1/13. dyskeratosis congenita associated with three malignancies.dyskeratosis congenita is a rare inheritable disorder characterized by abnormalities of the skin, nails and oral mucosa. Aplastic anaemia resulting from bone marrow hypoplasia is a frequent cause of death. Squamous cell carcinoma developing from leukoplakia and visceral malignancies are other complications of the disease. We report here a case of dyskeratosis congenita in a man who developed three neoplasias of different systems over a period of many years. Squamous cell carcinoma and gastric adenocarcinoma manifested 17 years after the man was diagnosed with Hodgkin's disease.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
2/13. dyskeratosis congenita: delay in diagnosis and successful treatment of pancytopenia by bone marrow transplantation.dyskeratosis congenita is an inherited disorder characterized by nail dystrophy, skin pigmentary changes, mucosal leukoplakia, pancytopenia and an increased incidence of malignancy. Because of a widely held view that the outcome of bone marrow transplantation in dyskeratosis congenita is poor, this treatment option is sometimes not considered when pancytopenia develops. We present a child currently doing well 3 years after bone marrow transplantation, and review the literature.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
3/13. Oral-dental findings in dyskeratosis congenita.A 13-yr-old girl with dyskeratosis congenita is presented. Besides oral leukoplakia and nail dystrophies, there was evidence of pancytopenia, growth retardation, alopecia, mental retardation and microcephaly. The oral findings included caries, gingival recession, short-blunted roots, gingival bleeding, tooth mobility and severe alveolar bone loss resembling juvenile periodontitis.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
4/13. dyskeratosis congenita preceded by severe aplastic anemia: report of one case.dyskeratosis congenita is a rare hereditary disease which usually manifests with skin hyperpigmentation, nail dystrophy, and leukoplakia of the mucous membrane (triad). This report describes a six-year-old boy with severe aplastic anemia who was later diagnosed to have dyskeratosis congenita. His unusual presentation was pancytopenia followed by leukoplakia of the tongue, hyperpigmentation of the skin and dystrophy of the nails. Treatment with horse anti-human lymphocyte immunoglobulin (ALG) for his aplastic anemia was not effective.- - - - - - - - - - ranking = 2keywords = leukoplakia (Clic here for more details about this article) |
5/13. dyskeratosis congenita: report of a case and review of the literature.A case of dyskeratosis congenita in a 10-year-old white boy is reported. The triad of oral leukoplakia, nail dystrophy, and skin pigmentation was present. Other associated anomalies included dysphagia and varied immunoglobulin levels, although there was no evidence of anemia, pancytopenia, and ocular involvement. Since it may be associated with future malignant change, dyskeratosis congenita should be considered in all cases in which an obscure white lesion exists in the mouth.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
6/13. dyskeratosis congenita in two male cousins.dyskeratosis congenita is reported in two male Singaporean Chinese cousins. The family pedigree is presented. lichen planus or lichenoid lesions, instead of leukoplakia, was the manifestation in the oral cavity. The mode of inheritance and the differential diagnosis of Fanconi's anaemia are discussed. Dental findings are emphasised as such information is lacking in previously reported cases. One of the subjects expired of aplastic anaemia, which together with oral malignancy, is one fatal complication of this disease.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
7/13. dyskeratosis congenita in a girl simulating chronic graft-vs-host disease.dyskeratosis congenita (DCG) is a rare genodermatosis characterized primarily by reticular hyperpigmentation of the skin, dystrophy of the nails, and leukoplakia. It is frequently associated with Fanconi-type pancytopenia. Although DCG has a male predisposition, it has been reported in several female patients. We encountered a case of DCG occurring in a girl whose clinical features simulated chronic graft-vs-host disease (GVHD). Because DCG and chronic GVHD share several clinical and histologic features, physicians should always examine a patient for possible DCG whenever a diagnosis of chronic GVHD is considered. In addition, the similar manifestations of the two disorders suggest a similar pathogenesis on a cellular level in the immunologic system.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
8/13. Avascular necrosis of bone in dyskeratosis congenita.A case of dyskeratosis congenita, a rare X-linked disorder, is presented. The patient had the classic triad of cutaneous findings: reticulated hyperpigmentation, nail dystrophy, and leukoplakia. In addition, a previously unreported finding developed, avascular necrosis of bone without systemic steroid therapy. Recognition of this disorder is important because pancytopenia and malignancy develop in up to 50 percent of patients.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
9/13. dyskeratosis congenita: two examples of this multisystem disorder.Two brothers with the X-linked disorder, dyskeratosis congenita, are described. They showed the dermatologic triad of reticular hyperpigmentation, dystrophic nails, and leukoplakia oris as well as the other major feature of this disorder, aplastic anemia. Less common features observed included prenatal and postnatal growth retardation, mental retardation, elevated immunoglobulin levels, and gastrointestinal hemorrhage from mucosal ulceration. Previously unreported findings were intracranial calcifications and nutmeg-like cirrhotic changes of the liver. These brothers demonstrated that skeletal changes and bony fragility may predate anemia or steroid therapy. Although a dna repair defect is postulated as a possible primary defect, cytogenetic studies revealed no evidence of increased chromosomal breakage.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
10/13. dyskeratosis congenita with pancytopenia and horseshoe kidney.dyskeratosis congenita is an X-linked ectodermal dysplasia characterized by hyperpigmentation, nail dystrophy, and leukoplakia. pancytopenia develops in 50% of patients. We have reported another case with serious pancytopenia and the previously unreported occurrence of horseshoe kidney, and have discussed the similarities and differences between dyskeratosis congenita and Fanconi's anemia.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
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