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- Presentation
Skinternal Medicine: A Case Based Challenge
Description
In a case presentation by Dr. Courtney Schott from the University of Louisville, a 75-year-old woman with a two-year history of skin plaques was discussed. Initially misdiagnosed as dermatofibromas, her lesions were biopsied, revealing a dermal infiltrate with multinucleate giant cells, suggesting granulomatous conditions. Diagnostic considerations included necrobiotic xanthogranuloma (NXG) and granuloma annulare, with the final diagnosis being NXG associated with smoldering multiple myeloma. Initial treatments involved intralesional triamcinolone, which showed minimal efficacy. Further investigation revealed a monoclonal IgG lambda light chain protein, and a subsequent bone marrow biopsy indicated a low level of atypical plasma cells. As her skin lesions progressed and her condition deteriorated, a multidisciplinary approach was taken, leading to the recommendation for treatment of the smoldering myeloma. The discussion emphasized the importance of recognizing the relationship between skin manifestations and underlying hematologic disorders, as well as the potential timing of associated conditions. Treatment options for NXG were explored, highlighting the varied responses to intravenous immunoglobulin, corticosteroids, and other agents. Ultimately, the patient was encouraged to initiate treatment for her smoldering myeloma, indicating a holistic approach to her complex condition.
View moreConclusions
- The patient was diagnosed with necrobiotic xanthogranuloma (NXG) in conjunction with smoldering multiple myeloma.
- NXG is a type of non-Langerhans histiocytosis characterized by reddish-brown or yellowish cutaneous lesions that can ulcerate and often present at multiple sites.
- A significant proportion of NXG patients (around 82%) have a monoclonal gammopathy, mostly IgG kappa, and about 25% are associated with malignancies, primarily multiple myeloma.
- Timing of hematologic disorders such as multiple myeloma can vary; they may precede, coincide with, or follow the development of NXG.
- First-line treatments for NXG include immunoglobulin therapy (IVIG), corticosteroids, and addressing underlying malignancies due to the lack of randomized controlled trials.
- Patients with multiple myeloma who also exhibit symptoms of NXG may benefit from a combination of myeloma treatment and dermatology consultation for their skin lesions.
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