1/10. A paraneoplastic mixed bullous skin disease: breakdown in tolerance to multiple epidermal antigens.We report a new type of paraneoplastic mixed bullous skin disease in a patient with a B-cell lymphoma associated with monoclonal IgM kappa paraproteinaemia. The patient's clinical and histological features were reminiscent of bullous pemphigoid. Characterization of antiepidermal antibodies by immunoblotting and indirect immunoelectron microscopy demonstrated a novel pattern of reactivity: IgG antibodies were directed against desmoplakins I-II and BPAG2, and were associated with antidesmoglein 3 polyclonal IgM antibodies. Such an autoreactive pattern involving antidesmoplakins, anti-BPAG2 and antidesmoglein 3 antibodies has not been previously reported. It reflects the breakdown of tolerance to multiple epidermal antigens observed in some patients with malignancy.- - - - - - - - - - ranking = 1keywords = bullous (Clic here for more details about this article) |
2/10. A bullous neutrophilic dermatosis in a patient with severe rheumatoid arthritis and monoclonal IgA gammopathy.A 35-year-old man presented with a 2-month history of itchy grouped vesiculobullae with crusted papules and confluent plaques, distributed symmetrically on the thighs, knees, and elbows. The man had experienced severe disabling seropositive rheumatoid arthritis for 18 years previously. Histologically, subepidermal vesicles were noted, and a dense, diffuse neutrophilic infiltration of the dermis. Direct immunofluorescence studies did not detect IgA deposits. The patient responded well to dapsone (100 mg/d) but not to systemic steroids. This patient's eruption might have been an unusual manifestation of rheumatoid neutrophilic dermatitis.- - - - - - - - - - ranking = 11.547051487475keywords = dermatosis, bullous (Clic here for more details about this article) |
3/10. Mucocutaneous bullous amyloidosis with an unusual mixed protein composition of amyloid deposits.We describe a case of fatal systemic amyloidosis presenting with mucocutaneous bullous lesions in a patient with IgA kappa monoclonal gammopathy. The amyloid plaques were composed of an unusual mixture of immunoglobulin kappa light chain and amyloid A proteins. Whereas oesophageal and oropharyngeal blisters are known to occur in several types of bullous dermatoses, to our knowledge this is the first report of oesophagopharyngeal blisters complicating bullous amyloidosis.- - - - - - - - - - ranking = 1.1666666666667keywords = bullous (Clic here for more details about this article) |
4/10. Subcorneal pustular dermatosis and IgA paraproteinaemia: response to both etretinate and PUVA.A non-insulin dependent male diabetic is reported with subcorneal pustular dermatosis associated with intraepidermal IgA deposits and a benign IgA paraproteinaemia. Treatment with dapsone and etretinate was reasonably effective, but etretinate had to be discontinued due to the development of diffuse idiopathic skeletal hyperostosis. His subcorneal pustular dermatosis subsequently flared and was troublesome for 2 years until he was commenced on PUVA, with excellent response.- - - - - - - - - - ranking = 5411.2064477701keywords = subcorneal pustular dermatosis, pustular dermatosis, subcorneal, dermatosis (Clic here for more details about this article) |
5/10. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) in association with a monoclonal IgA gammopathy: a report and review of the literature.Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) may be associated with a monoclonal gammopathy, most commonly an IgA paraproteinemia. This review looks at the available immunofluorescence staining data in the hope of finding a better characterization of this subset of patients and speculation as to the underlying pathophysiology.- - - - - - - - - - ranking = 1962.967621989keywords = pustular dermatosis, dermatosis (Clic here for more details about this article) |
