1/8. A case of pachyonychia congenita with oral leukoplakia and steatocystoma multiplex.Pachyonchia congenita (PC) is an uncommon autosomal dominant genodermatosis affecting the nails and other ectodermal tissues. The most striking features are symmetrically thickened dysmorphic nails and hyperkeratotic skin lesions. We report a case of pachyonychia congenita in a 30-year-old male patient who had thickening and gray-brown discoloration of all nails and many nodules on his back and neck. He also had hyperkeratotic skin lesions on both feet. His tongue had irregularly-shaped, whitish plaques. histology of these nodules revealed the characteristic features of steatocystoma multiplex. After treatment with oral retinoic acid, his hyperkeratotic skin lesions improved.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
2/8. pachyonychia congenita. Manifestations for the otolaryngologist.When discovered, leukoplakia of the oral cavity is commonly an acquired lesion. pachyonychia congenita is a member of a rare group of disorders in which congenital white lesions of the oral cavity are present. Additional findings of note to the otolaryngologist are described as well.- - - - - - - - - - ranking = 0.25keywords = leukoplakia (Clic here for more details about this article) |
3/8. 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 = 0.25keywords = leukoplakia (Clic here for more details about this article) |
4/8. pachyonychia congenita with laryngeal involvement.pachyonychia congenita (Jadassohn-Lewandowsky syndrome) is a rare autosomal dominant disorder characterized by nail dystrophy, hyperkeratosis of the palms and soles, leukoplakia of the mucosa of the upper respiratory tract and anus, follicular keratoses especially about the knees and elbows, and palmar and plantar hyperhidrosis. We present a patient with pachyonychia congenita and an exophytic lesion in the larynx at the posterior commissure. He is the youngest of 4 family members with this disorder covering 3 generations. Each of the 4 patients also exhibited both oral leukoplakia compatible with the Jadassohn-Lewandowsky syndrome (Ikonograph Dermatol. Lab., 1 (1906) p. 29), and subcutaneous cysts of the face and scalp as described by Jackson and Lawler (Ann. eugenics (1951) 142.- - - - - - - - - - ranking = 0.5keywords = leukoplakia (Clic here for more details about this article) |
5/8. 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 = 0.25keywords = leukoplakia (Clic here for more details about this article) |
6/8. 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 = 0.25keywords = leukoplakia (Clic here for more details about this article) |
7/8. Oral squamous cell carcinoma in a case of dyskeratosis congenita.oral manifestations of dyskeratosis congenita (DCG) have received little attention in dental literature. This report is a case of dyskeratosis congenita in a 17-year-old female which was associated with oral lesions such as leukoplakia, superimposed candidal infection, desquamative gingivitis, and severe periodontitis. Histopathologic examination of the granular lesion on the right lateral border of the dorsum of the tongue showed well-differentiated squamous cell carcinoma. Etiopathogenesis, clinical features, and laboratory findings of this disease are discussed.- - - - - - - - - - ranking = 0.25keywords = leukoplakia (Clic here for more details about this article) |
8/8. Treatment of the hematological manifestations of dyskeratosis congenita.dyskeratosis congenita is a congenital multisystem disorder, characterized by skin pigmentation, dystrophic nails, and leukoplakia. Hematologic abnormalities progressing to severe pancytopenia play a significant role in the poor prognosis of afflicted patients. We report on a patient with dyskeratosis congenita and severe aplastic anemia, complicated by life threatening infection. The patient was treated with recombinant granulocyte-macrophage colony-stimulating factor. This therapy resulted in a moderate and transient improvement in absolute neutrophil counts. Current concepts regarding the pathogenesis and etiology of dyskeratosis congenita are discussed, while reviewing the available therapeutic options.- - - - - - - - - - ranking = 0.25keywords = leukoplakia (Clic here for more details about this article) |