Cases reported "pemphigoid, bullous"

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1/351. Childhood bullous pemphigoid: report of a case with characterization of the targeted antigens.

    The clinical and immunopathologic features of children with acquired subepidermal blistering disorders show considerable overlap, and their classification frequently requires characterization of the targeted antigens. A 8-month-old boy developed a generalized subepidermal blistering disorder with striking palmoplantar involvement. The patient's serum contained antibodies reacting against the epidermal side of 1 M sodium chloride separated normal human skin. immunoblotting analysis demonstrated circulating IgG autoantibodies that reacted against a eukaryotic recombinant form of human bullous pemphigoid antigen 180 (BP180). In addition, the patient had circulating IgG autoantibodies that bound a protein of 120 kDa in skin basement membrane zone extracts, that might correspond to the linear IgA bullous disease (LABD) antigen. This study illustrates that a child with clinical and immunopathologic features considered characteristic of childhood bullous pemphigoid (BP) had circulating IgG antibodies that bound to an eukaryotic recombinant form of human BP180, and hence, fulfilled the diagnostic criteria of BP. review of the literature disclosed only 10 cases of childhood BP, that were characterized on the basis of the targeted antigens. The concomitant presence of circulating IgG autoantibodies against BP180 and a 120 kDa protein may signify either coexistence of autoantibodies with distinct specificities or reflect antigenic cross-reactivity between BP180 and the 120/97 LABD antigen. ( info)

2/351. Childhood bullous pemphigoid associated with IgA antibodies against BP180 or BP230 antigens.

    linear iga bullous dermatosis (LABD) comprises a heterogeneous group of subepidermal blistering disorders characterized by in situ bound IgA antibodies in epidermal basement membrane. We report three children presenting clinical and immunopathological features characteristic of LABD. By immunoblotting, the three patients' sera contained IgA antibodies that reacted against the bullous pemphigoid (BP) antigen 180 and or BP230, molecular markers for BP. In addition, IgG antibodies directed against the ectodomain of BP180 were detected by an enzyme-linked immunosorbent assay using a eukaryotic recombinant form of BP180. Consistent with recent studies suggesting that the LABD antigen 1, the predominant autoantigen of LABD, is either a proteolytic product of BP180 or an isoform of the BP180 gene, our findings indicate that a subset of children with features of LABD have a distinct form of BP associated with an IgA response. ( info)

3/351. Immunoadsorption for the treatment of bullous pemphigoid.

    We treated 2 intractable patients with pemphigoid by absorbent plasmapheresis using dextran sulfate conjugated cellulose columns with an automated regenerating unit. During a 2 week period, this plasmapheresis was performed 4 to 6 times for Cases 1 and 2, respectively. Clinical findings including skin eruptions were remarkably improved, and the titers of antibasement membrane zone antibodies were decreased after the treatment of 1.8 to 4.2 L of plasma. These results suggest that the absorbent column is effective for intractable pemphigoid. ( info)

4/351. Childhood vulval pemphigoid: a clinical and immunopathological study of five patients.

    We describe five girls with vulval pemphigoid: two had bullous pemphigoid confined to the vulva and three had cicatricial pemphigoid. They demonstrate a spectrum of severity from localized disease to extensive vulval scarring necessitating long-term immunosuppressive therapy and surgical correction. The age at onset of their disease ranged between 6 and 13 years. All presented with vulval discomfort and erosions. Three had oral lesions, two perianal and one eye and cutaneous involvement. Two girls with only vulval lesions and one with vulval and oral lesions responded well to topical steroids. In two, systemic treatment with prednisolone and dapsone or azathioprine was required. The diagnosis was made on the basis of histology and immunofluorescence (IF). All had positive direct IF with IgG and C3. Indirect IF demonstrated circulating IgG binding to the basement membrane zone in four, with dermal or epidermal binding on salt-split skin substrate. immunoblotting revealed antibodies to the BP230 and BP180 antigens. Immunoelectron microscopy in the child with dermal binding IgG and BP180 and BP230 on immunoblotting showed labelling at the lamina densa-lamina lucida interface adjacent to hemidesmosomes. ( info)

5/351. Resolution of bullous pemphigoid and improvement of vitiligo after successful treatment of squamous cell carcinoma of the skin.

    The significance of the association of malignant diseases with bullous pemphigoid is still unknown. We report a case of squamous cell carcinoma of the skin associated with both bullous pemphigoid and vitiligo. It is possible that there is a common underlying pathogenic mechanism involved in the co-existence of these three skin diseases as successful treatment of the carcinoma was accompanied by resolution of the bullous pemphigoid and improvement of the vitiligo. ( info)

6/351. Severe subepidermal blistering disorder with features of bullous pemphigoid and herpes gestationis.

