Oral Juvenile Xanthogranuloma: a case report of gingival hyperplasia and osteolysis in male adult patient.

BACKGROUND: Juvenile Xanthogranuloma (JXG) is a non-hereditary, self-limiting disease which is usually presented in infancy or early childhood and in males over females. CASE PRESENTATION: We report a rare case of oral Juvenile Xanthogranuloma with recurrent progressive gingival hyperplasia and concomitant presentation of osteolysis in a 21-year-old adult male with no significant medical history. Patient presented with generalized gingival hyperplasia, osteolysis of the maxilla and mandible, and a round, firm, nodular mass with clear circumference on the left shoulder. Results of gingival tissue biopsy, karyotype, bone marrow biopsy and immunohistochemistry were suggestive of a diagnosis of Juvenile Xanthogranuloma with no association to hematologic malignancy. Unfortunately, patient declined treatment and elected to be transferred back to local hospital for future evaluation. CONCLUSIONS: Juvenile Xanthogranuloma in adults can have atypical manifestations including generalized gingival hyperplasia and osteolysis of the maxilla and mandible. It should be differentiated between Langerhans cell histiocytosis, Papillon-Lefevre Syndrome, and Pyogenic Granulomas. Despite uncommon incidence, it should be included in differential diagnoses in cases of similar clinical presentations.

Generalised gingival enlargement as a sole manifestation of IgG4-related disease.

An 18-year-old woman presented with enlarged gingivae of 1-year duration with no history of drug consumption or systemic conditions that might explain the enlargement. Biopsy revealed hyperplastic gingival epithelium and a plasma cell-rich inflammatory infiltrate in the subepithelial connective tissue with the presence of scattered multinucleated giant cells. Immunostaining revealed the plasma cells to be positive for IgG4. Serum IgG4 levels were elevated at 3.6 g/L (reference range: 0.049-1.985). These findings led towards the diagnosis of an IgG4-related disease (RD). Other granulomatous conditions were also ruled out via series of investigations. The enlarged tissue was surgically excised. No corticosteroids were administered owing to the localised nature of the manifestations. After 3 months, the gingival size remained unchanged. This case has been reported with a relatively shorter follow-up period because of the rarity of the presentation. IgG4-RD should be considered while diagnosing a patient with gingival enlargement.

Hiperplasia gingival fibrosa asociada a incisivo central superior retenido / Fibrous gingival hiperplasia associated with upper central incisor retained / Hiperplasia gengival fibrosa associada a incisivo central superior retido

RESUMEN Se describió el caso clínico de un adolescente de 12 años de edad, atendido en el Departamento de Ortodoncia de la Clínica Estomatológica Provincial "Mártires del Moncada" de Santiago de Cuba, al cual se le diagnosticó retención del incisivo central superior derecho hace más de dos años y para la cual recibió tratamiento con aparatología fija previo entorche del diente y seguimiento clínico-radiográfico. Durante el proceder ortodóncico, es remitido al Departamento de Periodoncia por presentar en la zona de dicho diente un aumento de volumen de color rosa coral, de consistencia dura y aspecto fibroso, que imposibilitó la incorporación definitiva del diente en su arco y plano de oclusión. Se realizaron los exámenes complementarios correspondientes, se diagnosticó hiperplasia gingival fibrosa localizada y se realizó tratamiento quirúrgico (gingivectomía), lo que permitió llevar a feliz término el tratamiento ortodóncico sin ninguna implicación psicológica y social para el paciente. La poca frecuencia de aparición de este tipo de lesión asociada a la retención de incisivos centrales superiores motivó el presente informe de caso.

