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epidermolysis bullosa dystrophica/edema

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5 結果
Radiographic findings in two patients with uncommon manifestations of epidermolysis bullosa dystrophica are described. A girl with recurrent urinary tract infections had scarring of the external genitalia producing chronic vaginal and uterine reflux and retention of urine. A second patient with
A black baby, born at full term of a normal healthy mother, showed multiple erosions and ulcers. No history of skin diseases was known in the family. A skin biopsy taken shortly after birth from an arm lesion showed subepidermal edema and cavity formation. Electron microscopy showed clumping of
Previous reports of glomerular disease in adult patients with autosomal dominant dystrophic epidermolysis bullosa (EB) are limited and include post-infectious glomerulonephritis, IgA nephropathy, amyloidosis, and leukocytoclastic vasculitis. To our knowledge, membranoproliferative glomerulonephritis
BACKGROUND Absence of collagen VII causes blistering of the skin, eyes and many other tissues. This disease is termed dystrophic epidermolysis bullosa (DEB). Corneal fibrosis occurs in up to 41% and vision loss in up to 64% of patients. Standard treatments are supportive and there is no cure. The
BACKGROUND Epidermolysis bullosa acquisita is a subepidermal bullous disease characterized by IgG autoantibodies directed against type VII collagen in anchoring fibrils. These autoantibodies are believed to play an important role in the pathogenesis of sub-lamina densa blister formation in this
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