Cases reported "Pemphigoid, Bullous"

Filter by keywords:



Filtering documents. Please wait...

1/28. epidermolysis bullosa acquisita with combined features of bullous pemphigoid and cicatricial pemphigoid.

    epidermolysis bullosa acquisita (EBA) is an acquired subepidermal blistering disease associated with autoantibodies against type VII collagen. The classical or mechanobullous form of EBA is characterized by skin fragility, trauma-induced blisters and erosions with mild mucous membrane involvement and healing with scars. Furthermore, bullous-pemphigoid-like and cicatricial pemphigoid-like features have been described. We report a patient who developed a bullous skin disease with upper airway obstruction requiring tracheotomy. The diagnosis of EBA was established by immunoblot, showing a band at 290 kD (collagen VII), and NaCl-split skin immunofluorescence (IgG deposition at the floor of the split). This case presented with clinical features of both bullous pemphigoid and cicatricial pemphigoid which to our knowledge is the first report of such a combination in EBA. The patient also presented tracheal involvement that has never been described either.
- - - - - - - - - -
ranking = 1
keywords = bullosa acquisita, acquisita, bullosa
(Clic here for more details about this article)

2/28. A subepidermal bullous eruption associated with IgG autoantibodies to a 200 kd dermal antigen: the first case report from the united states.

    We describe an 81-year-old white man in whom a subepidermal bullous eruption developed that clinically resembled bullous pemphigoid. The eruption promptly responded to oral tetracycline and niacinamide and topical clobetasol. Histologic examination of perilesional skin revealed neutrophilic infiltration with formation of papillary microabscesses and subepidermal cleavage. Direct immunofluorescence showed linear deposition of IgG and C3 along the basement membrane zone. By indirect immunofluorescence, circulating IgG autoantibodies bound exclusively to the dermal side of salt-split normal human skin. Immunoblot analysis demonstrated that the patient's autoantibodies reacted with a 200 kd dermal protein that was different from type VII collagen, the epidermolysis bullosa acquisita autoantigen. This patient represents the first confirmed case from the united states with a recently reported novel autoimmune subepidermal bullous disease associated with IgG autoantibodies to a 200 kd dermal antigen.
- - - - - - - - - -
ranking = 0.94558712683108
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
(Clic here for more details about this article)

3/28. Eosinophilic spongiosis in association with bullous pemphigoid and chronic lymphocytic leukaemia.

    We report the onset of an unusual blistering eruption following a diagnosis of B-cell chronic lymphocytic leukaemia (CLL). The histology consistently showed eosinophilic spongiosis, but the diagnosis of bullous pemphigoid was only confirmed after 13 years by repeated immunofluorescence studies. The occurrence of subepidermal blistering diseases in association with B-cell lymphoproliferative disorders is rare; a recent study showed that the majority of these cases are epidermolysis bullosa acquisita, confirmed by immunological studies. Only two cases of immunofluorescence-proven bullous pemphigoid in association with CLL have been previously reported.
- - - - - - - - - -
ranking = 0.94558712683108
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
(Clic here for more details about this article)

4/28. 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.
- - - - - - - - - -
ranking = 0.94558712683108
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
(Clic here for more details about this article)

5/28. A Korean case of anti-p200 pemphigoid.

    Anti-p200 pemphigoid is a newly defined autoimmune subepidermal blistering disease, which is characterized by the presence of IgG autoantibodies to the dermal side of 1 M NaCl split skin as well as by the reactivity of these antibodies to a novel 200-kDa antigen on immunoblot analysis of a dermal extract. We describe a 49-year-old Korean male who presented with a bullous eruption on the whole body, which clinically resembled bullous pemphigoid or epidermolysis bullosa acquisita. A histopathological examination of a lesional skin biopsy specimen showed an area of dermal-epidermal separation and mixed dermal inflammatory infiltrates consisting of lymphocytes, neutrophils, and eosinophils. Direct immunofluorescence showed a linear deposition of IgG and C3 along the basement membrane zone. Indirect immunofluorescence demonstrated circulating IgG autoantibodies directed against the dermal side of the 1 M NaCl split skin. Immunoblot analysis of dermal extracts revealed the patient's sera recognized the 200-kDa antigen. This is the first Korean case of an anti-p200 pemphigoid who showed good response to the treatment with systemic corticosteroids and dapsone.
- - - - - - - - - -
ranking = 0.94558712683108
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
(Clic here for more details about this article)

6/28. Paraneoplastic immunobullous disease with an epidermolysis bullosa acquisita phenotype: two cases demonstrating remission with treatment of gynaecological malignancy.

    Two cases of paraneoplastic immunobullous disease occurring in women with gynaecological malignancies are reported. Both cases demonstrated mechanobullous mucocutaneous blistering as is typically seen in epidermolysis bullosa acquisita. Their immunopathology, however, favoured a dermal-binding mucous membrane pemphigoid (MMP) (or possibly bullous pemphigoid) for patient 1 and laminin-5 MMP for patient 2. Both patients showed resolution of blistering within 1 year of treatment of their malignancies; uterine and ovarian carcinoma, respectively. These cases are of interest because of their paraneoplastic nature; as well as overlapping clinicoimmunopathological features. In addition, patient 2 is, as far as we are aware, the first report of ovarian-carcinoma-associated laminin-5 MMP.
- - - - - - - - - -
ranking = 4.7279356341554
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
(Clic here for more details about this article)

7/28. A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading.

