1/12. Gingival fibromatosis combined with cherubism and psychomotor retardation: a rare syndrome.Gingival fibromatosis is frequently an isolated condition, but rarely associated with some uncommon syndromes. This paper describes an 11-year-old patient with pronounced gingival enlargement, cherubic facial appearance, and psychomotor retardation and discusses the major aspects of the case. The most striking finding orally was the presence of grossly hyperplastic gingiva, which completely covered all teeth except the occlusal surfaces of some teeth. The swelling in the lower part of the face and the appearance of sclera beneath the iris suggest cherubism. The diagnosis was confirmed by the detection of giant cell regenerative granuloma and perivascular eosinophilic particles and osteoclasts after biopsy of the mandible. In this case, surgery was the only effective way to treat the patient. A full-mouth gingivectomy procedure was performed under general anesthesia in 2 stages. The case was followed for 12 months and no recurrence was seen. An appropriate oral hygiene regimen was established.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
2/12. Congenital generalized fibromatosis. An African case with gingival hypertrophy and other unusual features.What is believed to be the first reported case of congenital generalized fibromatosis in an African infant is described. Features in our patient, which were not noted in previous reports of the disease, include gingival hypertrophy, ankylosis of joints, skeletal hyperostosis, and lymphatic dilation of the ileal villi. Corticosteroid therapy was tried in the patient, but did not produce any beneficial effect.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
3/12. syndrome of gingival hypertrophy, hirsutism, mental retardation and brachymetacarpia in two sisters: specific entity or variant of a described condition?Two sisters born to consanguineous Lebanese parents had mental retardation and epilepsy, brachymetacarpalia, hirsutism, bulbous soft nose, thick floppy ears with abnormal configuration and gingival hypertrophy. One girl presented additionally with tetralogy of fallot and the other with congenital hypothyroidism and bilateral ureteral stenosis. These manifestations resemble the syndrome of hypertrichosis-gingival fibromatosis-mental retardation and seizures of Anavi et al. [1989: Dev Med child Neurol 31:538-542] but our two girls additionally have brachymetacarpia. The inheritance seems to be autosomal recessive. These two sisters may represent a hitherto undescribed syndrome. We discuss the findings in our patients in relation to the literature.- - - - - - - - - - ranking = 0.2keywords = fibromatosis (Clic here for more details about this article) |
4/12. Juvenile hyaline fibromatosis complicated with oral squamous cell carcinoma: a case report.A 45-year-old woman was referred because of swelling of the palate, gingival hypertrophy, and multiple cutaneous tumors. She had many cutaneous tumors, which covered most of her body, and she also displayed contractures of the major joints. Maxillary and mandibular gingival hypertrophy, malposition of the teeth, and swelling of the hard palate were the oral findings. The histopathologic features of the cutaneous and gingival tumors were consistent with hyaline fibromatosis, and the swelling of the palate proved to be a squamous cell carcinoma. The carcinoma was treated with tegafur/uracil and seemed to respond to this therapy.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
5/12. A solitary calvarial lytic lesion with typical histopathological findings of juvenile hyaline fibromatosis.Juvenile hyaline fibromatosis (JHF) is a rare systemic disease characterized by papulonodular skin lesions, gingival hyperplasia, joint contractures, and osteolytic lesions on long bones and the skull. It has recently been reported that the disease is caused by mutations in the gene encoding capillary morphogenesis protein-2 (CMG-2). To date, fewer than 60 cases have been published in the literature. Partial disease expression is common, but no cases featuring a solitary calvarial lesion have been reported. The authors discuss this 4-year-old boy with a solitary calvarial osteolytic lesion whose histopathological examination exhibited findings characteristic of JHF. Mutational analysis, however, revealed that there were no mutations in the CMG-2 gene. Two years after surgery, he was free of any complaints as well as gingival hyperplasia, joint contractures, and new skull or skin lesions. This patient's condition may represent clinical or genetic heterogeneity associated with JHF. Whether solitary lesions mimicking JHF can arise from somatic mutation of the CMG-2 gene remains to be proven.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
6/12. Juvenile hyaline fibromatosis: a case report.Juvenile hyaline fibromatosis (JHF) is a rare, autosomal recessively inherited disorder characterized histologically by deposition of hyaline, collagen like substance aberrantly synthesized by the cells of the connective tissue and deposited within many organs, typically within the skin, gingiva, joints and bones. We report this rare case of Juvenile hyaline fibromatosis in a young boy who presented clinically with multiple papulonodular skin lesions, non tender soft tissue masses over the scalp, face, anterior chest wall, back, periarticular regions of the extremities with restricted mobility of joints and gingival hypertrophy. Calcifications were seen within the tumor shadows in the skull x-rays. Histopathological study revealed characteristic features consistent with Juvenile hyaline fibromatosis. We report this case in view of its rarity.- - - - - - - - - - ranking = 1.4keywords = fibromatosis (Clic here for more details about this article) |
7/12. Zimmerman-Laband syndrome and profound mental retardation.A young man was diagnosed as having Zimmerman-Laband syndrome (ZLS) on the basis of gingival fibromatosis and absence of nails on thumbs and halluces in addition to other anomalies. He also had profound mental retardation.- - - - - - - - - - ranking = 0.2keywords = fibromatosis (Clic here for more details about this article) |
8/12. Juvenile hyaline fibromatosis.A case of juvenile hyaline fibromatosis in a 7-year-old Asian boy is presented. This autosomal recessive inherited condition has not been described in the otolaryngology literature before. We demonstrate the benefits surgical intervention, for treatment of gingival hypertrophy, can bring to the patient and outline the other features of this rare condition. The literature on juvenile hyaline fibromatosis and infantile systemic hyalinosis is also reviewed.- - - - - - - - - - ranking = 1.2keywords = fibromatosis (Clic here for more details about this article) |
9/12. Juvenile hyaline fibromatosis. Case report with five years' follow-up.Juvenile hyaline fibromatosis (JHF) is a rare hereditary disorder named by Drescher et al. in 1969. As recently as 1985, only 30 cases had been reported worldwide. We report the case of a 9-year-old girl who was diagnosed with JHF at age 3 and has been closely followed since. She initially had slowly growing multiple soft tumors over her entire body as well as hypertrophic gingiva and mild bone deformities. She was originally misdiagnosed with infantile myofibromatosis at age 3. However, at age 6, because of the light and electron microscopic findings of the tumors, she was diagnosed as having JHF. Currently, at age 9, she has nodular lesions developing over her body as well as bone changes that are progressing with no evidence of regression.- - - - - - - - - - ranking = 1.2keywords = fibromatosis (Clic here for more details about this article) |
10/12. Juvenile systemic hyalinosis--a rare cause of gingival hypertrophy: a case report.A 5-year-old boy was referred because of gross gingival hypertrophy which caused severe feeding difficulties in addition to obvious aesthetic concern. The patient also suffered from frequent upper respiratory tract infections and diarrhoea. In addition, he had pigmentation on bony prominences of his hands, elbows, knees and ankles, cutaneous nodules behind his ear and granulomatous tissue adjacent to his nose. Excess gingival tissue was removed under general anaesthesia. Histological features suggested a diagnosis of juvenile hyaline fibromatosis, which is considered to represent the same underlying pathological condition as infantile systemic hyalinosis. It is suggested that systemic hyalinosis should be preceded by 'infantile' or 'juvenile' depending on the clinical presentation.- - - - - - - - - - ranking = 0.2keywords = fibromatosis (Clic here for more details about this article) |
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