorata, and alopecia areata.

Alteraciones odonto-estomatológicas en el niño con síndrome de Down / Odonto-stomatology alterations in the child with Down’s syndrome

Las personas con síndrome de Down presentan una variedad de complicaciones médicas y de características odontoestomatológicas específicas. Muchas de estas características pueden tener relación directa con la salud oral y con la calidad de vida del niño afectado. El objetivo de este artículo es revisar las manifestaciones orales, dentales y oclusales más frecuentes del niño con síndrome de Down, así como la relación de estas con la patología bucodental más frecuente. En este grupo de pacientes se ha descrito una menor prevalencia de lesiones de caries dental y una mayor frecuencia de enfermedades del periodonto con especial referencia a la enfermedad periodontal que tiene un inicio más precoz y un carácter agresivo (AU)

Persons with Down’s syndrome have a variety of medical complications and specific odonto-stomatology characteristics. Many of these characteristics may have a direct relation with oral health and the quality of life of the affected child. This article has aimed to review the most frequent oral, dental and occlusal manifestations found in the child with Down’s syndrome and their relation with the most frequent buccodental conditions. A lower prevalence of cavities and greater frequency of periodontal disease has been described in this group of patient, special emphasis being placed on the periodontal disease who onset is earlier and has an aggressive character (AU)

Benign migratory glossitis with fissured tongue.

Symptomatic benign migratory glossitis (BMG) or "geographic tongue" is a rare occurrence in pediatric dentistry though asymptomatic BMG is comparatively common. BMG presents itself as an ulcer-like region on the dorsum of the tongue, which may recur at different sites on the tongue, creating a migratory appearance. Asymptomatic cases usually resolve on their own but symptomatic cases need treatment. Fissured tongue is a benign condition characterized by deep grooves on the dorsum of the tongue and, in many cases, is associated with geographic tongue. This article presents a case of symptomatic geographic tongue with fissured tongue with a history of asthma.

Two novel de novo mutations of KRT6A and KRT16 genes in two Chinese pachyonychia congenita pedigrees with fissured tongue or diffuse plantar keratoderma.

BACKGROUND: Mutations in the KRT6A or KRT16 gene cause pachyonychia congenita type 1 (PC-1), while mutations in KRT16 or KRT6C underlie focal palmoplantar keratoderma (FPPK). A new classification system of PC has been adopted based on the mutated gene. PC rarely presents the symptoms of diffuse plantar keratoderma. Mutation in the tail domain of keratins is rarely reported. PC combined with fissured tongue has never been described. OBJECTIVES: To investigate the genotype-phenotype correlations between clinical features and gene mutational sites in two unrelated southern Chinese PC pedigrees (one family presented with specific fissured tongue, the other with diffuse plantar keratoderma). MATERIALS & METHODS: The whole coding regions of the KRT6A/KRT16/KRT17/KRT6B genes were amplified and directly sequenced to detect the mutation. To confirm the effect of the IVS8-2A>C mutation in KRT6A at the mRNA level, total RNA from the plantar lesion of a patient was extracted and reverse-transcribed to cDNA for sequence analysis. RESULTS: Two novel de novo mutations, a splice acceptor site variant IVS8-2A>C (p.S487FfsX72) in KRT6A and a heterozygous substitution c.AA373_374GG (p.N125G) within exon 1 of KRT16, were found separately in the two PC families. CONCLUSION: Genotype-phenotype correlations among PC patients with codon-125 mutation in KRT16 were established, while the phenotypes caused by the IVS8-2A>C mutation in KRT6A need further studies to confirm the rare feature of fissured tongue.

Psoriasis and oral lesions: multicentric study of Oral Mucosa Diseases Italian Group (GIPMO).

OBJECTIVE: This is a multicentric, observational and controlled study designed to verify the existence of a significant association between plaque-type psoriasis and oral lesions, such as geographic tongue and/or fissured tongue. STUDY DESIGN: during a period of 9 months all consecutive patients with plaque-type psoriasis were enrolled using simple nonrandom (sequential) sampling. The control group included healthy subjects presenting to the same Dermatology centers to monitor pigmented skin lesions; the patients were matched for age and sex. All patients were examined for oral lesions. RESULTS: Out of a total of 535 psoriatic patients and 436 control group patients, oral mucosal lesions were detected in 188 (35.1%) and 86 (19.7%) cases, respectively, and the difference is statistically significant. Fissured tongue (FT) and geographic tongue (GT), which were most frequently detected, were seen more frequently in psoriatic patients (FT: 22.6%; GT: 9.1%) than the control group (FT: 10.3%; GT: 5.2%) (p<0.05). CONCLUSIONS: On the basis of the similar studies reported in the literature and the large number of patients involved in our study, we can conclude that FT and GT can be clearly suggested as oral manifestations of plaque-type psoriasis, although the reason for this association is not clear.

