Orofacial granulomatosis associated with gingival enlargement: a case report.

Orofacial granulomatosis (OFG) is an uncommon condition with varying clinical presentation. Gingival enlargement in children could be due to a varied etiology. The present case report is of an adolescent female with initial presentation of generalized gingival enlargement, lip swelling and perioral discoloration without any known etiopathological factors or systemic involvement. Conservative excision of the enlargement was performed and histopathological examination revealed a non caseating granulomatous lesion. Diagnosis of orofacial granulomatosis in context to sarcoidosis was arrived after excluding other granulomatous diseases. Follow up after 18 months showed no recurrence and regression of lip swelling and perioral discoloration. Gingival enlargement can be considered as one of the presenting features of sarcoidosis.

Urbach-Wiethe disease in a young woman: A case report.

Urbach-Wiethe disease (lipoid proteinosis) is an autosomal recessive disorder that is characterized by a general thickening of the skin and mucous membranes. We report the case of a 22-year-old woman with lipoid proteinosis who presented with hoarseness, poor dentition, and skin lesions, and we discuss the management of this rare disease.

[Neonatal mucolipidosis type II]. / Révélation néonatale d'une mucolipidose de type II.

Mucolipidosis type II (ML II, OMIM 252,500) is an autosomal recessive disorder clinically characterized by facial dysmorphia similar to Hurler syndrome and pronounced gingival hypertrophy. The disorder is caused by a defect in targeting acid hydrolases on the surface of lysosomes, which impede their entry and lead to accumulation of undigested substrates in lysosomes. The onset of the symptoms is usually in infancy, beginning in the 6th month of life. Early onset, at birth or even in utero, is a sign of severity and involves the specific dysmorphia as well as skeletal dysplasia related to hyperparathyroidism. We report on a severe neonatal form of this disorder revealed by respiratory distress with severe chest deformity. The dysmorphic syndrome, combining coarse features, pronounced gingival hypertrophy, with diffuse bone demineralization and secondary hyperparathyroidism associating significant elevation of parathyroid hormone and alkaline phosphatase with normal levels of vitamin D and calcium were characteristics of mucolipidosis type II. Recognizing this specific association of anomalies helps eliminate the differential diagnosis and establish appropriate diagnosis and care.

Diagnosis of acute myeloid leukemia in a dental hospital; report of a case with severe gingival hypertrophy.

Acute myeloid leukemias (AMLs) are aggressive hematopoietic neoplasms that, if untreated, can lead to death within days. Owing to its high morbidity rate, early diagnosis and appropriate medical therapy is essential. Oral lesions may be the presenting feature of acute leukemias and are, therefore, important diagnostic indicators of the disease. Erythematous or cyanotic gingival hyperplasia with or without necrosis is reported to be the most consistent symptom leading to a diagnosis of acute leukemia that directs the patient to seek early dental consultation. This report refers to a patient with AML that was provisionally diagnosed in the dental hospital due to severe gingival enlargements.

Anti-inflammatory protein tumor necrosis factor-α-stimulated protein 6 (TSG-6) promotes early gingival wound healing: an in vivo study.

BACKGROUND: Human multipotent mesenchymal stromal cells (hMSCs) produce tumor necrosis factor (TNF)-α-stimulated protein 6 (TSG-6). TSG-6 modulates proinflammatory cytokine cascades and enhances tissue repair. This study tests the effects of recombinant human TSG-6 (rhTSG-6) on gingival wound healing within the first 2 days post-surgery. METHODS: After gingival resection in 120 Sprague-Dawley rats, 2 µg rhTSG-6 in 5-µL phosphate-buffered saline (PBS) or the same volume of only PBS solution was injected into gingival tissue approximating the surgical wound. Control animals did not receive injections. Tissue biopsies and blood were collected at 1 to 2, 6 to 8, 24, and 48 hours post-surgery (n = 10 per group). Specimens were analyzed via histologic analysis and enzyme-linked immunosorbent assay (ELISA) for quantification and comparison of inflammatory markers interleukin (IL)-1ß, IL-6, TNF-α, and myeloperoxidase (MPO). Wound photographs were taken for a double-masked clinical assessment at each time period. Weights were recorded for all animals pre- and post-surgery. RESULTS: Animals injected with rhTSG-6 had significantly less severe clinical inflammation at 6 to 8 (P = 0.01228), 24 (P = 0.01675), and 48 (P = 0.0186) hours. Sham and control animals had more weight loss at 24 and 48 hours. Sham and control animals had more pronounced cellular infiltrate. rhTSG-6-treated animals had significantly less MPO (P = 0.027) at 24 hours and IL-1ß (P = 0.027) at 24 and 48 hours. IL-6 showed a marginal significant difference at 6 to 8 hours, but there was no significant difference for TNF-α. CONCLUSION: rhTSG-6 reduced postoperative gingival inflammation by reducing levels of proinflammatory cytokines and cellular infiltrate and may offer significant promise as an anti-inflammatory agent for gingival surgery.

