Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Necrobiotic xanthogranuloma of the skin is associated with paraproteinemia and, often, with plasma proliferative disorders, including multiple myeloma. Other commonly recognized systemic abnormalities include hepatosplenomegaly, a highly increased erythrocyte sedimentation rate, and leukopenia. Fifteen of 16 patients (seven men and nine women with a mean age of 54 years) with this condition had ophthalmic manifestations. Thirteen patients had lesions affecting the skin of the eyelids and periorbital tissue; on casual examination these lesions resembled plane xanthoma. Unlike plane xanthoma, however, the lesions of necrobiotic xanthogranuloma were almost always indurated. Further, the lesions frequently became inflamed, leading to superficial ulceration. Deeper lesions occasionally involved the orbit. Yellow lesions were sometimes visible in the episcleral tissues where they were associated with recurrent symptoms of scleritis and episcleritis. Biopsy specimens of the skin lesions disclosed a distinctive pattern of subepidermal granulomatous masses with focal aggregates of histiocytes and giant cells surrounded by hyaline necrobiosis. Surgical excision of the eyelid lesions was often followed by recurrence and increased activity of the lesions. Low-dose chemotherapy is likely to produce a favorable response, with regard to both the skin lesions and the paraprotein abnormalities.
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PMID:Ophthalmic features of necrobiotic xanthogranuloma with paraproteinemia. 669 27

Herein, the authors report a case of relapsing polychondritis (RP) presenting as isolated bilateral nodular episcleritis. A 23-year-old male presented to us with bilateral large ocular surface masses for which he had received antitubercular medications. A workup was performed to rule out infective, neoplastic, and immune etiologies, after which the patient was then treated empirically with systemic steroids. No response to steroids was noted, so the lesions were removed surgically. On follow up, he developed redness of both ears sparing the lobules. A biopsy from ear lesions supported the diagnosis of RP. At a follow up of 2 years, the patient is free of any ocular or systemic manifestation. To the best of the authors' knowledge, this is the first reported case of RP presenting with bilateral giant nodular episcleritis and treated successfully with surgery. A multidisciplinary approach is essential for the management of such cases. A long-term close follow up is vital for early detection of associated malignancies like multiple myeloma.
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PMID:Bilateral Nodular Episcleritis: A Rare Presentation of Relapsing Polychondritis. 3306 May 14