ABSTRACT It was described a clinical case of a 12-year-old adolescent, treated at the Orthodontic Department of the Provincial Stomatological Clinic "Mártires del Moncada" in Santiago de Cuba, who was diagnosed with retention of the upper right central incisor more than two years ago and for which he received treatment with fixed appliances prior to tooth straightening and clinical-radiographic follow-up. During the orthodontic procedure, he was referred to the Department of Periodontics because he had a coral pink increase in volume in the area of that tooth, with a hard consistency and fibrous appearance, which made it impossible to definitively incorporate the tooth into its arch and occlusion plane. The corresponding complementary examinations were carried out, localized fibrous gingival hyperplasia was diagnosed and surgical treatment (gingivectomy) was performed, which allowed the successful completion of orthodontic treatment without any psychological or social implications for the patient. The rare occurrence of this type of injury associated with retention of upper central incisors motivated this case report.

RESUMO O caso clínico de um adolescente de 12 anos de idade, atendido no Departamento de Ortodontia da Clínica Estomatológica Provincial "Mártires del Moncada" de Santiago de Cuba, diagnosticado com retenção do incisivo central superior direito, foi descrito mais de dois anos e para os quais ele recebeu tratamento com aparelhos fixos antes do bloqueio dentário e monitoramento clínico-radiográfico. Durante o procedimento ortodôntico, é encaminhado ao Departamento de Periodontia por apresentar na área do referido dente um aumento no volume rosa coral, de consistência dura e aparência fibrosa, o que impossibilitou a incorporação definitiva do dente em seu arco e plano de oclusão. Foram realizados os exames complementares correspondentes, diagnosticada hiperplasia gengival fibrosa localizada e realizado tratamento cirúrgico (gengivectomia), o que permitiu o tratamento ortodôntico sem envolvimento psicológico e social do paciente. A baixa frequência de ocorrência desse tipo de lesão associada à retenção dos incisivos centrais superiores motivou este relato de caso.

Gingival Neurofibroma With Teardrop-Shaped Defects of the Interdental Alveolar Bone: An Unusual Oral Manifestation of Neurofibromatosis Type 1.

Gingival enlargement, although frequently encountered in clinical settings, is rarely associated with systemic diseases or syndromes. Among the diverse pathological conditions of neurofibromatosis type 1 (NF-1), minor manifestations in the orofacial region are occasionally overlooked. Herein, the authors present an unusual case of gingival neurofibroma in a patient with NF-1 associated with characteristic osseous defects in the alveolus in the long-term course of 17 years from the first examination.A 5-year-old boy with NF-1 was referred for the evaluation of gingival enlargement in the posterior left maxilla. An incisal biopsy led to the diagnosis of neurofibroma. At 22 years of age, the patient was referred again with a complaint of bleeding and pain in the same region refractory to periodontal therapy. The gingiva and tuberosity were swollen, and the second molar was affected by the tumor. Radiography revealed a low level of the interdental septum beneath the tumor with a relatively intact periodontal cortical bone, exhibiting a teardrop-shaped bone defect. The lesion was completely resected with the periosteum.Gingival neurofibroma in NF-1 may be associated with osseous and dental abnormalities and can be mistaken for periodontitis. Raising awareness of this clinical entity can lead to proper management of the esthetic and functional problems in the oral and maxillofacial region.

Oral manifestations in stem cell transplantation for acute myeloid leukemia.

Acute myeloid leukemia is a highly aggressive malignancy with a high morbidity rate, for which an accurate and rapid diagnostic is essential. Acute myeloid leukemia manifestations frequently include oral abnormalities. Still, there is a limited number of studies reporting the incidence of oral manifestations in acute leukemia, the prevalence of periodontal status and periodontal parameters in these patients. Our aim was to emphasize the importance of early recognition by the dental practitioners of oral cavity manifestations as signs of acute myeloid leukemia, so that prompt referral to the hematologists is being done, and appropriate treatment is offered.

Distribution of biopsied non plaque-induced gingival lesions in a Chilean population according to the classification of periodontal diseases.

