Cases reported "Chronic Disease"

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1/12. epidermolysis bullosa acquisita in a 3 1/2-year-old girl.

    A 3 1/2-year-old girl had a subepidermal bullous eruption with immunopathologic features that were consistent with epidermolysis bullosa acquisita or bullous systemic lupus erythematosus. This report highlights the difficulty encountered in distinguishing between epidermolysis bullosa acquisita and other bullous disorders that involve the dermoepidermal junction and the need for modern immunologic investigations in the diagnosis of bullous diseases in children.
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ranking = 1
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, epidermolysis, acquisita, bullosa
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2/12. Altered expression of L-arginine metabolism pathway genes in chronic wounds in recessive dystrophic epidermolysis bullosa.

    Individuals with the severe, mutilating Hallopeau-Siemens form of recessive dystrophic epidermolysis bullosa (HS-RDEB) have trauma-induced blisters and skin erosions which often progress to wounds that are slow to heal. These chronic wounds cause considerable morbidity and there is an increased risk of squamous cell carcinoma arising in the wound margins. Currently, little is known about the keratinocyte cell biology in these wounds. Therefore, we compared the gene expression profiles of wound edge with nonwounded skin from two individuals with HS-RDEB. Trauma-induced wound sites had been present in both patients for more than 3 months. Hybridizations using DermArray gene expression filters showed relative differences in gene expression between wounded and unwounded skin. Notably, there was a fivefold increase in expression of arginase-1 (ARG1) in the chronic wound samples. Expression of seven other genes relevant to L-arginine metabolism also showed differences greater than twofold. L-arginine is known to have a critical role in the synthesis of nitric oxide as part of normal tissue repair. Although alterations in arginase isoenzymes have been detected previously in other chronic wounds (human and animal models), this is the first study to demonstrate differences in several components of the L-arginine metabolism pathway in chronic wounds, and the first to examine chronic wounds in HS-RDEB. The data show that the cascade of L-arginine metabolites is altered in HS-RDEB and the findings may provide new insight into the pathology of chronic wounds in this genodermatosis.
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ranking = 0.29253456176283
keywords = epidermolysis bullosa, epidermolysis, bullosa
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3/12. Chronic cicatrizing conjunctivitis in a patient with epidermolysis bullosa acquisita.

    OBJECTIVE: To describe a nonconventional diagnostic technique used to diagnose a case of cicatrizing conjunctivitis associated with epidermolysis bullosa acquisita. methods: Direct immunofluorescence of a biopsy specimen of the patient's conjunctiva was performed using fluorescein-conjugated rabbit antihuman antibodies against IgA, IgG, and IgM; complement c3; and fibrinogen. Immunoblot assay using healthy human skin as substrate was performed to investigate for the presence of antibodies in the patient's serum. After the diagnosis of systemic autoimmune disease was established, intravenous immunoglobulin therapy was administered. RESULTS: Direct immunofluorescence of the conjunctiva revealed linear deposition of IgA and IgG, and C3 at the epithelial basement membrane zone. Immunoblot analysis demonstrated the presence of IgG antibodies in patient serum directed against a 290-kDa protein in human skin. A diagnosis of epidermolysis bullosa acquisita was established. All signs and symptoms improved dramatically 4 months after initiation of intravenous immunoglobulin therapy and remained stable during follow-up. CONCLUSIONS: epidermolysis bullosa acquisita can manifest in the eye as chronic cicatrizing conjunctivitis indistinguishable from ocular cicatricial pemphigoid. A nonconventional diagnostic tool (immunoblot assay) might be helpful in establishing the diagnosis of an underlying systemic autoimmune disease in patients with chronic cicatrizing conjunctivitis. Intravenous immunoglobulin therapy was effective against chronic cicatrizing conjunctivitis associated with epidermolysis bullosa acquisita.
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ranking = 2.5616404249796
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, epidermolysis, acquisita, bullosa
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4/12. Childhood epidermolysis bullosa acquisita. Report of three cases and review of literature.

    epidermolysis bullosa acquisita is an acquired subepidermal blistering disease with variable clinical, pathologic, and immunologic features. The disease has been reported infrequently in adults and only rarely in children. We describe three new cases of childhood epidermolysis bullosa acquisita, review three previously reported cases, and contrast the features of the disease in children with those in adults. The results suggest that both children and adults with epidermolysis bullosa acquisita have variable clinical and pathologic features that may mimic other bullous diseases. epidermolysis bullosa acquisita is characterized by a chronic course, poor response to therapy, and occasional clinical remissions.
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ranking = 2.278184942292
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, epidermolysis, acquisita, bullosa
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5/12. Generalized atrophic benign form of junctional epidermolysis bullosa.