Gingival fibromatosis with hemi-osseous hyperplasia of jaws, focal maxillary viral papillomatosis of gingiva, fissured tongue and congenitally missing anterior teeth: a case report and surgical management of a new syndrome.

Gingival fibromatosis is characterized by fibrotic enlargement of the gingiva that can occur as inherited or sporadic form. Inherited form can be an isolated trait or as a component of a syndrome. This article reports a 35 year old male patient affected by gingival fibromatosis associated with hemiosseous hyperplasia of mandible, maxilla, and zygoma on the right side, viral papillomatosis of maxillary anterior gingiva, fissured tongue and congenitally missing anterior teeth. The patient was subjected to phase I and phase II periodontal therapy. There was no evidence of recurrence of the enlargement after one year but the papillomatosis recurred. Gingival fibromatosis has been reported to be associated with various other abnormalities but not with those described in our case. This observation raises the possibility that the coexistence of these entities in our case may represent a new syndrome.

Bifid tongue, corneal clouding, and Dandy-Walker malformation in a male infant with otopalatodigital syndrome type 2.

We report on a male infant with otopalatodigital syndrome type 2 (OPD2) associated with a novel c.514C>G FLNA mutation and unusual clinical features including bifid tongue and congenital corneal clouding. Bifid tongue and congenital corneal clouding have each only been described once previously in a patient with OPD2, and this is the first description of Dandy-Walker malformation (DWM) in OPD2. The presence of these clinical findings in a mutation-confirmed case of OPD2 supports the notion that corneal clouding, bifid tongue, and DWM are part of the constellation of abnormalities caused by mutations in FLNA.

[Oral lesions in psoriasis]. / Manifestations buccales du psoriasis.

BACKGROUND: The literature contains little information about the incidence or prevalence of oral signs in psoriasis. The pustular forms are the most commonly incriminated, with geographic tongue being the most frequently cited oral sign. The aim of our study was to determine the various types of oral signs seen in a large population of psoriasis patients. PATIENTS AND METHODS: Four hundred psoriasis patients were recruited consecutively and a detailed oral examination performed by two clinicians. Similar examinations were performed over the same period in 1000 nonpsoriasis subjects comprising a control group in order to assess the incidence of the same oral signs in the general population. RESULTS: Comparing the two groups, fissured tongue was seen in 33.2% of psoriasis patients versus 9.9% of control subjects (P<0.0001). There was thus a highly significant (P<0.0001) correlation between geographic tongue and psoriasis (7.7% of psoriasis patients versus 1% of controls). In addition, a strong correlation was seen between the presence of pustular psoriasis and fissured tongue (83.3% of patients with pustular psoriasis versus only 30% of patients with other forms of psoriasis). DISCUSSION: Our study shows a strong correlation between psoriasis and fissured and geographic tongue, although these features are not pathognomonic for the disease. As regards geographic tongue, our results are consistent with the data in the literature. However, the prevalence of fissured tongue was considerably higher among our patients than in series published to date. These two types of sign involving the tongue, and which can occur in all forms of psoriasis, appear to be particularly strongly associated with pustular psoriasis. Patients are generally unaware of this sign and rarely complain of it.

The prevalence and risk factors associated with benign migratory glossitis lesions in 7619 Turkish dental outpatients.

OBJECTIVE: The aim of this study was to investigate the frequency of benign migratory glossitis (BMG) in a Turkish population with respect to gender, age, smoking, atopic and allergic diseases, dermatological diseases (psoriasis and seborrheic dermatitis), systemic diseases, and fissured tongue (FT). PATIENTS AND METHODS: The study was conducted on 7619 patients (3819 female, 3800 male) with various dental complaints, ranging in age from 4 to 60 years, recruited from the Department of Oral Diagnosis and Oral Radiology, Faculty of Dentistry, Atatürk University, Erzurum, Turkey. RESULTS: BMG was found in 1.5% (95% CI: 0.9-1.9) of all patients examined. Multivariate logistic regression indicated that age less than 30 (OR: 6.7 [95% CI: 2.9-15.5]), not smoking (6.3 [2.8-14.1]), and history of allergy or atopy (6.5 [3.3-12.5]) were significantly related to BMG. Of the patients with BMG, 34.5% (n = 40) had FT. No significant associations existed with gender, dermatological conditions (psoriasis and seborrheic dermatitis), or systemic diseases (diabetes mellitus, hematological disorders, cardiovascular disorders, hepatitis, gastrointestinal disorders, respiratory disorders, connective tissue disorders, and genitourinary disturbances). CONCLUSION: The present study demonstrated that BMG is more prevalent in young, nonsmokers, and atopic or allergic individuals.