    Herpes gestationis (HG) and bullous pemphigoid (BP) are blistering disorders with similar features, including urticarial lesions that progress to blisters and immunodeposition of C3 in a linear pattern at the basement membrane zone. Among their differences, HG is distinguished by its association with pregnancy, the puerperium, or hormonal perturbation. We describe the immunopathologic findings and clinical course in a multiparous woman with a severe blistering eruption. The patient was not pregnant. Malignancy evaluation was negative, and hormonal testing was normal. Histologic examination demonstrated a subepidermal bulla with eosinophils. Direct immunofluorescence showed C3 in a strong linear band at the dermal-epidermal junction. Indirect immunofluorescence demonstrated circulating IgG and IgG3 antibodies to basement membrane zone (epidermal component on salt-split skin), and complement-fixing IgG. immunoprecipitation demonstrated antibodies to a 180-kd keratinocyte protein antigen. By clinical definition, this patient has BP. However, her disease presentation demonstrated features of both BP and HG. ( info)

7/351. Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin-6, and type VII collagen.

    BACKGROUND: Bullous systemic lupus erythematosus is a generalized subepidermal blistering skin eruption in patients suffering from systemic lupus erythematosus. Type VII collagen was initially identified as the target antigen. observation: We studied an unusual patient who had bullous systemic lupus crythematosus. The patient fulfilled the criteria of systemic lupus with an antinuclear antibody titer of 1:5120. Immunopathological testing revealed in vivo deposition of all IgG subclasses, secretory IgA1, and both light chains at the patient's skin basement membrane. The in vivo-bound IgG and IgA were localized at the hemidesmosomes and lamina densa. The patient's IgG and IgA circulating autoantibodies labeled both the epidermal roof and the dermal floor of salt-split skin and recognized the hemidesmosomal protein BP230 as well as the full-length native form and the recombinant noncollagenous domain 1 of type VII collagen (anchoring fibril). In addition, the patient's IgG autoantibodies recognized the anchoring filament proteins laminin-5 and laminin-6 (alpha3 chain and gamma2 chain). CONCLUSIONS: We conclude that patients with bullous systemic lupus erythematosus may have autoantibodies to multiple basement membrane components critical for epidermal-dermal junctional adhesion. Possible pathogenic mechanisms in this patient's clinical diseases include provocation of organ-specific disease (bullous disease) by systemic autoimmunity (lupus) and the "epitope spreading" immune phenomenon. ( info)

8/351. Corticosteroid-induced pancreatitis in patients with autoimmune bullous disease: case report and prospective study.

    Corticosteroid pulse therapy using very high doses may produce corticosteroid-induced pancreatitis (CIP) that is unexpected during conventional oral corticosteroid therapy and may sometimes be fatal. Our goal was to evaluate the relation between pulse corticosteroid administration and pancreatitis. A case of CIP is reported, and a prospective study was performed. Corticosteroid pulse therapy followed by 30 mg prednisolone orally was utilized in 7 hospitalized patients with autoimmune bullous disease, and serum pancreatic enzymes were measured during therapy. The case report revealed reproducible pancreatitis in a dose-dependent manner after 2 corticosteroid regimens. In the prospective study, serum pancreatic enzyme levels increased significantly within several days after pulse therapy, then decreased with tapering of the dose of oral prednisolone. Laboratory pancreatic alterations appear to be induced within days after pulse corticosteroid administration in a dose-dependent manner: less than 25 mg of oral prednisolone may be below threshold to alter the pancreatic enzyme level. ( info)

9/351. 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. ( info)

10/351. Demonstration of antibody to 230 kDa bullous pemphigoid antigen in lichen planus-like keratosis.

    We describe a 67-year-old man with lichen planus-like keratosis associated with anti-230 kDa bullous pemphigoid antigen (BPAG1) autoantibody. The patient had noticed solitary dark brown macule more than 6 years previously on his left chest. Histological findings showed hypergranulosis, irregular acanthosis, liquefaction degeneration of basal cells, band-like infiltration of lymphocytes at the subepidermal portion, and a cleft at the basement membrane zone (BMZ), resulting in the formation of subepidermal blisters. Direct immunofluorescence findings of perilesional skin showed a linear deposition of IgG at BMZ. On indirect immunofluorescent study using normal human skin, circulating IgG autoantibody to BMZ was present in the patient's serum at a titer of 1:80. The antigen located on the epidermal site of normal skin split by 1M NaCl was reacted with the patient's serum. Immunoblot analysis using epidermal extracts demonstrated the presence of IgG antibody directed to BPAG1 in the patient's serum. These observations suggest that the presence of an antibody to BPAG1 could be caused by the damage of basal cells following lichen planus-like keratosis. ( info)
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