    Bullous pemphigoid (BP) and epidermolysis bullosa acquisita are distinct autoimmune blistering disorders. BP is characterized by autoantibodies directed against the NC16A domain of collagen XVII, whereas patients with epidermolysis bullosa acquisita have autoantibodies against the NC1 domain of type VII collagen. We followed up a patient with BP for 9 years. During that time his clinical disease took on several features suggestive of epidermolysis bullosa acquisita. The objective of this study was to determine if the patient's autoantibody profile reflected the change in his clinical picture. Enzyme-linked immunosorbent assay and immunoblotting for detection and subclass determination of autoantibodies to type XVII and type VII collagen were performed on banked patient sera from the 9-year period. The patient's initial autoantibodies were exclusively IgG1 directed against collagen XVII. During the course of his illness, the subclass specificity of the patient's type XVII collagen autoantibodies shifted to the IgG4 subclass and during the same time interval the patient developed IgG2 autoantibodies directed against type VII collagen. This patient with BP exhibited both subclass shifting and development of a second autoantibody system that correlated with a change in the clinical appearance of the disease. The analysis of the patient's autoantibodies provides strong evidence for the involvement of epitope spreading in the evolution of his autoimmune disease.
- - - - - - - - - -
ranking = 6.6191098878176
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
(Clic here for more details about this article)

8/28. A case of anti-p200 pemphigoid with autoantibodies against both a novel 200-kD dermal antigen and the 290-kD epidermolysis bullosa acquisita antigen.

    BACKGROUND: Anti-p200 pemphigoid with autoantibodies against the 200-kD dermal antigen has recently been identified. OBJECTIVES: Our patient showed small and tense blisters on her face and trunk. methods: immunoblotting (IB), using extracts of normal human epidermis and dermal skin, and immunoelectron microscopy (IEM), using normal human skin, were performed using the patient's serum. RESULTS: IB analysis showed that the patient's serum did not react with 180-kD bullous pemphigoid (BP180) or BP230 antigens; however, IgG autoantibodies in the patient's serum reacted with a 200-kD dermal antigen as well as the 290-kD epidermolysis bullosa acquisita (EBA) antigen. IEM showed that IgG antibodies in the patient's serum bound to the lamina lucida, as well as both the lamina densa and sublamina densa. After the treatment with prednisolone, the 290-kD protein reactivity decreased rapidly; however, the 200-kD protein band was still observed. CONCLUSION: We describe a rare case with immunoreactive autoantibodies against both a novel dermal 200-kD autoantigen and the 290-kD EBA antigen.
- - - - - - - - - -
ranking = 4.7279356341554
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
(Clic here for more details about this article)

9/28. A case of epidermolysis bullosa acquisita with autoantibody to anti-p200 pemphigoid antigen and exfoliative esophagitis.

    BACKGROUND: Some cases of a subepidermal blistering disease associated with autoantibodies to more than two antigens have been reported. observation: A 52-year-old Japanese woman had pruritic blisters on almost the whole body as well as erosive lesions in the oral cavity and esophagus. A histological finding was subepidermal bullae. Direct immunofluorescence (IF) revealed a linear deposition of IgG, IgM and C3 at the epidermal basement membrane zone (BMZ). Indirect IF using human skin split by 1 M NaCl as a substrate showed IgG antibody reactive with the dermal side. By immunoblot analysis using normal human dermal extract, the 200-kDa and 290-kDa bands were detected. Indirect IF did not show any anti-BMZ antibody activity, when using the skin of the patient with recessive dystrophic epidermolysis bullosa as a substrate. CONCLUSION: We regarded our case as epidermolysis bullosa acquisita with autoantibody to anti-p200 pemphigoid antigen. This is the second case in the literature associated with autoantibodies to these two antigens.
- - - - - - - - - -
ranking = 4.8283752287149
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
(Clic here for more details about this article)

10/28. Acquired melanocytic naevus in childhood vulval pemphigoid.

    BACKGROUND: Eruptive epidermolysis bullosa (EB) naevi comprise a subset of melanocytic naevi with atypical features that characteristically occur in areas of former blistering in patients suffering from hereditary EB. observation: The case is reported of a girl who presented with pruritus, blistering and erosions of the vulval region. Clinical and immunopathological features were consistent with the diagnosis of childhood vulval pemphigoid. In the course of the disease, she developed an atypical melanocytic naevus on the left labium at a site of former blistering. Although its clinical and dermoscopic features resembled malignant melanoma, the lesion completely regressed clinically during the 24-month follow-up. CONCLUSION: This is the first report describing the development of a melanocytic naevus at sites of blistering in an auto-immune subepidermal blistering disease in childhood. Our observation extends the spectrum of disorders, in addition to the group of congenital EB, in which 'eruptive' atypical melanocytic naevi may occur. knowledge of this complication is important for appropriate management and follow-up and to avoid radical surgery.
- - - - - - - - - -
ranking = 0.10043959455947
keywords = epidermolysis bullosa, epidermolysis, bullosa
(Clic here for more details about this article)
| Next ->


Leave a message about 'Pemphigoid, Bullous'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.