1/238. linear iga bullous dermatosis in a patient with chronic renal failure: response to intravenous immunoglobulin therapy.linear iga bullous dermatosis is a blistering disease with a heterogeneous clinical manifestation, characterized by deposition of IgA along the basement membrane zone of perilesional skin on direct immunofluorescence. We describe a patient with chronic renal failure who experienced linear iga bullous dermatosis. Long-term administration of intravenous immunoglobulin therapy was associated with clinical remission lasting more than 12 months.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
2/238. linear iga bullous dermatosis associated with ulcerative colitis.A 41-year-old woman developed skin vesicles one week before examination. She had been suffering from ulcerative colitis since the age of 30. Histologic examination of a vesicle showed a subepidermal bulla accompanied by mononuclear leukocyte infiltration intermingled with eosinophils and neutrophils. eosinophilia was also noted. Direct immunofluorescent test revealed linear deposits of IgA in the basement membrane zone.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
3/238. Linear IgA disease associated with lymphocytic colitis.A 66-year-old woman presented with a bullous skin eruption and chronic diarrhoea. Lesional skin showed subepidermal blistering, and direct immunofluorescence of perilesional skin revealed linear deposits of IgA at the dermoepidermal junction, establishing a diagnosis of linear IgA disease (LAD). Chronic watery diarrhoea complicated by substantial loss of body weight preceded the skin eruption for several months. On endoscopy, the colon appeared macroscopically normal. On histology, the colon mucosa showed increased numbers of intraepithelial lymphocytes and infiltrates of mononuclear cells in the lamina propria, indicative of lymphocytic colitis. Treatment with methylprednisolone and dapsone led to complete clearing of the bullous skin eruption and marked improvement of the patient's diarrhoea. Gastrointestinal disorders such as lymphocytic colitis have rarely been reported in patients with LAD. Whether the simultaneous occurrence of these two diseases is coincidental or due to related pathogenetic mechanisms remains to be seen.- - - - - - - - - - ranking = 3.187772944025keywords = lamina, mucosa (Clic here for more details about this article) |
4/238. Drug-induced linear iga bullous dermatosis probably induced by furosemide.linear iga bullous dermatosis (LABD) is an autoimmune disease, characterized by linear deposition of IgA along the basement membrane zone. Drug-induced LABD is rare but increasing in frequency. A new case of drug-induced LABD associated with the administration of furosemide is described.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
5/238. Linear IgA dermatosis mimicking ocular cicatricial pemphigoid.PURPOSE: To describe an unusual case of cicatrizing conjunctivitis. methods AND RESULTS: A 59 year old male presented in July 1997 with a complaint of intermittent redness and discharge of the right eye. Medical history was significant for a buccal mucosal lesion biopsy taken two years earlier that revealed subepidermal fibrosis. Conjunctival biopsy, performed to rule out ocular cicatricial pemphigoid, yielded a diagnosis of linear IgA dermatosis, an unusual diagnosis not often considered by ophthalmologists. CONCLUSIONS: The diagnosis of cicatrizing conjunctivitis is often overlooked. Its importance as it pertains to the life threatening disease of ocular cicatricial pemphigoid is clear.- - - - - - - - - - ranking = 0.20022190967774keywords = mucosa (Clic here for more details about this article) |
6/238. Unique immunobullous disease in a child with a predominantly IgA response to three desmosomal proteins.We report the case of a 15-year-old girl who presented at 11 years of age with an interesting, acquired and, to our knowledge, unique blistering disease. It involved both skin and mucous membranes with extensive oral and periungual lesions, clinically resembling paraneoplastic pemphigus. skin biopsy showed an inflammatory cell infiltrate in the upper dermis with numerous leucocytoclastic nuclear fragments, neutrophilic papillary microabscesses and a small subepidermal bulla. Direct and indirect immunofluorescence studies showed marked intercellular staining with IgA and less prominent staining with IgG. Granular deposition of IgA and, to a lesser extent IgG and C3, was also seen along the basement membrane zone. immunoblotting and enzyme-linked immunosorbent assay studies showed both IgG and IgA antibodies to desmocollin, desmoglein 3 and desmoplakin. However, despite extensive investigation, no underlying neoplasm was found. Treatment with dapsone and sulphapyridine proved ineffective but methylprednisolone and azathioprine have reduced the blistering. We believe that this patient is unique for her combination of IgA and IgG antibodies to desmoplakin, desmocollin and desmoglein 3, although further studies may provide further clarification.- - - - - - - - - - ranking = 44.242326204438keywords = mucous membrane, membrane (Clic here for more details about this article) |