    There are at least six variants of junctional epidermolysis bullosa (JEB). About 20 cases of the generalized atrophic benign variant of JEB (GABEB) have been previously reported. We present an additional case of GABEB, occurring in a 14-year-old girl. Generalized cutaneous blisters occurred since birth and healed without severe scarring or milia, but with slight atrophy. In addition, mucous membrane involvement and hair, nail and tooth abnormalities were found. Electron microscopic examination showed a cleavage within the lamina lucida and the presence of numerically and structurally abnormal hemidesmosomes.
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ranking = 0.29253456176283
keywords = epidermolysis bullosa, epidermolysis, bullosa
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6/12. Pyocele of the middle turbinate.

    Mucoceles are uncommon and the majority occur in the fronto-ethmoidal complex. We present the first case of a secondarily infected mucocele arising from a concha bullosa of the middle turbinate. It was excised intranasally with complete resolution of symptoms.
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ranking = 0.0038925733689946
keywords = bullosa
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7/12. epidermolysis bullosa acquisita: efficacy of high-dose intravenous immunoglobulins.

    A 16-year-old boy had a 6-year history of a generalized bullous eruption that was resistant to multiple therapies. Findings of immunofluorescent split-skin studies and electron microscopy were consistent with a diagnosis of epidermolysis bullosa acquisita. Treatment with cyclosporine and prednisolone decreased new blister formation. Additional therapy with high-dose intravenous immunoglobulins was successful in controlling the patient's disease.
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ranking = 0.64436301154161
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, epidermolysis, acquisita, bullosa
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8/12. Brunsting-Perry cicatricial bullous pemphigoid: a clinical variant of localized acquired epidermolysis bullosa?

    An 84-year-old man who had the typical clinical features of Brunsting-Perry cicatricial pemphigoid is described. Direct immunofluorescence microscopic examination of salt-split skin revealed linear deposits of IgG and C3 on the floor of the artificial bullae. Direct immunoelectron microscopic examination of peribullous skin showed dermal cleavage level below the lamina densa and granular deposits of IgG and C3 attached to and below the lamina densa in a pattern identical to epidermolysis bullosa acquisita. These findings suggest that Brunsting-Perry cicatricial pemphigoid may represent a clinical variant of epidermolysis bullosa acquisita.
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ranking = 0.94530162632705
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, epidermolysis, acquisita, bullosa
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9/12. Surgical management of esophageal stricture due to recessive dystrophic epidermolysis bullosa.

    Esophageal stricture may occur as a complication of the dystrophic recessive form of epidermolysis bullosa. Mechanical dilatation by bougienage may cause more severe esophageal injury. The fourth reported case of esophageal replacement by colonic interposition for this condition is presented. Central venous total parenteral alimentation was a helpful adjunct in the surgical management of this patient. Colonic replacement is preferred over local resection of an esophageal stricture because in most instances there is extensive inflammatory involvement of the entire esophagus.
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ranking = 0.29253456176283
keywords = epidermolysis bullosa, epidermolysis, bullosa
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10/12. Chronic recurrent esophageal strictures treated with balloon dilation in children with autosomal recessive epidermolysis bullosa dystrophica.

    Two children (8- and 17-yr old) with autosomal recessive epidermolysis bullosa dystrophica and chronic esophageal strictures were treated with repeated balloon dilations. General anesthesia was by face mask adapted specifically for this procedure, with careful attention to avoid skin and mucus membrane damage. A balloon dilator positioned fluoroscopically over an angiographic guidewire was gently inflated until narrowings resolved. Dilations have been performed every 1-11 [4.3 /- 3.2 (mean /- SD)] months in the younger patient over the last 4.3 yr, and every 8-20 (14.5 /- 5.9) months in the older patient over the last 4.8 yr, without serious complications. Both patients were able to swallow normal foods following dilations. Repeated balloon dilations of esophageal strictures in patients with epidermolysis bullosa dystrophica can be done safely. Further studies are indicated to determine its effectiveness and appropriateness as an alternative to colonic interposition.
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ranking = 0.3510414741154
keywords = epidermolysis bullosa, epidermolysis, bullosa
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