Cases reported "Recurrence"

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1/10. Congenital neonatal herpes simplex virus infection.

    Intrauterine-acquired neonatal herpes simplex virus infection is a severe, often life-threatening infection that has a wide spectrum of clinical presentations. We describe a 31-week-gestation male who had a rarely reported epidermolysis bullosa-like clinical presentation. He acquired herpes simplex virus type II in utero secondary to a primary maternal infection that occurred on the knee. A review of the infection, the need for early therapy, and the prognosis are presented.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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2/10. 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 = 11.224472459379
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, bullosa acquisita, epidermolysis, acquisita, bullosa
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3/10. Remission of recalcitrant epidermolysis bullosa acquisita (EBA) with colchicine monotherapy.

    A 42-year-old woman with widespread recalcitrant epidermolysis bullosa acquisita (EBA) responded well to colchicine monotherapy after attempts to control her disease with other modalities failed. These included dapsone, tetracycline, prednisolone, and combination of cyclophosphamide/azathioprine with dexamethasone 'pulse therapy'. The useful role of colchicine in controlling some difficult cases of EBA, a therapeutically problematic condition, is stressed.
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ranking = 20.122254526445
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, bullosa acquisita, epidermolysis, acquisita, bullosa
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4/10. cyclosporine controls epidermolysis bullosa acquisita co-occurring with acquired factor viii deficiency.

    BACKGROUND: epidermolysis bullosa acquisita (EBA) is a rare antibody-mediated autoimmune blistering disease of adults. Likewise, acquired factor viii deficiency is a rare antibody-mediated disease of adulthood. Both diseases can be exceedingly difficult to treat and refractory to immunomodulatory therapies. methods: Herein, we report a challenging case of EBA and acquired factor viii deficiency in the same patient. RESULTS: cyclosporine 4 mg/kg/day rapidly controlled both disease processes after the patient failed to respond to prednisone, colchicine, and pulse cyclophosphamide. The EBA relapsed when the cyclosporine was decreased to 2 mg/kg/day and the patient wore snuggly fitting new shoes, but it cleared quickly when the dose was increased. cyclosporine has since been decreased gradually to 1.5 mg/kg/day without relapse of either condition or detectable side-effects. CONCLUSIONS: cyclosporine should be considered when EBA and/or acquired factor viii deficiency fail to respond to conservative therapy. Both conditions usually respond rapidly to cyclosporine 4-6 mg/kg/day.
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ranking = 16.891696283357
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, bullosa acquisita, epidermolysis, acquisita, bullosa
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5/10. Recurrent bilateral corneal erosions due to an association of epidermolysis bullosa simplex Kobner and X-linked ichthyosis with steroid sulfatase deficiency.

    Since early infancy a male patient now 24 years of age had suffered from painful, recurrent, bilateral corneal erosions and blister formation after minimal skin trauma. Corneal erosions are quite unusual in non-scarring types of epidermolysis bullosa. The dermatological examination disclosed that the patient had clinical hallmarks of two rare genetic skin disorders, epidermolysis bullosa simplex (Kobner) and X-linked ichthyosis. Both disorders could be traced back in the patient's maternal family to the mother's father and his brother, who were both said to have had similar eye complaints. Using biochemical means and electron microscopy of skin biopsy specimens, both diagnoses could be proven in the patient and his mother. We discuss the importance of lid hyperkeratoses in hemizygous males as a causative factor for the recurrence of bilateral corneal erosions due to the unique combination of epidermolysis bullosa simplex (Kobner) and X-linked ichthyosis with steroid sulfatase deficiency.
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ranking = 7
keywords = epidermolysis bullosa, epidermolysis, bullosa
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6/10. epidermolysis bullosa simplex Dowling-Meara: troublesome blistering and pruritus in an adult patient.

    A 46-year-old woman with the Dowling-Meara variant of epidermolysis bullosa simplex (DM-EBS) presented with worsening recurrent, pruritic, circinate crops of clear and haemorrhagic herpetiform blisters affecting her trunk and limbs. Electron microscopy showed tonofilament aggregation and an intra-epidermal level of blister formation confirming a diagnosis of DM-EBS rather than an acquired immunobullous disorder. Antihistamines failed to control the intense pruritus, but dapsone (up to 150 mg daily) was beneficial. Mutations of basal cell keratin genes (K14 and K5) are thought to be of aetiopathological significance in this form of epidermolysis bullosa, but the underlying cellular mechanisms responsible for the clinical deterioration and severe itching in this adult patient are not yet clear.
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ranking = 2.3147717634638
keywords = epidermolysis bullosa, epidermolysis, bullosa
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7/10. 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 = 6
keywords = epidermolysis bullosa, epidermolysis, bullosa
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8/10. Angina bullosa hemorrhagica.

    Angina bullosa hemorrhagica is characterized by acute blood blisters, mainly on the soft palate. Elderly patients are usually affected and lesions heal spontaneously without scarring. The pathogenesis is unknown, although it may be a multifactorial phenomenon. Trauma seems to be the major provoking factor and long term use of steroid inhalers has also been implicated in the disease. No underlying hematologic or immunopathogenic disorder has been found. Treatment is symptomatic. We present a 67-year-old patient with recurrent oral blood blisters which were diagnosed as angina bullosa hemorrhagica. Trauma by dental injections and use of steroid inhalers were identified as etiologic factors in this case. Erosions healed with a week. Although this is a benign condition, it may result in acute airway obstruction. Recognition is, therefore, of great importance for dermatologists.
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ranking = 0.47215764519576
keywords = bullosa
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9/10. epidermolysis bullosa acquisita localized to the face.

    A 39-year-old woman had a three-year history of recurrent bullous eruption localized to her left cheek. The diagnosis of epidermolysis bullosa acquisita was confirmed by means of direct immunofluorescence and direct immunoelectron microscopic studies performed on the perilesional salt-split skin. Topical corticosteroid treatment reduced pruritus and bullae formation. This case of localized epidermolysis bullosa acquisita on the face is reminiscent of Brunsting-Perry cicatricial pemphigoid. We also review the previously reported cases of localized epidermolysis bullosa acquisita.
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ranking = 15.248923364669
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, bullosa acquisita, epidermolysis, acquisita, bullosa
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10/10. A case of dystrophic epidermolysis bullosa: surgical treatment for hand contracture using abdominal skin flap.

    The patient was a 15-year-old male. Since birth, he has suffered from recurrent blistering, especially on the extremities. histology and electron microscopic finding of the cutaneous lesions were compatible with those of dystrophic epidermolysis bullosa. contracture and mobility limitation of the hands had gradually deteriorated. Prior to the admission to our clinic, both hands' contracture had been surgically treated 4 times in another hospital; however, after treatment with skin grafting, hand contracture recurred each time within one year. This time, surgical treatment was carried out for his left hand contracture using an abdominal skin flap under local anesthesia. In comparison with the free skin graft the abdominal skin flap improved hand contracture for a longer time. Abdominal skin flap is recommended as a method for the treatment of hand contracture of patients with dystrophic epidermolysis bullosa.
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ranking = 6
keywords = epidermolysis bullosa, epidermolysis, bullosa
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