7/238. A subepidermal bullous eruption associated with IgG autoantibodies to a 200 kd dermal antigen: the first case report from the united states.We describe an 81-year-old white man in whom a subepidermal bullous eruption developed that clinically resembled bullous pemphigoid. The eruption promptly responded to oral tetracycline and niacinamide and topical clobetasol. Histologic examination of perilesional skin revealed neutrophilic infiltration with formation of papillary microabscesses and subepidermal cleavage. Direct immunofluorescence showed linear deposition of IgG and C3 along the basement membrane zone. By indirect immunofluorescence, circulating IgG autoantibodies bound exclusively to the dermal side of salt-split normal human skin. Immunoblot analysis demonstrated that the patient's autoantibodies reacted with a 200 kd dermal protein that was different from type VII collagen, the epidermolysis bullosa acquisita autoantigen. This patient represents the first confirmed case from the united states with a recently reported novel autoimmune subepidermal bullous disease associated with IgG autoantibodies to a 200 kd dermal antigen.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
8/238. Linear IgA disease.PURPOSE: A case of linear IgA disease is reported to alert ophthalmologists and physicians to this unusual cause of chronic cicatrizing conjunctivitis. methods: Clinical records of a patient suffering from linear IgA disease were reviewed. RESULTS: A 65-year-old woman with a complicated medical history experienced rapidly progressive chronic cicatrizing conjunctivitis leading to corneal perforation. Undiagnosed gingivitis and palatal ulceration had been present for 5 years prior to the onset of ocular symptoms and vitamin C deficiency had followed the consequent dietary restrictions. A diagnosis of linear IgA disease was made on conjunctival biopsy, which demonstrated linear deposits of IgA along the epithelial basement membrane. The perforation was managed successfully with a conjunctival pediculate flap. Control of the inflammation was achieved with systemic prednisolone and cyclophosphamide but at the expense of serious systemic side-effects. CONCLUSIONS: Linear IgA disease causes progressive conjunctival cicatrization in many affected individuals.Although dapsone generally controls the inflammation, heavier systemic immunosuppression was required in this case. Involvement of skin or other mucosal surfaces may become symptomatic before the conjunctivitis, and physicians must be educated to refer patients for ophthalmological review on diagnosis. Conversely, ophthalmologists encountering ocular linear IgA disease should be aware of the possibility of other mucosal involvement requiring physician intervention.- - - - - - - - - - ranking = 1.4004438193555keywords = membrane, mucosa (Clic here for more details about this article) |
9/238. case reports of linear iga bullous dermatosis of childhood.linear iga bullous dermatosis of childhood (LADC) is an uncommon acquired blistering skin disorder which affects young children. It is characterised by a linear band of IgA at the epidermal basement membrane on direct immunofluorescence. From 1984 to 1988 at the previous skin centre at Middle Road Hospital, and 1989 to 1997 at the present National skin Centre, a total of 4 cases were seen. All the patients were female and their ages ranged from 5 to 14 years (mean 8 years). There were 2 Chinese, 1 Caucasian and 1 Malay. All presented with an acute onset of blistering of 1 to 3 weeks' duration. Their skin biopsies showed subepidermal bullae with neutrophils, with/without eosinophils and direct immunofluorescence tests revealed linear deposits of IgA along the dermoepidermal junction. All were treated with prednisolone and dapsone with good results. One patient developed dapsone-induced haemolysis and treatment was changed to colchicine. We review the epidemiology, aetiology, current concepts and treatment modalities of this condition.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
10/238. Linear IgA disease with IgA antibodies directed against 200- and 280-kDa epidermal antigens.We report an 80-year-old man with the lamina lucida type of linear IgA disease, with IgA autoantibodies reactive with 200-kDa and 280-kDa epidermal proteins. The patient presented with widespread bullous lesions on his trunk and extremities without mucosal involvement. Histopathology of lesional skin showed a subepidermal blister with papillary microabscesses of neutrophils and a few eosinophils. Direct immunofluorescence microscopy of perilesional skin showed linear deposits of IgA and C3 at the basement membrane zone. The patient's serum contained IgA autoantibodies that bound exclusively to the epidermal side of 1 mol L-1 NaCl split skin as determined by indirect immunofluorescence microscopy. Circulating IgA autoantibodies to 200- and 280-kDa antigens were detected in the patient's serum by immunoblot analysis using extracts from normal human epidermis and human epidermal keratinocytes. These two antibodies, eluted from individual nitrocellulose membranes, reacted with the epidermal side of 1 mol L-1 NaCl split skin on indirect immunofluorescence microscopy, and bound to hemidesmosomes as determined by immunoperoxidase electron microscopy. This observation suggests the presence of hitherto uncharacterized 200- and 280-kDa hemidesmosomal proteins distinct from BPAG1, BPAG2 and beta4 integrin as target antigens in linear IgA disease.- - - - - - - - - - ranking = 5.187772944025keywords = lamina, membrane, mucosa (Clic here for more details about this article) |
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