1/13. dyskeratosis congenita vs. chronic graft versus host disease: report of a case and a review of the literature.dyskeratosis congenita (DC) is a rare inherited bone marrow failure syndrome characterized by the triad of reticular pigmentation of the skin, nail dystrophy, and mucosal leukoplakia. DC is often associated with severe pancytopenia, and bone marrow failure is the principal cause of early mortality. Malignant transformation of mucosal leukoplakias may also occur. Rarely, aplastic anemia precedes the other clinical manifestations of the disease. We present a case of a 13-year-old boy who was diagnosed at age four with idopathic aplastic anemia, was treated successfully with an allogeneic bone marrow transplant, then subsequently developed skin, nail, and tongue lesions. While the initial impression was chronic graft-versus-host disease, additional work-up confirmed the diagnosis of DC.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
2/13. leukoplakia of the cornea presenting as fingerprint epithelial lines: a case report.Opacities in association with fingerprint patterns of the epithelium were seen in the upper half of the right cornea of a 52-year-old patient complaining of decreased vision. Removal of the corneal epithelium from the involved area resulted in visual improvement and normalization of corneal appearance. Histological examination of the removed epithelium failed to reveal the typical intraepithelial formation of basement membrane invaginations even in the areas corresponding to the fingerprint patterns. Moderate to severe degree of atypia were seen in the epithelial cells. No abnormalities were observed in 3 conjunctival specimens excised at the time of epithelial removal. The diagnosis of corneal dysplasia (leukoplakia) was made. Our report is the first description of epithelial fingerprint patterns of the cornea associated with leukoplakia.- - - - - - - - - - ranking = 0.034747725018899keywords = membrane (Clic here for more details about this article) |
3/13. Diseases of the oral mucosa.The dermatologist is often called upon to evaluate diseases of the oral mucosa. He should be prepared to give expert advice based on sound knowledge of oral diseases. This may be aided by biopsy study if the principles and pitfalls of obtaining and interpreting a biopsy specimen are followed. These are discussed and examples are given to illustrate some of the more common and important problems that may be encountered.- - - - - - - - - - ranking = 2.5keywords = mucosa (Clic here for more details about this article) |
4/13. thrombocytopenia: first symptom in a patient with dyskeratosis congenita.A case of dyskeratosis congenita is reported. This rare hereditary disease usually has the following progression: ectodermal dystrophy (reticular skin pigmentation, nail dystrophy, leukokeratosis of mucosal membranes), appearing in the first decade, followed in about 50% of these patients by a hematopoietic disorder resembling Fanconi's anemia, usually developing in the second or third decade. Carcinomas may occur in leukokeratotic areas in the third, fourth, or fifth decade. This patient's clinical course is interesting because the thrombocytopenia developed as an isolated symptom at the age of 5 years and preceded the skin anomalies by three years. The diagnosis of dyskeratosis congenita was made only after an evolution of five years. The diagnosis of dyskeratosis congenita--although it is a rare disease--should be considered in every child first seen with aplastic anemia or thrombocytopenia.- - - - - - - - - - ranking = 0.5347477250189keywords = mucosa, membrane (Clic here for more details about this article) |
5/13. Caustic cicatrization of the pharynx associated with dysphagia and premalignant mucosal changes.Although esophageal strictures with attendant malignant degeneration are well known sequelae of caustic ingestion, fixed pharyngeal structural lesions and development of oral leukoplakia are not well appreciated consequences of such ingestion. We present a patient 12 yr after lye ingestion who displayed rapidly progressive dysphagia suggestive of esophageal carcinoma. Instead, adhesions bisecting the cricopharyngeal inlet and an upper esophageal stricture were found endoscopically. In addition, an area of hypopharyngeal leukoplakia with marked acanthosis, parakeratosis, and dysplasia was noted and resected. The management of this patient's dysphagia and subsequent follow-up for his potentially precancerous oral lesion form the basis of this report.- - - - - - - - - - ranking = 2keywords = mucosa (Clic here for more details about this article) |