BACKGROUND: Many gingival lesions are not induced by plaque. The aim of this study was to analyze the frequency of biopsied non-plaque-induced gingival lesions (NPIGL) in a Chilean population. METHODS: One thousand twelve cases of biopsied gingival lesions with confirmed anatomopathologic diagnosis were included, from the records of the Oral Pathology Referral Institute (OPRI), Faculty of Dentistry, University of Chile, between years 1990 and 2009. RESULTS: The most frequent non plaque-induced gingival lesions categories from biopsied cases included hyperplastic lesions, malignancies and benign neoplasms. The most frequent diagnoses in each category were fibrous hyperplasia (35.47%), squamous cell carcinoma (3.85%) and giant cell fibroma (2.08%), respectively. From all lesions, only 8.3% fitted in the specified categories of the current classification of periodontal diseases. CONCLUSIONS: The most frequent biopsied NPIGL were hyperplastic lesions and neoplasms. These categories represent relevant lesions to be included in a future periodontal classification system to improve the care needs of the patients, as well as early diagnosis and treatment.

Genital ulcers as diagnostic clue for acute myeloid leukaemia.

Acute myeloid leukaemia is a myeloid neoplasm with an extremely varying clinical appearance. Skin lesions are common for specific subtypes of acute myeloid leukaemia but are often misinterpreted. Here, we present a case of acute myeloid leukaemia in a young woman exhibiting genital ulcerations and gingival erosions.

[Pregnancy gingivitis and tumor gravidarum]. / Ciazowe zapalenie dziasel ze szczególnym uwzglednieniem guzów ciazowych.

During pregnancy periodontal tissues may become more susceptible to internal and external factors promoting inflammation. Changes in hormone levels, alterations in the periodontal tissue structure and a predisposition to dilating blood vessels during pregnancy may lead to a painful inflammation as a response to a slightest amount of biofilm. Tumor gravidarum emerges in 5% of pregnant women during the first or second trimester - it may recede and fade completely right after the labour when hormone levels normalize. This paper explains the aetiology and potential risk factors of pregnancy gingivitis.

Clinical experience of conversion from cyclosporine to tacrolimus prolonged-release in stabilized kidney transplant patients.

BACKGROUND AND AIMS: The CONCERTO study results showing the beneficial effects of conversion from cyclosporine to tacrolimus prolonged-release (tacrolimus PR) in stabilised patients after kidney transplantation, were first published in 2011. This communication describes our first experience of conversion from cyclosporine to tacrolimus PR in stabilised kidney transplant patients. The aim was to determine whether it could be used in routine clinical practice in the Czech and Slovak Republics. METHODS: Evaluation was carried out at five transplantation centres in the Czech Republic and Slovakia. In all participating Centres, the drug conversion was conducted according to the ICH/GCP guidelines. A total of 104 patients stabilised after kidney transplantation were converted from maintenance therapy with cyclosporine to treatment with tacrolimus PR. The data were collected 26 weeks after the switch. The primary endpoint was change in kidney graft function measured from the estimated glomerular filtration rate (GFR). The effect of conversion on blood pressure, metabolic parameters and cosmetic changes was also recorded. Special attention was paid to the safety and tolerability of treatment with tacrolimus PR. RESULTS: GFR increased after six months by 10 % (P = 0.040). In addition a significant decrease in serum creatinine and triglycerides level was found together with major reduction in the incidence and severity of gingival hyperplasia and hirsutism. 3% of patients developed new onset of diabetes mellitus. Otherwise, the switch was very well-tolerated, without serious adverse events or acute rejections. CONCLUSION: Conversion from cyclosporine to tacrolimus PR was shown to be a safe therapeutic alternative with patient benefits.

Sturge Weber Syndrome: An Unusual Case with Multisystem Manifestations.

BACKGROUND: Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder. It is characterized by the presence of facial port wine stains, neurological abnormalities like seizures and mental retardation, ocular disorders, oral involvement and leptomeningeal angiomas. CASE REPORT: A 13-year-old boy presented with the chief complaint of swollen, bleeding gums and deposits on the teeth. Detailed medical and dental history, clinical examination and investigations confirmed the diagnosis of Sturge-Weber syndrome. The treatment comprised of a thorough plaque control regimen to reduce the gingival enlargement, and it included oral hygiene instructions, thorough scaling, root planing at regular intervals and plaque index scoring which motivated the patient at each visit. CONCLUSION: This case illustrates that early intervention in a patient with Sturge-Weber syndrome is quintessential because of its associated gingival vascular features and their complicating manifestations. Furthermore, the need for periodic oral examinations and maintenance of good oral hygiene to prevent any complications from the oral vascular lesions has been highlighted.