Juvenile hyaline fibromatosis: a case report.

Juvenile hyaline fibromatosis is a rare, hereditary disease with distinct clinical and histopathological features. Clinically, it presents with gingival hypertrophy, pappulonodular skin lesions and joint contractures. Bone involvement is usually an uncommon finding. We report a case of a 2-year-old patient, daughter of consanguineous parents, who presented since the age of 2 months with impairment of mental development, multiple joint contractures, motion limitation and nodules on the scalp. The calvarian lesions were surgically removed, and histopathological examination concluded to juvenile hyaline fibromatosis.

[Surgical correction of excessive gingival enlargements. Case studies]. / Extrém méretu iatrogén eredetu inyhiperpláziák sebészi korrekciója. Esetsorozat.

INTRODUCTION: Gingival enlargement is a common form of periodontal tissue reaction to several irritating factors. The most common form is the drug related gingival hyperplasia--nevertheless the heredity gingival fibromatosis and hematological cases can also occur and might impose a challenge to periodontists. After a short literature summary three Case reports are presented. The first case is a drug related gingival overgrowth in a young kidney transplant women who took Cyclosporin-A. The excessive mass of fibrotic tissue was removed by a series of internal beveled incision and the oral and buccal gingival flaps were united with sutures. The healing was uneventful and during the follow up patient's compliance and oral hygiene was superb. The second case is a very severe antihypertensive drug related gingival overgrowth in a 62 years old man interfering with the closure of his lip and corrected with a combination of conventional gingivectomy and internal reverse beveled incision both and Ca-channel blockers. The third case is a 42 years old woman with chronic idiopathic hemolytic anemia who presented a sudden onset acute excessive generalized gingival enlargement accompanied with severe pain and fever. At admission she was suspect for leukemia. After obtaining biopsy samples and having negative histology the soft tissue mass was removed under general anesthesia with conventional gingivectomy technique, but after a couple of days the severe pain and gingival swelling recurred. With administering systemic corticosteroid therapy (32 mg Medrol), the gingiva healed in five days and the one year follow-up showed a stable hematological and periodontal status. Today the more conservative internal beveled incision is preferred over the conventional gingivectomy in the most cases because it provides a more predictable healing and better esthetics. The recurrence of the drug related gingival hyperplasia can be anticipated by meticulous postoperative individual oral hygiene and regular supportive therapy. CONCLUSION: The combined conservative and surgical therapy leads to predictable postoperative result even in very severe systematically motivated gingival enlargements, nevertheless the successful patients management needs good cooperation with medical doctors and with the patients themselves.

[Cowden syndrome]. / Le syndrome de Cowden.

INTRODUCTION: Cowden syndrome is rare; oral symptoms are not always diagnosed. This case of Cowden syndrome was revealed by gingival hypertrophy. OBSERVATION: A 21-year-old female patient consulted for gingival hypertrophy and multiple papules in the mouth. She had a history of thyroid lobectomy due to a goiter. The gingival papillomatosis and the goiter suggested a Cowden syndrome. The diagnosis was confirmed clinically by facial skin papules. DISCUSSION: Cutaneous and oral lesions are usually the first symptoms of the syndrome. Diffuse gingival papillomatosis may suggest a Cowden syndrome and should lead to screen for associated symptoms. A high-risk diagnosis of breast and thyroid cancer is associated to Cowden syndrome and the patient should have a yearly follow-up.

Acute leukemias: a dentist's perspective.

Oral cavity reflects the health status of an individual. Many systemic diseases have signs and symptoms that manifest in the oral cavity, which in most of the cases precede the systemic manifestations. These array of diseases also include acute leukemias which present as gingival hyperplasia as the most consistent symptom seeking dental consultation that can be easily confused with many other benign conditions that present as gingival enlargements. One such rare case of adult acute lymphoblastic leukemia is presented in this article.

Case report: Infantile systemic hyalinosis: a dental perspective.

BACKGROUND: Infantile systemic hyalinosis is a rare genetic disorder which involves accumulation of hyaline in the skin, bones, mucous membranes, and occasionally, also in internal organs. The major manifestations include painful articular contractures, cutaneous lesions (hyperpigmentation, subcutaneous nodules), malnutrition resulting from diarrhoea, gingival, labial and buccal hypertrophy. CASE REPORT: The phenotype characteristics of infantile systemic hyalinosis (ISH) in a two year old boy were present. The characteristics of flattered occiput, limited limb movements and articular abnormalities of elbows and knees. Dental findings showed excessive gingival hypertrophy completely covering maxillary and mandibular teeth treatment. The gingival hypertrophy was surgically treated by gingivectomy under general anaesthesia. FOLLOWUP: The patient showed a full constellation of clinical manifestations of the disease. Despite the surgical intervention no improvement in oral hygiene was observed. CONCLUSIONS: Surgical treatment of the gingival hypertrophy was the treatment of choice.

