Published online Oct 26, 2020. doi: 10.12998/wjcc.v8.i20.4922
Peer-review started: May 18, 2020
First decision: June 7, 2020
Revised: June 24, 2020
Accepted: September 5, 2020
Article in press: September 5, 2020
Published online: October 26, 2020
Human herpes virus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder driven by proinflammatory cytokines, which is still poorly understood. Pulmonary parenchyma lesion is a rare condition in iMCD, which mainly manifests as lymphocytic interstitial pneumonia and is an indicator of severe iMCD. Cutaneous lesion is also very rare and mainly occurs in Asians. There have been few reports of iMCD patients with both skin and lung parenchyma involvement.
We present a Chinese man who complained about a 3-year history of intermittent dry cough and a 2-year history of diffuse reddish-brown maculopapules. Laboratory examination revealed polyclonal hypergammaglobulinemia and hypercytokinemia including interleukin 6. Chest computed tomography revealed small patchy shadows with ground-glass nodules scattered in two lobes and mediastinal lymphadenopathy. The pathological result of the lymph node was consistent with the plasma cell type of Castleman disease. As serum human immunodeficiency virus test and HHV-8 staining of the lymph node were negative, the patient was finally diagnosed with HHV-8 negative iMCD. He was treated with tocilizumab at an intravenous (i.v.) dose of 8 mg/kg every 2 wk combined with methylprednisolone at an i.v. dose of 80 mg/d initially with gradual dose tapering. Partial remission was achieved 9 mo later.
iMCD with lung parenchyma and skin involvement is a rare condition that requires clinicians’ attention and awareness for early diagnosis.
Core Tip: We report a case of idiopathic multicentric Castleman disease (iMCD) with pulmonary parenchyma and skin involvement as the primary clinical manifestations. The patient had presented to several tertiary hospitals for more than 3 years before he was finally diagnosed. iMCD is a rare systemic lymphoproliferative disease, and delays in diagnosis are common and remain a concern. Clinicians need to improve their understanding of iMCD. Combination therapy with anti-interleukin 6 (IL-6) monoclonal antibodies and glucocorticoids is an effective frontline treatment regimen, especially for those with an elevated level of serological IL-6.