Cases reported "Cicatrix"

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1/15. Squamous cell carcinoma secondary to recessive dystrophic epidermolysis bullosa: report of eight tumours in four patients.

    BACKGROUND: The development of squamous cell carcinomas is the main cause of death of patients with Dystrophic epidermolysis bullosa. We think it is of interest to know their clinical characteristics and the treatment difficulties they cause. methods: We followed the clinical evolutions and carried out histopathological studies of eight primary cutaneous squamous cell carcinomas on three males and one female with recessive dystrophic epidermolysis bullosa. Patient ages ranged from 16 to 34 years (mean 27). RESULTS: Two patients had two tumours and one three, all were in characteristically scarred skin; four on upper limbs; four on lower. Maximum tumour dimensions ranged from 2 to 28 cm (mean 13), and represented 6 or more months of evolution. In three cases the affected limb must be amputated. Five tumours were well differentiated, three were moderately differentiated. CONCLUSIONS: The patients consulted too late and their large lesions needed aggressive treatment. Appropriate information and regular examination of patients with RDEB helps early diagnosis of tumours and may avoid disabling operations.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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2/15. Ongoing blistering in a boy with congenital erosive and vesicular dermatosis healing with reticulated supple scarring.

    Congenital erosive and vesicular dermatosis healing with reticulated supple scarring is a rare entity presenting in the newborn with crusted erosions and vesicles that heal relatively rapidly, forming unique reticulated scars. A 9-year-old boy presented with a diagnosis of junctional epidermolysis bullosa, but displayed this characteristic scarring pattern and very mild ongoing blistering. In addition, he had severe chronic conjunctivitis due to cicatricial alopecia of the eyelashes and lacrimal duct obstruction. He had no evidence of enamel defects or other features of junctional epidermolysis bullosa. Ultrastructural analysis of his skin biopsy specimens showed a normal dermoepidermal junction. This characteristic scarring disorder may be associated with mild ongoing blistering and must be distinguished from other congenital blistering disorders.
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ranking = 0.33333333333333
keywords = epidermolysis bullosa, epidermolysis, bullosa
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3/15. 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 = 2.8720684292296
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, epidermolysis, bullosa acquisita, bullosa, acquisita
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4/15. epidermolysis bullosa acquisita. Direct immunofluorescence and ultrastructural studies.

    A case of epidermolysis bullosa acquisita (EBA), associated with inflammatory bowel disease in which cicatricial alopecia was present, was studied by electron microscopy and direct immunofluorescence microscopy. Direct immunofluorescence studies were performed on both clinically normal and perilesional skin, with and without previous separation of the epidermis from the dermis by incubation with 1 M sodium chloride. We propose the use of this separation technique to identify the level of antibody deposition in patients with EBA in whom circulating antibodies are lacking. This technique may be particularly beneficial in delineating between EBA and the clinically similar scarring localized forms of bullous pemphigoid in which circulating antibodies are often absent.
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ranking = 0.65423416942796
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, epidermolysis, bullosa acquisita, bullosa, acquisita
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5/15. Bart's syndrome: microscopic, ultrastructural, and immunofluorescent mapping features.

    Bart's syndrome has been clinically described as the association of congenital localized absence of skin (CLAS), epidermolysis bullosa (EB), oral mucosal lesions, and dystrophic nails. Transmission occurs through an autosomal dominant gene with complete penetrance but variable expression. It has been difficult to classify this type of EB because of lack of microscopic and ultrastructural studies on affected family members. This is the first report of microscopic, ultrastructural, and immunofluorescent mapping studies of an affected individual with the complete inherited syndrome initially described by Bart. This study is also the first to document the association of CLAS and dominant dystrophic epidermolysis bullosa by histology, electron microscopy, and immunofluorescent mapping. Our two patients and one other affected family member had diminution or absence of a specific basement membrane antigen as defined by immunofluorescence with a monoclonal antibody (KF-1) in perilesional skin.
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ranking = 0.33333333333333
keywords = epidermolysis bullosa, epidermolysis, bullosa
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6/15. Cicatricial junctional epidermolysis bullosa.

