Cases reported "Paraneoplastic Syndromes"

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1/6. Paraneoplastic skin manifestations of lung cancer.

    Two cases are reported illustrating a parallel course of extensive skin manifestations and lung cancer. The cases presented features of paraneoplastic acrokeratosis (Bazex's syndrome) and erythema gyratum repens though they did not completely correspond to these well-defined conditions. In both cases the cutaneous eruption appeared more than a year prior to the diagnosis of lung cancer, and the skin disease resolved completely within half a year following surgical removal of the cancer. The need is stressed for repeated cancer screens when a cutaneous marker of internal malignancy is suspected.
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2/6. Successful treatment of paraneoplastic pemphigus in follicular NHL with rituximab: report of a case and review of treatment for paraneoplastic pemphigus in NHL and CLL.

    Paraneoplastic pemphigus is a severe mucocutaneous disease associated in most cases with B-cell lymphoproliferative disorders. Independent of the course of the underlying malignancy, this autoimmune skin disease is resistant to any treatment in most cases and may lead to death by infectious complications. We observed a patient with recovery of paraneoplastic pemphigus associated with follicular non-Hodgkin's-lymphoma after therapy with rituximab. In addition, we reviewed the literature and analyzed the few cases with comparable outcome with other treatment modalities.
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3/6. A localized variant of paraneoplastic pemphigus: acantholysis associated with malignant melanoma.

    We report a 72-year-old male patient with a nodular malignant melanoma that was associated with focal suprabasal acantholysis (FSA). This phenomenon, which is regarded as an incidental finding by dermatopathologists, may be associated with inflammatory and also neoplastic skin diseases. Haematoxylin and eosin stained sections from an erythematous plaque surrounding the patient's tumour showed FSA, direct immunofluorescence (DIF) and indirect immunofluorescence (IIF) on normal human skin, monkey oesophagus and rat urinary bladder were negative. On electron microscopy few desmosomes could be detected in the basal cell layer of the acantholytic areas and there was a nearly complete loss of these structures in the spinous cell layer. Only remnants of cytoplasmic plaques and keratin filaments could be observed in those areas. In contrast, adherens junctions appeared to be well preserved. An enzyme-linked immunosorbent assay (ELISA) using recombinant fusion proteins as antigens did not show circulating autoantibodies against desmoglein 1 (Dsg1) or desmoglein 3 (Dsg3). In contrast, immunoblotting revealed autoantibodies directed against keratinocyte antigens with a molecular weight of 85 kDa and 250 kDa, the first band corresponding to the molecular weight of comigrating plakoglobin. immunoprecipitation with patient serum also revealed a 85-kDa band. We conclude that these autoantibodies, probably in conjunction with cofactors produced by the tumour, could play a part in the pathogenesis of this variant of FSA, for which we propose the term 'localized paraneoplastic pemphigus.'
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4/6. Paraneoplastic acrokeratosis: Bazex syndrome.

    Paraneoplastic acrokeratosis, or Bazex syndrome, is a rare, distinct dermatosis characterized by psoriasiform acral hyperkeratosis. In most cases, it is a specific cutaneous sign of an occult squamous cell carcinoma of the upper aerodigestive tract that has metastasized to cervical lymph nodes. We report the fifth American case of paraneoplastic acrokeratosis. The patient's skin disease was more remarkable for its hyperpigmentation than its hyperkeratosis. Nearly all of the hyperpigmentation resolved, but the nail dystrophy persisted seven months after the tumor had been treated using surgery and radiation.
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keywords = skin disease
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5/6. A paraneoplastic mixed bullous skin disease associated with anti-skin antibodies and a B-cell lymphoma.

    BACKGROUND--The full spectrum of bullous diseases associated with underlying cancers remains to be fully defined. observation--We describe a patient with a mixed bullous disease exhibiting combined features of cicatricial pemphigoid and pemphigus and associated with a B-cell lymphoma producing an IgM paraprotein to intercellular antigens in human skin. The patient had the clinical features of cicatricial pemphigoid and the histologic and immunofluorescence abnormalities of both cicatricial pemphigoid and pemphigus. These included oral and cutaneous erosions; ocular scarring; subbasal and acantholytic intraepidermal bullae; and circulating and tissue-fixed basement membrane zone and intercellular antibodies. The antibodies were directed to a 140-kd antigen in dermal extracts of skin split with 1 mol/L of sodium chloride and to antigens with approximate molecular weights of 150, 180, 230, and 285 kd in the dermal extract. In contrast to paraneoplastic pemphigus, the intercellular antibodies did not react to mammalian bladder. The intercellular antibodies were of the IgM class and were associated with the paraprotein produced by the malignant B cells. CONCLUSIONS--We believe that this condition represents a novel bullous disease, which we refer to as paraneoplastic mixed bullous disease. This condition illustrates that distinct bullous diseases are associated with paraneoplastic syndromes and that at least one possible mechanism for such eruptions is the production of anti-skin antibodies by malignant B cells.
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keywords = skin disease
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6/6. Paraneoplastic pemphigus: the first case report from japan.

    Paraneoplastic pemphigus is a rare skin disease characterized by painful mucosal ulcerations and polymorphous skin lesions in association with an underlying neoplasm. A 54-year-old Japanese man, who had received chemotherapy and radiation therapy due to a diagnosis of non-Hodgkin's lymphoma, developed marked stomatitis, conjunctivitis and blisters. Histologic examination showed suprabasal cleft formation with acantholysis and keratinocyte necrosis. Direct immunofluorescence revealed the deposition of IgG at the cell surface of the keratinocytes and C3 at the basement membrane zone. Indirect immunofluorescence on normal human skin and rat bladder sections revealed circulating autoantibodies to the cell surface of both keratinocytes and transitional epithelia. immunoprecipitation disclosed antibodies reactive to the 250-kD, 230-kD, 210-kD and 170-kD proteins. From these results, a diagnosis of paraneoplastic pemphigus was made. This is the first report of paraneoplastic pemphigus from japan.
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