1/142. Familial cerebellar hypoplasia and pancytopenia without chromosomal breakages.Two siblings manifested a neuro-haematologic syndrome characterised by low birth weight, failure to thrive, chronic persistent tongue ulceration, severe truncal ataxia and pancytopenia without either telangiectasia or chromosomal instability. One sibling died from sepsis and the cerebellum demonstrated reduced cellularity of the molecular and granular layers with relative preservation of purkinje cells and minimal gliosis. A surviving sibling has shown haematologic progression to a myelodysplastic disorder. There was no evidence of any chromosomal instability following exposure of fibroblasts and lymphocytes to irradiation. monosomy-7 was not present in the surviving sibling. We suspect that these two patients represent another example of the rare Hoyeraal-Hreidarsson syndrome and we are currently engaged in very close monitoring of the surviving sibling for evidence of any karyotypic abnormality.- - - - - - - - - - ranking = 1keywords = ulcer (Clic here for more details about this article) |
2/142. Immunoablative high-dose cyclophosphamide without stem cell rescue in paraneoplastic pemphigus: report of a case and review of this new therapy for severe autoimmune disease.Paraneoplastic pemphigus (PNP) is a refractory and life-threatening autoimmune mucocutaneous disease. We have recently reported the effectiveness and safety of ablative intravenous cyclophosphamide (200 mg/kg daily over 4 days) without stem cell rescue in patients with refractory autoimmune diseases including systemic lupus erythematosus, autoimmune cytopenias, chronic inflammatory demyelinating polyneuropathy, and aplastic anemia. We report chronic lymphocytic leukemia-associated PNP in a patient who presented with extensive and debilitating painful oral ulcerations and received ablative therapy. The patient tolerated the regimen well and showed a slow but sustained improvement despite persistence of the underlying neoplasm. Eighteen months after therapy, the oral ulcerations were almost completely healed and the circulating autoantibodies became negative. Currently, the patient remains on cyclosporine and a low dose of prednisone. This provides further evidence for the efficacy and safety of this regimen in the management of severe autoimmune diseases including PNP.- - - - - - - - - - ranking = 2keywords = ulcer (Clic here for more details about this article) |
3/142. nicorandil-induced severe oral ulceration: a newly recognized drug reaction.nicorandil, a potassium-channel activator used for the long-term treatment of ischemic heart disease, has been recently implicated as having a drug side effect of recurrent aphthous stomatitis. Three patients are reported with drug-induced aphthouslike ulceration secondary to nicorandil.- - - - - - - - - - ranking = 5keywords = ulcer (Clic here for more details about this article) |
4/142. Recurrent buccal space abscesses: a complication of Crohn's disease.Oral features of Crohn's disease include ulcerations, lip fissuring, cobblestone plaques, and mucosal tags. We report the case of a 16-year old male patient with a 3-month history of abdominal pain, diarrhea, and oral ulceration. Clinical examinations revealed established intestinal lesions, a marked cobblestone appearance in the oral cavity, and an unusual pattern of presentation not previously reported in the literature: persistent, recurrent buccal space abscesses.- - - - - - - - - - ranking = 2keywords = ulcer (Clic here for more details about this article) |
5/142. Severe oral ulcerations induced by alendronate.The authors report the case of a 54-year-old man suffering from steroid-induced osteoporosis, who presented with severe oral ulcerations due to sucking alendronate tablets instead of swallowing them. The misuse of alendronate in this case, underlies the risk of direct mucosal injury with this drug.- - - - - - - - - - ranking = 5keywords = ulcer (Clic here for more details about this article) |
6/142. Anti-neutrophil cytoplasmic antibody-associated mucocutaneous allergic vasculitis with oral manifestations caused by propylthiouracil.A 49-year-old woman treated with increasing dosage of propylthiouracil (PTU) in order to control hyperthyroidism, developed progressive necrotic ulcers in the oral cavity, oropharynx and rhinopharynx and vasculitic ulcers on both auricular pinnae after a few days. The PTU treatment was immediately discontinued and the mucosal and skin manifestations resolved promptly. Laboratory findings, including anti-neutrophil cytoplasmic antibodies (ANCA), suggested allergic vasculitis. This is to our knowledge the first reported case of oral manifestations of PTU-induced allergic vasculitis.- - - - - - - - - - ranking = 2keywords = ulcer (Clic here for more details about this article) |
