1/172. skin eruption with gabapentin in a patient with repeated AED-induced Stevens-Johnson's syndrome.skin eruptions have been reported with the use of all antiepileptic drugs and there is a significant risk of cross-reactivity between these agents in causing serious eruptions such as Stevens-Johnson's syndrome. Gabepentin is usually considered a safe agent for patients with a previous history of drug allergies and there have been no cases of skin eruption reported to the gabapentin post marketing surveillance. We report a patient who had severe Stevens-Johnson's syndrome induced by phenytoin and later by carbamazepine. Subsequent use of gabapentin also resulted in a skin eruption which was limited to the lower extremities but without systemic or mucosal involvement. This case suggests that patients with a strong history of drug-induced idiosyncratic reactions may experience such reactions to gabapentin as well.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
2/172. Cutaneous necrosis as a terminal paraneoplastic thromboembolic event in a patient with non-Hodgkin's lymphoma.Thrombotic complications in non-Hodgkin's lymphoma often originate in the large veins. We describe a patient with refractory advanced high-grade lymphoma who presented with the rare complication of extensive cutaneous necrosis due to thrombosis of dermal vessels; there was also a recent new peak of monoclonal IgM-kappa protein. Direct immunofluorescence demonstrated immune deposits with complement in the dermal vessel wall. Based on these observations and on published data, we suggest that these complexes were the trigger for the thrombotic events and that the monoclonal IgM acted as xenoreactive antibodies, initiating a cascade of events. The first step of this cascade was activation of the complement and the membrane attack complex, which caused secretion of IL-1 alpha by endothelial cells, followed by overexpression of tissue factor on the surface of the dermal vessel wall endothelium. Dermal vessel thrombosis was the final event in this cascade.- - - - - - - - - - ranking = 0.208840991438keywords = membrane (Clic here for more details about this article) |
3/172. skin fragility and hypohidrotic ectodermal dysplasia resulting from ablation of plakophilin 1.We report a 2-year-old boy with an unusual autosomal recessively inherited skin disease comprising trauma-induced skin fragility and congenital ectodermal dysplasia affecting hair, nails and sweat glands. skin biopsy showed widening of intercellular spaces between keratinocytes and ultrastructural findings of small, poorly formed desmosomes with reduced connections to the keratin filament cytoskeleton. Immunohistochemical analysis revealed a complete absence of staining for the accessory desmosomal plaque protein plakophilin 1 (PKP1; band 6 protein). The affected individual was a compound heterozygote for null mutations on both alleles of the PKP1 gene. Both mutations occurred within the amino terminus of PKP1, the domain which normally binds the cytoskeletal keratin filament network to the cell membrane. Apart from its localization within desmosomal plaques, PKP1 may also be present within the cytoplasm and nucleus and has putative roles in signal transduction and regulation of gene activity. The clinicopathological observations in this patient demonstrate the relevance of PKP1 to desmosome formation, cutaneous cell-cell adhesion and epidermal development and demonstrate the specific manifestations of human functional knockout mutations in this gene.- - - - - - - - - - ranking = 0.208840991438keywords = membrane (Clic here for more details about this article) |
4/172. Comel-netherton syndrome complicated by papillomatous skin lesions containing human papillomaviruses 51 and 52 and plane warts containing human papillomavirus 16.We describe a 28-year-old woman with characteristic clinical signs of Comel-netherton syndrome (CNS) who showed numerous plane warts on her face and forearms and papillomatous skin tumours affecting her groins and genitoanal skin. Using human papillomavirus (HPV) type-specific primers for cutaneous and mucosal HPV types we identified HPV 16-specific sequences in plane warts and HPV 51- and HPV 52-specific dna in papillomatous skin from the patient's groins, suggesting a pathogenetic role (cofactor) for HPV in the development of verrucous skin lesions in patients with CNS. Whether the susceptibility to HPV infections is due to decreased cellular immunity or epidermal defence mechanisms remains to be seen.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
5/172. Cutaneous ciliated cyst of the right lower leg.A 23-year-old Japanese woman with a cutaneous ciliated cyst on her right lower leg is reported. A subcutaneous cyst, measuring 2.5 cm in diameter with papillary projections into the lumen, was lined with ciliated cuboidal to columnar epithelia with partial stratification, histologically. These lining cells did not produce mucin. Immunohistochemically, the ciliated lining cells of the cyst were diffusely positive to epithelial membrane antigen and cytokeratin. In addition, positive immunoreaction with anti-desmin monoclonal antibody was observed in the body of the cilia. Less than 10% of the epithelial cells revealed positive immunoreaction to S-100 protein and estrogen receptor.- - - - - - - - - - ranking = 0.208840991438keywords = membrane (Clic here for more details about this article) |