Mucocutaneous findings in 100 children with Down syndrome.

Down syndrome is the most common chromosomal abnormality affecting numerous organs, including the skin. Our aim was to study mucocutaneous findings in this syndrome. To do so, 100 children with Down syndrome attending Schools for Children with Special Educational Needs and centers affiliated to the Welfare and Rehabilitation Organization in two provinces of Iran were thoroughly examined for skin and mucosal lesions. Their mean age was 11.2 years (3-20 years). Mucocutaneous findings were noted in 61. These were as follows (in decreasing order)--fissured tongue 28%, hypertrophy of tongue papilla 22%, premature graying 14%, cheilitis 13%, xerosis 12%, alopecia areata 11%, palmoplantar hyperkeratosis 10%, syringoma 6%, keratosis pilaris 4%, geographic tongue 4%, trichotillomania 4%, vitiligo 3%, seborrheic dermatitis 3%, livedo reticularis 2%. No instances of atopic dermatitis or skin infections and infestations were noted. The lack of atopic dermatitis in our series was in concordance with its low prevalence reported in recent articles. Improved hygiene due to patient care at home may underlie the absence of infections. With the increasing survival rate of those with Down syndrome, physicians should be more aware of the skin findings seen so frequently in this genetic disorder.

Prevalence and risk factors associated with geographic tongue among US adults.

OBJECTIVE: To characterize the prevalence of geographic tongue (GT) among US adults. DESIGN: Population-based case-control study. MAIN OUTCOME MEASURES: The presence or absence of GT. SUBJECTS AND METHODS: Data from 16 833 adults examined during The Third National Health and Nutrition Examination Survey, 1988-1994 (NHANES III), a study based on multistage probability sampling were analyzed using SAS-callable SUDAAN 9.0.1. RESULTS: Geographic tongue point prevalence was 1.8% (95% CI: 1.4, 2.3). Multivariate logistic regression showed significant effects of race-ethnicity, with Whites (AOR = 1.8; 1.3, 2.5) and Blacks (AOR = 1.6; 1.2, 2.1) having greater odds of GT than Mexican-Americans; current corticosteroid therapy (AOR = 3.7; 1.54, 8.6). Cigarette smokers had lower GT prevalence (AOR = 0.4; 0.3, 0.6). Fissured tongue (FT) was strongly associated with GT among non-smokers: AOR = 17.5 (7.8, 39.5). We did not find significant associations with age, gender, oral contraceptive use, diabetes mellitus, allergy or atopy, psychological or dermatological conditions as previous research has suggested. CONCLUSIONS: Geographic tongue was more prevalent among Whites and Blacks compared with Mexican-Americans, positively associated with FT, and inversely associated with cigarette smoking.

Geographic tongue: clinical characteristics of 188 cases.

The purpose of this study was to investigate the clinical characteristics and assess other factors associated with geographic tongue in Thailand. One hundred and eighty-eight Thais with geographic tongue and 188 controls were interviewed regarding their medical history, symptoms, and the nature and migratory pattern of their lesions. Variations in the clinical appearance, lesion location, and any associated tongue fissures were recorded. The age range for the 188 subjects was 9 to 79 years. The highest incidence (39.4%) occurred in the 20-29 age group. Women were affected more than men (1.5:1). The leading group of medical conditions consisted of allergy-related disorders; however, the incidence of these problems among both subjects and controls was not significantly different (55.2% vs. 44.8%). Our results demonstrated a significant co-existence of geographic tongue and fissured tongue. Most of the geographic tongue lesions manifested a typical appearance consisting of a central atrophic area bounded by a raised white circinate line (69.1%) with multiple tongue sites affected (62.8%). The most common locations were at the lateral margins and tip of the tongue. The majority of our subjects (75.5%) were asymptomatic. The results of this study correspond with the findings of previous geographic tongue studies in other populations.

A case of Melkersson-Rosenthal syndrome associated with Ehlers-Danlos syndrome.

Melkersson-Rosenthal syndrome (MRS) is characterized by the triad of recurrent facial palsy, lingua plicata, and facial edema. Herein, we report a case of MRS associated with Ehlers-Danlos syndrome due to rare presentation. To the best of our knowledge only one case of MRS associated with Ehlers-Danlos syndrome has been reported in the literature until now.