6/13. Acute urinary tract obstruction in dyskeratosis congenita.dyskeratosis congenita is a multisystem disease affecting internal organs as well as the skin, nails, and mucous membranes. Tumors of the skin, tongue, cervix, and esophagus may develop in the disease. In this report we describe acute urinary retention that developed due to congenital meatal atresia and small foci of white plaques constricting the urethral meatus in a 27-year-old man with dyskeratosis congenita.- - - - - - - - - - ranking = 7.4720645652363keywords = mucous membrane, membrane (Clic here for more details about this article) |
7/13. Effective stimulation of neutropoiesis with rh G-CSF in dyskeratosis congenita: a case report.dyskeratosis congenita (DC) is a rare congenital X-linked disorder. The major clinical manifestations are abnormal skin pigmentation, nail dystrophy, and leukoplakia of mucosal membranes. About 50% of the patients develop bone marrow failure, which is partly responsible for the poor prognosis. Recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) has been administered to some neutropenic patients with DC, but only a moderate stimulation of neutropoiesis has been observed. We report on a patient with DC treated with recombinant human granulocyte-colony-stimulating factor (rhG-CSF). This treatment resulted in a substantial dose-dependent increase in the neutrophil count.- - - - - - - - - - ranking = 0.5347477250189keywords = mucosa, membrane (Clic here for more details about this article) |
8/13. Oral florid papillomatosis and leukoplakia of the esophagus associated with keratoderma and showing transepidermal elimination.A 37-year-old male presented with a cauliflower-like tumor in the right buccal mucosa, leukoplakia of the tongue and esophagus, and keratotic papules or plaques on the extremities including bilateral palms and soles, associated with borderline diabetes mellitus and a repeated history of infections. A multi-system keratinizing abnormality was suspected, although no distinct disease category has been found showing similar involvements.- - - - - - - - - - ranking = 0.5keywords = mucosa (Clic here for more details about this article) |
9/13. candida laryngitis appearing as leukoplakia and GERD.The symptoms and physical findings of reflux laryngopharyngitis are characteristic, but the mucosal alterations evident in the larynx are not exclusive to reflux. When conventional reflux management produces an equivocal response, other etiologic factors such as mycotic laryngitis, need consideration. The differential diagnosis of dysphonia in an immunocompromised patient always includes mycotic infections, but mycotic laryngitis can also occur in the immunocompetent host. Laryngeal candidiasis can produce physical findings, such as erythema and leukoplakia, that are similar to reflux laryngopharyngitis. Predisposing factors include previous radiation therapy, antibiotic therapy, corticosteroids, and any alteration in the mucosal barrier. diagnosis is established by mucosal biopsy with special staining of the tissue to identify the characteristic hyphae. Treatment of mycoses can be difficult and long-term therapy is often required.- - - - - - - - - - ranking = 1.5keywords = mucosa (Clic here for more details about this article) |
10/13. Novel human papillomavirus (HPV) dna sequences from recurrent cutaneous and mucosal lesions of a stoma-carrier.Recent studies have demonstrated a high prevalence of human papillomavirus (HPV) types originally believed to be restricted to patients with epidermodysplasia verruciformis (EV) in benign and malignant skin tumors of the general population. Other groups detected typical mucosal HPV in skin tumors. We have investigated recurrent leukoplakial cutaneous and mucosal lesions located around the ileostoma of a woman with ulcerative colitis for the presence of HPV. Cutaneous, mucocutaneous, and mucosal ileostoma-biopsies were analyzed by three different polymerase chain reaction protocols for genital, cutaneous, and cutaneous EV-associated HPV types. polymerase chain reaction products were cloned, sequenced, and submitted to phylogenetic analyses. HPV-dna sequences of the EV-HPV group could be detected in all biopsies, whereas genital/mucosal or cutaneous HPV types were not found. HPV types detected comprised HPV20, HPV23, HPV38, and four putatively novel HPV types that belong to different clusters of the EV-HPV group B1. Different HPV types prevailed in cutaneous, mucocutaneous, and mucosal lesions and the number of HPV sequences found per lesion varied between one and three. Our data show the association of recurrent lesions around a stoma and at the ileum with known and novel EV-HPV types. These results emphasize the plurality of HPV and yield data for the possible transmission of cutaneous HPV to mucosal areas of the intestine.- - - - - - - - - - ranking = 5keywords = mucosa (Clic here for more details about this article) |
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