6/172. association of lymphocytic colitis with linear IgA dermatosis.The case of a 66-year-old female patient is presented, who suffered from chronic watery diarrhea. In addition, she developed linear IgA dermatosis after oral treatment of a presumed yeast infection with nystatin. To evaluate the reason for her diarrhea, colonoscopy was performed. The macroscopic aspect of the colon mucosa was described as normal with no specific alterations for chronic inflammatory bowel disease or for bacterial infections. In contrast, the histologic examination revealed the typical characteristics of lymphocytic colitis. This disease is thought to be caused by immunological reactions against as yet unknown luminal antigens. After treatment with steroids and dapsone the diarrhea as well as the skin disease disappeared. To our knowledge, the present report describes for the first time the association of linear IgA dermatosis with lymphocytic colitis after oral treatment with nystatin. A possible causative link between these two disease entities is discussed.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
7/172. Treatment of cutaneous lesions of xanthoma disseminatum with a CO2 laser.We describe a case of a 15-year-old African American girl with widespread papulonodular lesions of xanthoma disseminatum especially in the periorbital area and on the flexural surfaces of the neck, axillae, arms, and legs. There were no mucosal lesions. An initial trial in the distant past of liquid nitrogen cryotherapy resulted in partial shrinking of cutaneous lesions but was too painful for the patient. She then underwent surgical excision of bilateral eyelid lesions with improvement, but additional procedures were deemed impractical when considering the great number and size of the lesions. Consequently we treated the patient with a carbon dioxide (CO2) laser with excellent results. The relatively great speed at which the CO2 laser procedure can be performed has made the removal of multiple lesions in each session possible. Additional advantages included precise vaporization of lesions, hemostasis during the operative procedure, and minimal postoperative pain and edema.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
8/172. A novel genodermatosis caused by mutations in plakophilin 1, a structural component of desmosomes.desmosomes are adhesive intercellular junctions that link adjacent cells and provide anchoring points for the keratin filament cytoskeleton. The mechanical integrity of desmosomes depends on a complex network of transmembranous and cytoplasmic proteins and glycoproteins each encoded by distinct genes. Recently, naturally occurring human mutations in one of these desmosomal structural components, plakophilin 1, have been described. The clinical features of the affected individuals, who have total ablation of plakophilin 1, comprise a combination of skin fragility and ectodermal dysplasia with loss of hair, reduced sweating and nail dystrophy. desmosomes in the skin are small and poorly formed and there is widening of intercellular spaces between keratinocytes as well as detachment of the keratin filament network from the cell membrane. These clinicopathological observations demonstrate the relevance of plakophilin 1 to keratinocyte adhesion and epidermal morphogenesis. This new form of genodermatosis represents the first example of human desmosome gene mutations and its clinical and ultrastructural characteristics are highlighted in this article.- - - - - - - - - - ranking = 0.208840991438keywords = membrane (Clic here for more details about this article) |
9/172. Cicatrizing conjunctivitis associated with paraneoplastic lichen planus.PURPOSE: To report two cases of cicatrizing conjunctivitis associated with paraneoplastic lichen planus. methods: case reports. RESULTS: Two patients were examined because of redness and discomfort in both eyes. A 63-year-old woman with follicular, small-cleaved cell lymphoma had cicatrizing conjunctivitis, stomatitis, vulvitis, and skin lesions. A 25-year-old man with malignant thymoma had cicatrizing conjunctivitis, erosive stomatitis, and penile papules. Histopathologic studies of conjunctiva and skin biopsy specimens in the first patient and labial biopsy specimens in the second revealed lichen planus. CONCLUSION: Paraneoplastic lichen planus is a possible cause of cicatrizing conjunctivitis associated with inflammatory skin and mucous membrane disease.- - - - - - - - - - ranking = 17.053974719446keywords = mucous membrane, membrane (Clic here for more details about this article) |
10/172. Tumoral calcinosis: a case report with an electron microscopic study.A 68-year-old woman developed large subcutaneous masses on her abdomen and thighs after a bruise sustained in a traffic accident. She had severe pain when sitting up straight. Histological examination revealed calcified tissues in the entire dermis of the injured areas. On electron microscopy, crystalline materials were observed in the dermis, which seemed to be formed by the deposition of hydroxyapatite on unusual proteoglycan. In a vessel wall, a thick, layered basement membrane was observed. This suggests that vascular injury and subsequent hypoxia play a role in the process of calcinosis. We performed a partial resection with good results in alleviating the patient's pain.- - - - - - - - - - ranking = 0.208840991438keywords = membrane (Clic here for more details about this article) |
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