    Six types of junctional epidermolysis bullosa (JEB) have been described previously. Each of these types may develop atrophy after recurrent blistering but healing with scar formation does not occur. We describe three cases with a new type of JEB that we have designated as cicatricial junctional epidermolysis bullosa (CJEB). This type is characterized clinically by blisters that heal with scarring and may result in syndactyly and contractures and by stenosis of the anterior nares. These cases were all believed to represent a dystrophic type of epidermolysis bullosa (EB) clinically and emphasize the necessity for electron microscopy of the skin in all EB patients with dystrophic features in order to make a definite diagnosis as to type. Electron microscopy revealed junctional bullae associated with rudimentary hemidesmosomes. Breaks in the basement membrane were noted in addition to active fibroblastic proliferation and laying down of new collagen.
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ranking = 1.1666666666667
keywords = epidermolysis bullosa, epidermolysis, bullosa
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7/15. Dystrophic epidermolysis bullosa. A new variant characterized by progressive symmetrical centripetal involvement with scarring.

    A patient is reported with a form of epidermolysis bullosa, hitherto undescribed to the best of our knowledge, characterized by the slow centripetal progression of symmetrical blister formation, milia, scarring, atrophy, and nail dystrophy. Electron microscopy, immunofluorescence mapping, and KF-1 monoclonal antibody studies confirm this disease to be a form of dystrophic epidermolysis bullosa, probably of autosomal recessive transmission despite the absence of acral deformities, contractures, mucosal involvement, and growth retardation.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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8/15. Esophageal involvement in epidermolysis bullosa dystrophica: clinical and roentgenographic manifestations.

    Epidermolysis bullosa is a rare hereditary mechanobullous skin disorder. Four patients with esophageal involvement are reported. These cases and review of reported cases of epidermolysis bullosa dystrophica-recessive (EBD-R) showed distinct clinical and roentgenographie features of esophageal involvement: diffuse inflammatory changes, motility disorders, small blisters or bullae seen as constant nodular-filling defects, esophageal ulcers, scars, pseudodiverticula, transverse and circumferential webs, various size strictures, shortening of the esophagus with development of traction hiatal hernia and gastroesophageal reflux, perforation, and complete obstruction of the esophageal lumen. Dysphagia is reversible when caused by bullae or webs and permanent when due to cicatrizing strictures. The strictures may remain unchanged in size for many years despite variations in dysphagic symptoms.
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ranking = 0.84266092851117
keywords = epidermolysis bullosa, epidermolysis, bullosa
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9/15. Upper airway and external genital involvement in epidermolysis bullosa dystrophica.

    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 long-standing stridor was found to have subglottic narrowing due to localized subglottic edema associated with an inflammatory membrane. The second case illustrates the occasional involvement of columnar epithelial surfaces which can occur in both epidermolysis bullosa dystrophica and epidermolysis bullosa hereditaria letalis.
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ranking = 1.1666666666667
keywords = epidermolysis bullosa, epidermolysis, bullosa
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10/15. Ultrastructural and immuno-electron microscopic study in a case of epidermolysis bullosa acquisita.

    skin biopsies from a patient with epidermolysis bullosa acquisita (EBA) were studied ultrastructurally and by immuno-electron microscopy. The specimens were taken from non-predilection areas as well as of the skin predilection areas of the patient. In vivo deposits of IgG were found below the lamina densa associated with the anchoring fibrils in all areas of the skin. The bullae and the artificial friction blisters appeared in the same position on the dermal side of lamina densa. The results suggest that epidermolysis bullosa acquisita is a distinct nosologic entity. The close resemblance of epidermolysis bullosa acquisita to cicatricial pemphigoid in immunofluorescence studies makes the use of immuno-electron microscopy important in the classification of the diseases.
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ranking = 1.6385069494641
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, epidermolysis, bullosa acquisita, bullosa, acquisita
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