Abstract
A 71-year-old woman presented with exquisitely tender mucosal erosions, a diffuse polymorphous eruption, and night sweats. Workup revealed multiple myeloma with a monoclonal IgG-kappa paraprotein in the serum. Her severe oral involvement was suggestive of paraneoplastic pemphigus, but direct and indirect immunofluorescence tests were negative. A skin biopsy showed spongiosis and a sparse perivascular lymphocytic infiltrate, with occasional CD8-positive lymphocytes in the epidermis. Her lesions improved with intravenous immune globulin. Immunohistochemical staining on the formalin-fixed biopsy specimen was strongly positive for IgG and IgG-kappa in an epidermal "chicken-wire" pattern, but negative for IgG-lambda. Her pulmonary tissue stained negative for IgG-kappa, suggesting clinical relevance of the myeloma paraprotein in her epidermis. To our knowledge, this is the first report of a multiple myeloma patient with such an eruption.
MeSH terms
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Aged
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Diagnosis, Differential
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Epidermis / chemistry
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Epidermis / pathology
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Fatal Outcome
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Female
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Fluorescent Antibody Technique, Indirect
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Humans
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Immunoglobulin kappa-Chains / analysis
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Immunoglobulins, Intravenous / therapeutic use
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Mucositis / diagnosis
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Mucositis / etiology*
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Mucositis / pathology
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Mucositis / therapy
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Multiple Myeloma / complications
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Multiple Myeloma / diagnosis*
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Multiple Myeloma / therapy
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Myeloma Proteins / analysis
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Paraneoplastic Syndromes / diagnosis
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Paraneoplastic Syndromes / etiology*
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Paraneoplastic Syndromes / pathology
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Paraneoplastic Syndromes / therapy
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Pemphigus / diagnosis
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Pruritus / etiology
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Pruritus / pathology
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Pruritus / therapy
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Pulmonary Alveolar Proteinosis / etiology
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Skin Diseases, Papulosquamous / diagnosis
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Skin Diseases, Papulosquamous / etiology*
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Skin Diseases, Papulosquamous / pathology
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Skin Diseases, Papulosquamous / therapy
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Sweating
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Weight Loss
Substances
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Immunoglobulin kappa-Chains
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Immunoglobulins, Intravenous
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Myeloma Proteins