Cutaneous Disorders Associated with Castleman’s Disease

Abstract

This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Acta Derm Venereol 2019; 99: 984-989 984 SIGNIFICANCECastleman's disease is a rare lymphoproliferative disorder, which is often accompanied by various cutaneous manifestations. However, Castleman's disease-related cutaneous manifestations have not yet been systematically reviewed. The aim of this study was to examine the clinical, histopathological and laboratory characteristics of cutaneous manifestations in Castleman's disease, through a retrospective review of medical records. The study aimed to provide a comprehensive understanding of Castleman's disease-associated cutaneous disorders by subclassifying cutaneous disorders according to their underlying pathomechanisms. The study highlights the role of dermatologists in an in-depth review of various skin disorders related to Castleman's disease.Castleman's disease is a rare disease of the lymph nodes and related tissues, presenting as angiofollicular or giant lymph node hyperplasia. Although various skin manifestations have been reported to occur in Castleman's disease, a comprehensive study of cutaneous disorders in Castleman's disease is lacking. Therefore, the aim of this study was to investigate Castleman's disease-associated cutaneous disorders. The medical records of 57 patients with Castleman's disease who visited our hospitals from January 2007 to May 2018 were analysed retrospectively. Patients were classified according to the presence of skin involvement. Plasma variant-type Castleman's disease and multicentric Castleman's disease were more commonly found in patients with Castleman's disease with a cutaneous disorder than in those without a cutaneous disorder. In addition, the skin disorders were classified according to pathomechanisms: immune complexrelated (paraneoplastic pemphigus, xanthogranulomas), cytokine-related (vasculitis-like lesion, cherry angioma, hyperpigmentation), and non-specific (pruritus). This study builds on previous case reports of cutaneous disorders in Castleman's disease and proposes a new classification system.

Cutaneous involvement as initial presentation of multicentric plasmacytic Castleman disease

“…The most common features at presentation are constitutional symptoms, fluid accumulation, cytopenias, and liver or kidney dysfunction 7‐10 . Skin abnormalities including rash, xanthogranulomas, hyperpigmentation, cherry hemangiomatosis, paraneoplastic pemphigus, and Kaposi sarcoma, though rare, have also been reported 11,12 . However, MCD with disseminated dark brown papules, patches, and plaques as initial presentation has rarely been reported.…”

Castleman disease. Interaction with dermatopathy: Case report

“…As an inference, it can be assumed that the erysipelas triggered the CD; however, there are paraneoplastic lesions that manifest as a result of UCD and present skin thickening, angiomas and telangiectasias [ 8 ]. Particular mention should be made of paraneoplastic pemphigus [ 6 ], which begins with lesions in the oral mucosa [ 8 ]. All these lesions regress with complete resection of the enlarged lymph node, which constitutes effective treatment of the disease [ 8 , 14 ].…”

“…UCD is typically asymptomatic, but there are cases in which the increased lymph node volume compresses neighboring structures, causing compressive symptoms [ 4 , 5 ]. Laboratory tests are frequently unaltered; therefore, the disease is diagnosed by lymph node biopsy [ 6 , 7 ].…”

“…Cutaneous manifestations, including paraneoplastic pemphigus, may occur in 55% of cases [ 6 ]. UCD requires surgical treatment for the removal of affected lymph nodes; surgery offers good results and the remission of symptoms [ 8 , 4 ].…”

Cryoglobulinemic vasculitis associated with multicentric Castleman disease

“…The cutaneous manifestations of MCD include paraneoplastic pemphigus, hemangioma, and Kaposi sarcoma. 2 We report a case of cryoglobulinemic vasculitis (CV) associated with idiopathic MCD.…”