Cases reported "Facial Dermatoses"

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1/9. Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin alpha3.

    BACKGROUND: The inflammatory variant of epidermolysis bullosa acquisita (EBA) may clinically closely resemble bullous or cicatricial pemphigoid. patients with inflammatory EBA have IgG autoantibodies against type VII collagen. patients with anti-epiligrin cicatricial pemphigoid have IgG autoantibodies against laminin 5. observation: We describe a patient with inflammatory EBA exhibiting nonscarring oral and vaginal involvement. Indirect immunofluorescence using skin substrate lacking an epidermal basement membrane molecule, direct immunoelectron microscopy, immunoblot, and immunoprecipitation studies revealed the simultaneous presence of circulating IgG autoantibodies against type VII collagen and laminin alpha3. A final diagnosis of EBA was based on the sublamina densa level of blister formation. CONCLUSION: This case illustrates the clinical and immunological overlap between EBA and anti-epiligrin cicatricial pemphigoid, a unique finding that may have developed as a consequence of epitope spreading.
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ranking = 1
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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2/9. Autoimmune vesicles on the face and neck. A variant of Brunsting-Perry type localized bullous pemphigoid?

    We report a case of blistering disease presenting a unique distribution of vesiculobullous lesions on the face and neck which is similar to Brunsting-Perry type of localized bullous pemphigoid (BP). Histopathology of a lesional skin biopsy demonstrated a subepidermal blister. Direct immunofluorescence demonstrated a strong linear deposition of IgG and IgA to the basement membrane zone, and a faint staining for C3. However, circulating antibodies were not detected by indirect immunofluorescence and immunoblotting. And the patient did not develop atrophic scars and was a relatively young woman. This case might be a variant of Brunsting-Perry type of localized BP or localized epidermolysis bullosa acquisita, presenting the clinical heterogeneity of subepidermal blistering diseases.
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ranking = 0.2
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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3/9. 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.
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ranking = 0.16594015197555
keywords = bullosa acquisita, acquisita, bullosa
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4/9. Treatment of chronic erosions of junctional epidermolysis bullosa with human epidermal allografts.

    We have successfully treated chronic central facial erosions of a patient with junctional epidermolysis bullosa using cultured epidermal allografts. keratinocytes isolated from a skin biopsy of the patient's biological mother were used to generate the epidermal allografts. Significant re-epithelialization occurred during the course of four grafting procedures. Cultured epidermal allografts appear to be a promising method for treatment of chronic erosions of junctional epidermolysis bullosa.
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ranking = 0.15587566857919
keywords = epidermolysis bullosa, epidermolysis, bullosa
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5/9. Bazex syndrome (acrokeratosis paraneoplastica). An analytic review.

    Bazex syndrome (acrokeratosis paraneoplastica) is characterized by a psoriasiform eruption that favors acral sites and has been associated with an underlying malignancy in all reported cases. Of the 93 patients in this series, 89 were male with a mean age of 60 /- 8.5 years. Squamous cell carcinomas of the head and neck and squamous cell tumors of unknown primary with cervical lymph node metastases were the most commonly associated neoplasms, suggesting that the factor(s) responsible for the development of the syndrome are relatively specific for tumors of the upper aerodigestive tract. The cutaneous lesions were erythematous to violaceous in color and had associated scale; the most frequently observed sites of involvement were the ears, nose, hands, and feet, including the nails. In 63% of the cases, the cutaneous lesions preceded the initial symptoms or diagnosis of the tumor by an average of 11 months (range, 1-72) and, in general, the eruption was resistant to a variety of topical treatments. Occasionally, a reappearance of the papulosquamous lesions signaled the recurrence of the tumor (6 cases) or the appearance of skin lesions coincided with the development of metastatic disease (3 cases). In 91% (64/70) of the patients, the skin eruption either improved significantly following treatment of the underlying malignancy or did not improve in the setting of persistent tumor. However, even when all of the skin lesions cleared, the nail dystrophy often persisted. Fifteen of the patients developed vesicles, bullae, and crusts in addition to papulosquamous lesions. Possible explanations include the formation of an epidermal-dermal split via a bullous lichen planus-like mechanism, or the coexistence of two diseases; i.e., acrokeratosis paraneoplastica plus either porphyria cutanea tarda, bullous pemphigoid, or epidermolysis bullosa acquisita. One possible explanation for the development of the characteristic cutaneous eruption is an immune reaction, humoral or cellular, directed against a common antigen present on the tumor and the normal skin. Alternatively, tumor production of a keratinocyte growth factor such as TGF-alpha may be involved in the induction of the psoriasiform skin lesions.
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ranking = 0.2
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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6/9. Childhood bullous pemphigoid--report of a case with dermal fluorescence on salt-split skin.

    Bullous pemphigoid (BP) is an acquired bullous disorder which predominantly affects the elderly. It is rare in children, and may be clinically indistinguishable from other immunobullous disorders. As routine histology may be non-specific, a definitive diagnosis of childhood BP usually depends on the results of direct and indirect immunofluorescence investigations. We report a 5-year-old girl who developed bullous pemphigoid, associated with atypical immunofluorescence findings. Indirect immunofluorescence on split-skin showed a pure dermal pattern of IgG binding. This is usually suggestive of epidermolysis bullosa acquisita, but Western immunoblotting was positive with epidermal extracts, confirming a diagnosis of BP. Dermal binding on split-skin occurs in about 5% of adult cases of BP, and has not been reported previously in childhood BP.
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ranking = 0.2
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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7/9. Localized cicatricial pemphigoid of the Brunsting-Perry type with transition into disseminated cicatricial pemphigoid. Report of a case proved by preembedding immunogold electron microscopy.

    BACKGROUND: In 1979, Provost described two patients with the clinical features of disseminated cicatricial pemphigoid for the first time. Until now, only four additional cases of disseminated cicatricial pemphigoid have been described. Existence of diagnosis of disseminated cicatricial pemphigoid has been discussed controversially because in four cases investigated by electron microscopy the blister formation was found below the lamina densa, which is indicative of an epidermolysis bullosa acquisita. observation: A 78-year-old woman is presented with a generalized eruption of blisters leaving behind scars that developed after a 7-year-long history of mild circumscribed recurrent blisters and scarring eruptions that had been diagnosed previously as Brunsting-Perry type of cicatricial pemphigoid. Immunofluorescence antigen mapping disclosed the blister formation above the lamina densa. Electron and immunoelectron microscopy using a preembedding immunogold technique revealed blister formation and antibody binding within the lamina lucida, predominantly below the subbasal dense plate. CONCLUSIONS: The clinical features of disseminated blistering followed by scarring, the immunofluorescence antigen mapping, and the electron and immunoelectron microscopic findings in our case for the first time clearly prove the existence of a disseminated cicatricial pemphigoid.
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ranking = 0.2
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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8/9. Autosomal recessive epidermolysis bullosa simplex. A case report.

    We report a male child with autosomal recessive epidermolysis bullosa simplex presenting at birth. The patient subsequently developed cutaneous atrophy, nail dystrophy, milia and alopecia. He had growth retardation and anaemia, but there were no other associated abnormalities. Electron microscopy showed epidermolytic cleavage. The family history indicated an autosomal recessive mode of inheritance.
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ranking = 0.12989639048266
keywords = epidermolysis bullosa, epidermolysis, bullosa
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9/9. 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 = 0.76594015197555
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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