1/7. Childhood epidermolysis bullosa acquisita: a novel variant with reactivity to all three structural domains of type VII collagen.Most patients with epidermolysis bullosa acquisita develop an autoimmune response to the non-collagenous (NC) 1 domain of type VII collagen. We report a 4-year-old girl of white European descent presenting with widespread blistering disease involving the face, hands, genital area and oral mucosa. Histopathology revealed subepidermal blisters, and linear deposits of IgG and C3 were seen along the dermal-epidermal junction on direct immunofluorescence (IF) microscopy of a perilesional skin biopsy. On indirect IF microscopy, circulating autoantibodies exclusively stained the dermal side of 1 mol L-1 NaCl-split skin. The patient's IgG autoantibodies labelled a 290-kDa protein on Western blotting of dermal extracts, and reacted with the NC1, NC2 and triple helical domains of type VII collagen on immunoblotting of recombinant and cell-derived fragments obtained by pepsin and collagenase digestion of the full-length protein. Oral methylprednisolone and dapsone led to clearance of lesions, which healed with mild scarring and milia formation. Treatment was discontinued after 1 year and the patient has now been in remission for more than 3 years.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
2/7. epidermolysis bullosa acquisita and multiple myeloma.The coexistence in the same patient of epidermolysis bullosa acquisita (a rare, autoimmune, acquired mucocutaneous blistering disorder) and multiple myeloma (a plasma cell neoplasm) is extremely uncommon. We describe a patient in whom both of these diseases occurred simultaneously. Intravenous immunoglobulins were used to treat both diseases.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
3/7. epidermolysis bullosa acquisita presenting with localized facial blisters.A 44-year-old woman presented with blisters on her face of 1-month's duration. A biopsy showed a subepidermal bulla with a dermal infiltrate of inflammatory cells and thick linear deposits of immunoreactants along the dermo-epidermal junction. Using Western immunoblots, the patient's IgG antibody was found to recognize type VII procollagen. Moderate doses of oral prednisolone resulted in a complete remission, without a recurrence for more than 3 years.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
4/7. A case of mixed bullous disease of epidermolysis bullosa acquisita and linear iga bullous dermatosis.A 75-year-old Japanese male visited us with bullous eruptions on the extremities. physical examination revealed large bullae on the hands, lower legs and feet. The oral mucosa was also involved. histology disclosed subepidermal blister with inflammatory cell infiltrates in the dermis. Direct immunofluorescence showed deposits of IgG and IgA at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient's IgG antibodies reacted with the dermal side of the split, while IgA antibodies reacted with the epidermal side. immunoblotting showed that the patient's serum reacted with the NC1 domain of type VII collagen (290-kDa epidermolysis bullosa acquisita antigen) as well as the 120-kDa linear iga bullous dermatosis antigen, LAD-1. Systemic prednisolone resulted in a favorable response. From the clinicopathological findings, the present case is not consistent with either epidermolysis bullosa acquisita or IgA bullous dermatosis. Therefore, we regarded the case as mixed bullous disease of epidermolysis bullosa acquisita and linear iga bullous dermatosis. Such a case has not been previously reported.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
5/7. Paraneoplastic epidermolysis bullosa acquisita associated with multiple myeloma.epidermolysis bullosa acquisita is a rare acquired autoimmune subepidermal blistering disease that clinically resembles other vesiculobullous lesions such as pemphigus vulgaris and cicatricial pemphigoid. multiple myeloma is the most common plasma cell malignant disorder characterized by a single clonal expansion and increased level of a single immunoglobulin. epidermolysis bullosa acquisita has been reported with other systemic diseases such as lymphoma. In this case report, we present a patient with paraneoplastic epidermolysis bullosa acquisita associated with multiple myeloma.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
6/7. Circulating anti-glomerular basement membrane antibodies in coeliac disease and epidermolysis bullosa acquisita.The demonstration of circulating anti-glomerular basement membrane (GBM) antibodies is almost diagnostic for anti-GBM disease and Goodpasture's syndrome. These antibodies are, however, occasionally present in SLE and diabetes, in association with IgA disease and membranous nephropathy and after transplantation in Alport's syndrome. In addition, we describe circulating anti-GBM antibodies in a research worker who handled GBM and in whom coeliec disease later developed, and in an individual with epidermolysis bullosa acquisita. Neither patient had impaired renal function nor an abnormal urinary sediment, suggesting either that these antibodies were of low affinity, or that additional factors are required for the pathogenesis of an aggressive glomerular lesion when circulating anti-GBM antibodies are present. In at least one of these individuals anti-GBM antibodies may have developed after the exposure of basement membrane collagen type iv to activated immunological mediators and cells.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
7/7. Epidermolysis bullosa aquisita with basal epidermal cytoplasmic antibodies.A 45-year-old woman with epidermolysis bullosa aquisita is presented. The clinical, histological, and immunopathological features were in keeping with the previous reports of this disease. The patient also had anti-basal cell cytoplasmic antibodies at a significant titer, which is considered an unusual finding associated with this disorder. Treatment with a moderate dose of corticosteroid was effective in controlling the bullous lesions.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |