Idiopathic multicentric Castleman disease preceded by cutaneous plasmacytosis successfully treated by tocilizumab

Abstract

A woman aged 45 years with a 1.5-year history of violaceous plaques on the forehead and chest presented with fever, weight loss and aggravation of the plaques. Inflammatory markers and interleukin-6 level were elevated, and superficial lymphadenopathies and splenomegaly were identified by CT scan. Immunohistochemical findings of the lymph node and the skin showed polyclonal plasmacytosis and follicular hyperplasia, leading to the diagnosis of idiopathic multicentric Castleman disease (iMCD) after human herpesvirus-8 infection was excluded. The patient was successfully treated with anti-interleukin-6 receptor antibody, tocilizumab, following relapse after prednisolone therapy.Our literature review found 11 case reports of pathologically confirmed iMCD preceded by cutaneous plasmacytosis. The median duration of asymptomatic phase with only skin lesions was 7.5 years, whereas the phase lasted only for 1.5 years in our case. iMCD can develop shortly after asymptomatic cutaneous plasmacytosis. Tocilizumab can be a treatment of choice for this type of iMCD.

Cutaneous plasmacytosis: A rare initial presentation of idiopathic multicentric Castleman's disease

“…Cutaneous plasmacytosis is characterized by the dermal infiltration of polyclonal mature plasmacytes, and it predominantly affects Asian descent 1 . Although most cutaneous plasmacytosis is indolent, the disease could develop plasma cell type of iMCD 2 . If a patient is diagnosed with cutaneous plasmacytosis, clinicians should carefully follow up with the patient keeping in mind the potential for the development of iMCD.…”

“…1 Although most cutaneous plasmacytosis is indolent, the disease could develop plasma cell type of iMCD. 2 If a patient is diagnosed with cutaneous plasmacytosis,…”

Skin‐associated lymphoid tissue could be a sign of systemic disease

“…4 Although corticosteroid treatment could provide symptomatic relief, its tapering always leads to recurrence of the skin lesions, which happened in the patient reported by Kamido et al, while a recent study suggested the effectiveness of tocilizumab by targeting IL-6. 3…”

“…It could precede the development of other systemic features of idiopathic multicentric Castleman disease. 3 Histologically, it may also be reported as 'cutaneous plasmacytosis', which is thought to share a similar pathogenesis with Castleman disease in terms of hypercytokinaemia, in particular interleukin (IL)-6. 4 Although corticosteroid treatment could provide symptomatic relief, its tapering always leads to recurrence of the skin lesions, which happened in the patient reported by Kamido et al, while a recent study suggested the effectiveness of tocilizumab by targeting IL-6.…”

“…2 Castleman disease is classified into several subtypes depending on the clinical findings, and any subtype manifests lymphadenopathy, which was not detected by ultrasound and positron emission tomography/computed tomography in our patient. 3 Although Castleman disease usually exhibits IgG gammopathy, a subtype of POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome-associated multicentric Castleman disease is reported to display IgM monogammopathy,…”