Matrix metalloproteinase 9 levels in gingival crevicular fluid in patients after periodontal microsurgery for orthodontic induced gingival hypertrophy.

INTRODUCTION: In this study, we aim to compare the levels of matrix metalloproteinase 9 (MMP9) in the gingival crevicular fluid (GCF), as indicators for healing, in two groups of patients - operated with a classic periodontal surgical technique and the same technique but using a dental microscope. MATERIALS: We included 14 patients with ages between 12 and 26 years, average 14±6.2 years. Eight patients were women and six men. All patients presented gingival hypertrophy because of the orthodontic treatment on the mandibular arch. We performed gingivectomy on one-half of the mandibular arch by classic periodontal surgery and on the other half of the mandibular arch by a microscope-assisted gingivectomy. METHODS: In the hypertrophied gingiva, the expression of MMP9 was identified using immunohistochemical-staining techniques. For immunological determination of MMP9 in GCF we performed Elisa tests. RESULTS: We found different levels in different moments of the healing process for the two hemiarcades. CONCLUSIONS: We consider that faster healing in case of microscope-assisted gingivectomy may be related to the expression of MMP-9 in the GCF.

Ligneous gingivitis associated with plasminogen deficiency: a challenge in diagnosis.

A 66-year-old female presented with gum bleeding and soreness. Her medical history was significant for delayed wound healing, which appeared to cause blindness in her right eye. A gingival incisional biopsy revealed replacement of fibrous connective tissue by an amorphous and eosinophilic material. Direct immunofluorescent staining for antibodies was negative. After the differential diagnosis of ligneous gingivitis (LG) was rendered, the activity and antigenic level of plasminogen was tested and found to be significantly decreased. Partial improvement was observed within 1 year following a regimen of scaling, gingival curettage, topical steroids, and improved oral hygiene. LG is a rare condition, occurring as a result of plasminogen deficiency and subsequent fibrin accumulation. It presents as pseudomembranous gingiva and might affect the eyes and other organs. Dentists should be familiar with this condition, since they might assist in a diagnosis of this disease, with significant morbidity often missed by medical professionals.

Estimation of salivary amylase and total proteins in leukemia patients and its correlation with clinical feature and radiographic finding.

BACKGROUND: Leukemia is a fatal disease. The oral manifestations of the leukemias occur early in the course of the disease and these oral features can at times act as a diagnostic indicator. Saliva has been used as a diagnostic aid in a number of systemic diseases. MATERIALS AND METHODS: In our study, samples of unstimulated saliva of 30 leukemia patients who were not on chemotherapy were collected and analyzed for salivary amylase and total protein. The oral manifestations and radiographic changes (OPG) were recorded. The correlation between the oral manifestations and the salivary components (salivary amylase and total protein) was assessed for prognostic significance. RESULTS: In the present study when the mean values of salivary amylase (1280±754 U/ml) and total protein (647.2±320.7 mg%) were compared with that in control subjects. There was a statistically significant difference for amylase levels (P<.05). On intraoral examination the study subjects showed pallor, gingivitis, gingival enlargement, petechiae, and ecchymosis. On the OPG, the radiographic features included generalized rarefaction of bone (20%), thinning of lamina dura (3.4%), generalized alveolar crest bone resorption (30%), thinning of walls of alveolar crypts (6.7%), besides others, e.g., periapical abscess (10%). CONCLUSIONS: The saliva of leukemic patients demonstrated obvious changes in composition. A rise in salivary amylase and total protein levels was evident, with the increase in amylase levels being statistically significant.

[Generalized gingival enlargement--early clinic manifestation in acute leukemia. Case report]. / Hiperplazia gingivala generalizata-- manifestare clinica precoce în leucemia acuta. Prezentare de caz.

Leukemia is a hematological disorder arises from a hematopoietic stem cell characterized by a disordered differentiation and proliferation of neoplastic cells. Rapidly forming generalized gingival hyperplasia is usually the first sign of this disease (especially in acute forms). This case report describes a 54-year-old female who presented rapid gingival enlargement in only three weeks time, heralding the presence of acute monocytic leukemia (AML-FAB M5). The patient was immediately referred for hematologic management, but died five weeks later. Dentists and generalists should always be on guard to observe any oral manifestations that may lead to the early diagnostic of systemic diseases.