Unusual cause for gum hypertrophy and skin nodules in a child.

Juvenile hyaline fibromatosis (JHF) is a rare progressive autosomal recessive disease that is characterised by papulonodular skin lesions, soft tissue masses, joint contractures, gingival hypertrophy and osteolytic bone lesions. We present an 18-month-old boy with JHF. This case demonstrates that JHF should be considered in the differential diagnosis when multiple subcutaneous nodules are observed in the face, head and neck. Gum hypertrophy with palatal nodules is unusual in JHF.

Generalised fibrotic gingival enlargement in a psoriatic patient: an association or a coincidence?

Gingival fibromatosis is a rare, benign, slow progressive fibrous overgrowth of gingiva, with great genetic and clinical heterogeneity. It can be inherited as an isolated trait (hereditary/idiopathic gingival fibromatosis), and/or as a component of a syndrome. We report a case of a young girl suffering from psoriasis who also presented with an unusual generalised idiopathic gingival fibromatosis. Psoriasis, a chronic inflammatory skin disease, of multifactorial origin, is characterised by keratinocyte hyperproliferation, dedifferentiation, neoangiogenesis and inflammation. T cell-mediated immunity is considered to be the key element in the disease process. The existence of oral mucosal alterations in patients with psoriasis is a controversial topic, as histopathological correlations are not clearly evident, and oral and cutaneous lesions do not follow a parallel course. However, this article highlights a possible association of T-lymphocyte stimulation inducing fibroblasts to undergo epidermal hyperproliferation and increased collagen production in the gingiva, which in turn may be responsible for inducing gingival hyperplasia.

Periodontitis associated with plasminogen deficiency: a case report.

BACKGROUND: Plasminogen deficiency is a rare autosomal recessive disease, which is associated with aggressive periodontitis and gingival enlargement. Previously described treatments of plasminogen deficiency associated periodontitis have shown limited success. This is the first case report indicating a successful therapy approach consisting of a non-surgical supra- and subgingival debridement in combination with an adjunctive systemic antibiotic therapy and a strict supportive periodontal regimen over an observation period of 4 years. CASE PRESENTATION: The intraoral examination of a 17-year-old Turkish female with severe plasminogen deficiency revealed generalized increased pocket probing depths ranging from 6 to 9 mm, bleeding on probing over 30%, generalized tooth mobility, and gingival hyperplasia. Alveolar bone loss ranged from 30% to 50%. Clinical attachment loss corresponded to pocket probing depths. Aggregatibacter actinomycetemcomitans, Porphyromonas gingivalis, Treponema denticola, Prevotella intermedia, Prevotella nigrescens and Eikenella corrodens have been detected by realtime polymerase chain reaction. Periodontal treatment consisted of full mouth disinfection and adjunctive systemic administration of amoxicillin (500 mg tid) and metronidazole (400 mg tid). A strict supportive periodontal therapy regimen every three month in terms of supra- and subgingival debridement was rendered. The reported therapy has significantly improved periodontal health and arrested disease progression. Intraoral examination at the end of the observation period 3.5 years after non-surgical periodontal therapy showed generalized decreased pocket probing depths ranging from 1 to 6 mm, bleeding on probing lower 30%, and tooth mobility class I and II. Furthermore, microbiological analysis shows the absence of Porphyromonas gingivalis, Prevotella intermedia and Treponema denticola after therapy. CONCLUSION: Adjunctive antibiotic treatment may alter the oral microbiome and thus, the inflammatory response of periodontal disease associated to plasminogen deficiency and diminishes the risk of pseudomembrane formation and progressive attachment loss. This case report indicates that patients with plasminogen deficiency may benefit from non-surgical periodontal treatment in combination with an adjunctive antibiotic therapy and a strict supportive periodontal therapy regimen.