What is it about?

An 83-year-old female patient presented to our nephrology outpatient clinic with complaints of weakness, edema, abdominal pain, and constipation, with a preliminary diagnosis of chronic kidney failure related to heart failure. The patient had undergone mitral valve replacement surgery 10 years prior and was diagnosed with chronic renal failure six years prior. Laboratory tests revealed mild normochromic normocytic anemia, consistently high erythrocyte sedimentation rate (ESR) above 100 mm/h, and nephrotic-range proteinuria, prompting suspicion of multiple myeloma. Further investigations, including bone marrow aspiration, confirmed the diagnosis of multiple myeloma. During follow-up, the patient began to complain of difficulty swallowing and symptoms of microstomia. Upon further questioning, it was discovered that these symptoms had been present for more than 10 years. Immunoblot tests revealed positive centromere protein B (CENPB), suggesting a diagnosis of scleroderma. Subsequently, during follow-up, bullous lesions appeared on the patient's chest. Biopsy samples confirmed a diagnosis of bullous pemphigoid (BP). The co-occurrence of scleroderma, multiple myeloma, and superimposed BP represents a rare and noteworthy case for his publication is about how important it is to follow up on a case. This means clinically looking at the case, seeing what's going on, and being able to evaluate it carefully.

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Why is it important?

It is essential to investigate each patient and notice every detail carefully. This case report describes the unusual coexistence of scleroderma, multiple myeloma, and BP in a single patient. The patient's worsening symptoms of dysphagia and microstomia, coupled with positive autoantibody findings, led to the diagnosis of scleroderma. Concurrently, the detection of monoclonal immunoglobulins confirmed the presence of multiple myeloma. The emergence of bullous lesions, followed by histopathological analysis, established the diagnosis of BP. The simultaneous occurrence of these conditions presents a unique clinical scenario, highlighting the necessity of a comprehensive and multidisciplinary approach for accurate diagnosis. This case also illustrates the potential paraneoplastic connection between autoimmune diseases and hematologic malignancies and the importance of monitoring for related comorbidities. Given the rarity of such a combination, this case adds valuable knowledge to the medical literature and may assist in the recognition and management of similar cases in the future fortunately, our case was diagnosed late, and our treatment was not as successful as it should have been. So, it is lifesaving not to be too late to analyze the cases and perform proper treatment.

Perspectives

We think this is very instructive; if you look at the case deeply, you can find fascinating clues and improve patients' outcomes.

Prof Itir Yegenaga
Maltepe University Medical School

Read the Original

This page is a summary of: A Case of Scleroderma With Coexisting Multiple Myeloma and Bullous Pemphigoid, Cureus, August 2024, Springer Science + Business Media,
DOI: 10.7759/cureus.66568.
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