7/142. African oral histoplasmosis mimicking lip carcinoma: case report.A case of localised African histoplasmosis with an unusual presentation in a 56 year old Nigerian farmer is reported. The lesion presented as an ulcer clinically mimicking squamous cell carcinoma of the lower lip. An incisional biopsy and culture studies confirmed African histoplasmosis and the ulcer healed spontaneously without treatment. This case is reported to highlight the unusual location and clinical course of African histoplasmosis.- - - - - - - - - - ranking = 2keywords = ulcer (Clic here for more details about this article) |
8/142. Metastasising malignant lymphoma mimicking necrotising and hyperplastic gingivostomatitis.This paper presents the case of a 65 year-old woman suffering from recurrent oral aphthoid ulcers which rapidly evolved towards hyperplastic and ulcerated lesions over the entire floor of the mouth. The initial lesions were interpreted as non-specific aphthoid ulcers. Later, a tentative diagnosis of necrotising stomatitis with secondary reactive proliferating epithelial hyperplasia was made. The clinical symptoms and the immuno-phenotyping of lymphocytes circulating in the peripheral blood suggested the diagnosis of CD30-positive large cell anaplastic lymphoma. The biopsy showed only a pseudoepitheliomatous hyperplasia, reactive infiltrates and no lymphoma cells. The disease ran a fulminant course leading to death within 4 weeks due to acute gastro-intestinal bleeding. autopsy revealed infiltrates of CD30 large cell anaplastic lymphoma in a submandibular lymph node, in a thrombus stenosing the right subclavian vein, in the spleen, the anterior and posterior gastric wall as well as in the depth of the tumour on the floor of the mouth. The clinical and histopathological spectrum of CD30 large cell anaplastic lymphoma is considerably variable. The particular feature of pseudoepitheliomatous hyperplasia has been reported especially in CD30 anaplastic large cell lymphomas. An early correct diagnosis is rendered difficult in insufficient biopsy size, becauses this type of lymphoma often simulates other inflammatory or neoplastic skin diseases. Thus, with a necrotising and hyperplastic gingivostomatitis, the diagnosis of a CD30 anaplastic large cell lymphoma should be considered.- - - - - - - - - - ranking = 3keywords = ulcer (Clic here for more details about this article) |
9/142. A bad eye and a sore lip.A 48-year-old woman developed painful visual loss in the left eye, meningismus, and painful oral ulcers. magnetic resonance imaging of the brain with gadolinium demonstrated enhancement of the left optic nerve. Lumbar puncture showed a lymphocytic pleocytosis, and a biopsy specimen of one of the oral ulcerations was consistent with Behcet's disease. epidemiologic factors and diagnostic criteria for Behcet's disease are discussed.- - - - - - - - - - ranking = 2keywords = ulcer (Clic here for more details about this article) |
10/142. Gingival telangiectases: an underappreciated physical sign of juvenile dermatomyositis.BACKGROUND: medline searches (1966-June 1969) failed to identify references that give detailed descriptions of the oral manifestations of dermatomyositis (DM). However, several reports predating medline provided more complete descriptions of oral lesions associated with DM. OBSERVATIONS: We describe 5 cases of juvenile DM with oral manifestations, primarily in the form of gingival telangiectases. These findings are compared with those descriptions found in earlier reports. CONCLUSIONS: Oral lesions in juvenile DM have rarely been reported. mucous membrane involvement associated with DM may include telangiectases, edema, erosions, ulcers, and leukoplakia-like areas. In cases of DM, gingival telangiectases likely represent an underappreciated diagnostic finding analogous to nail-fold telangiectases.- - - - - - - - - - ranking = 1keywords = ulcer (Clic here for more details about this article) |
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