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IgA nephropathy associated with Castleman disease with cutaneous involvement
Atsushi Komatsuda1, Hideki Wakui, Masaru Togashi
1Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Akita, Japan. komatsud@med.akita-u.ac.jp
The American Journal of the Medical Sciences
|March 26, 2010
Summary
This case study presents a rare instance of Castleman disease (CD) in a Japanese man, involving IgA nephropathy, skin nodules, and systemic lymphadenopathy. Treatment with an anti-interleukin-6 receptor antibody successfully improved his condition.
Area of Science:
- Nephrology
- Hematology
- Immunology
Background:
- Investigates a rare presentation of Castleman disease (CD) in an adult Japanese male.
- Highlights the association of CD with IgA nephropathy, cutaneous nodules, and elevated C-reactive protein.
- Discusses the role of immunosuppressive therapy in the context of these complex symptoms.
Observation:
- A 35-year-old Japanese man presented with systemic lymphadenopathy during immunosuppressive therapy.
- Diagnostic evaluations revealed plasmacytic Castleman disease with specific cutaneous involvement.
- The patient exhibited polyclonal hypergammaglobulinemia and persistently elevated serum C-reactive protein.
Findings:
- Lymph node biopsy confirmed the plasmacytic subtype of Castleman disease.
- Skin biopsy demonstrated characteristic pathological findings of cutaneous CD.
- The patient's IgA nephropathy was associated with multicentric CD and cutaneous manifestations.
Implications:
- This case represents the first documented instance of IgA nephropathy linked to multicentric Castleman disease with cutaneous involvement.
- It underscores the importance of considering Castleman disease in patients with unexplained systemic inflammation, renal complications, and skin lesions.
- Successful treatment with a humanized anti-interleukin-6 receptor antibody suggests a potential therapeutic avenue for similar complex cases.
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