6/10. A characteristic vesiculobullous eruption in patients with chronic lymphocytic leukemia.We report ten cases of a characteristic vesiculobullous eruption in patients with chronic lymphocytic leukemia. Clinically, six lesions were thought to represent insect bites. All ten patients had bone marrow examinations that confirmed the diagnosis of chronic lymphocytic leukemia. In six of ten patients serum protein and immunoelectrophoresis were performed. Six patients had immunoglobulin disturbances: one polyclonal IgG and IgM gammopathy, two monoclonal IgM gammopathies, two decreased levels of IgA, and one hypogammaglobulinemia. Direct and indirect immunofluorescence procedures were performed on two patients and results were negative. Immunoperoxidase procedures for IgG stained nonspecifically along torn/separated areas of the basement membrane in five patients. No immunohistochemistry was performed on the remaining three patients. The etiology and pathogenesis of these lesions are unknown. We favor the concept that they represent an unusual reaction to an arthropod bite, secondary to the underlying lymphoproliferative disorder. A second, and less likely theory, is that these lesions represent an unusual clinical presentation of bullous pemphigoid in patients with chronic lymphocytic leukemia.- - - - - - - - - - ranking = 1keywords = bullous (Clic here for more details about this article) |
7/10. pyoderma gangrenosum associated with transient acantholytic dermatosis (pemphigus erythematosus-like) and paraproteinemia.A 69-year-old man with recurrent eruptions of pyoderma gangrenosum for 4 years is described. The patient also suffered from paraproteinemia (increased IgA with M-component) and transient acantholytic dermatosis resembling pemphigus erythematosus. He had no intestinal symptoms.- - - - - - - - - - ranking = 13.600481026011keywords = dermatosis (Clic here for more details about this article) |
8/10. Neutrophilic dermatosis: a case of overlapping syndrome with monoclonal antineutrophil cytoplasmic autoantibody activity.A 66-year-old male patient presented, over a 10-year period, polymorphic cutaneous manifestations with extensive neutrophilic infiltration which supports the diagnosis of overlapping syndrome of neutrophilic dermatoses. This was associated with a benign monoclonal gammopathy of IgA lambda type that had antineutrophil cytoplasmic autoantibody (ANCA) activity. Neutrophilic dermatoses may be associated with or can trigger ANCA.- - - - - - - - - - ranking = 10.880384820809keywords = dermatosis (Clic here for more details about this article) |
9/10. Bullous amyloidosis. Report of 3 cases and review of the literature.We report 3 cases of bullous immunoglobulinic amyloidosis and review 25 published cases. In 2 of our patients, amyloid deposits were not detected with special staining, but by means of ultrastructural methods. Investigations of the skin lesions permitted the diagnosis of associated plasma cell dyscrasia in 2 patients. Unexplained bullous lesions should be investigated for amyloid deposits and the presence of monoclonal gammopathy by methods including electron microscopy and immunochemical analysis of serum and urine.- - - - - - - - - - ranking = 0.33333333333333keywords = bullous (Clic here for more details about this article) |
10/10. Subcorneal pustular dermatosis: a clinical study of ten patients.Subcorneal pustular dermatosis of Sneddon and Wilkinson (SPD) is a unique clinical and histologic entity. This disorder has been reported in association with monoclonal gammopathy and IgA pemphigus. We report ten additional cases of SPD and determine the frequency of monoclonal gammopathy and IgA pemphigus in patients with SPD and investigate the relationship of this entity with other dermatoses characterized histologically by a subcorneal pustule. The medical records of patients with SPD evaluated at the authors' institution from 1980 through 1995 were reviewed retrospectively. medical records of twenty patients with pustular psoriasis were reviewed for comparison. Ten patients met the criteria for SPD. Their average age at diagnosis was 66 years. The characteristic flaccid pustules were often generalized and had a tendency to involve the flexural areas. serum monoclonal gammopathy was present in four patients: three with IgA and one with IgG. On direct immunofluorescence examination, three patients had IgA deposits in the intercellular spaces. None of the patients exhibited both monoclonal gammopathy and IgA deposits. Repeated direct immunofluorescence studies were necessary to detect IgA pemphigus in one patient. SPD is a distinct clinical entity. The findings of IgA deposits intercellularly on immunofluorescence and monoclonal gammopathy may help to further identify this entity.- - - - - - - - - - ranking = 2091.1381393447keywords = pustular dermatosis, subcorneal, dermatosis (Clic here for more details about this article) |
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