Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042109 (urticaria)
 6,569 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Monoclonal gammopathy is a condition characterized by the abnormal proliferation of a single clone of plasma cells, which produces a homogeneous monoclonal protein. It has been reported to occur in association with urticaria in the context of Schnitzler's syndrome and also has been observed to occur in angioedema with acquired C1 esterase inhibitor deficiency. We report 11 cases of monoclonal gammopathy presenting to practicing allergists (>2.5% of those screened) primarily in association with dermatologic disorders, i.e., urticaria, angioedema, and nonspecific dermatitis, but also with allergic respiratory disorders, i.e., allergic rhinitis, chronic sinusitis, and asthma. Most of the patients with dermatologic manifestations had respiratory disorders as well, three with chronic sinusitis. To our knowledge, these are the only such cases reported in patients with urticaria or angioedema in the absence of Schnitzler's syndrome or C1 inhibitor deficiency or in association with chronic sinusitis, allergic rhinitis, or asthma. Monoclonal gammopathy, angioedema, urticaria, allergic respiratory disorders, and sinusitis could be linked through antigenic stimulation as a trigger, either infectious, as in chronic sinusitis; self-antigens, as in autoimmunity; or the monoclonal gammopathy itself, causing idiotype-anti-idiotype immune complexes and inflammatory disease. The allergist, dermatologist, otolaryngologist, and primary care physician should all maintain a high index of suspicion for the occurrence of monoclonal gammopathy in the "allergic" population. Serum protein electrophoresis and/or serum immunofixation are useful screening tools. When monoclonal gammopathy is found, the presence of light chains in the urine should be assessed and the patient should be referred for prompt hematology-oncology evaluation with periodic monitoring for the development of plasma cell dyscrasias. Additional prospective study is necessary to determine the true prevalence of monoclonal gammopathy in the population presenting to the practicing allergist.
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PMID:Monoclonal gammopathy in association with allergic disorders of the skin and respiratory tract. 1672 32

A 70-year-old-male had suffered from non-pruritic, erythematous eruptions on the trunk for 3 months without any general symptoms. The individual lesions lasted for several days. Laboratory investigation showed marked elevation of serum immunoglobulin A (2235 mg/dL) with monoclonal gammopathy (IgA k-type). Monoclonal gammopathy of undetermined significance was diagnosed. Histopathological examination of the eruption revealed diffuse neutrophilic infiltration with leukocytoclasia in the dermis. There was no vasculitis. Treatment with antihistamines alone was not effective. Diaphenyl sulfone (DDS) at 75 mg/day dramatically improved the skin lesions. A similar case of urticarial erythema associated with IgA myeloma has been previously reported. We suggest that neutrophilic, urticaria-like erythema associated with IgA monoclonal gammopathy may be regarded as a new entity.
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PMID:Neutrophilic, urticaria-like erythema associated with immunoglobulin A monoclonal gammopathy of undetermined significance. 1847 30

Systemic manifestations of monoclonal gammopathies (MG) are rare but extremely varied. This general review focuses on the hyperviscosity syndrome, neurological disorders, skin changes, the POEMS syndrome, and biological manifestations, with the exception of amyloidosis AL and cryoglobulinemia. The hyperviscosity syndrome usually involves a combination of general, hemorrhagic, ocular and central neurological disorders. The principal neurological manifestations are peripheral neuropathies, mainly due to IgM with anti-MAG activity. Skin disorders include overload dermatoses (xanthomatosis, mucinosis), neutrophilic dermatosis, urticaria, edema and the AESOP syndrome. The POEMS syndrome classically consists of polyneuropathy, organomegaly, endocrinopathy, monoclonal plasmocyte proliferation, and cutaneous manifestations. MG interference with assay methods can lead to false hyponatremia, hypoglycemia, hyperbilirubinemia, hypercalcemia and hypertransferrinemia. These systemic manifestations can reveal classical MG-related disorders such as monoclonal gammopathy of undetermined significance (MGUS), solitary plasmocytoma, multiple myeloma, and Waldenstrom's disease. They are due either to the chemicophysical properties of the monoclonal immunoglobulin, or to its antibody activity (especially against autoantigens), with potential therapeutic implications.
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PMID:Systemic manifestations of monoclonal gammopathy. 1971 43