Overlap of IgG4-related Disease and Multicentric Castleman's Disease in a Patient with Skin Lesions

Abstract

A 59-year-old man presented with multiple dark red erythemas with induration, anemia, and polyclonal hypergammaglobulinemia. A skin biopsy revealed the infiltration of lymphocytes and plasma cells and he was initially diagnosed with multicentric Castleman's disease (MCD). Glucocorticoid treatment was only partially effective. Four years later, the patient's bilateral lacrimal glands gradually became enlarged and a biopsy revealed dense lymphocyte and plasma cell infiltration with an IgG4+/IgG+ plasma cell ratio of 70%. The patient was diagnosed with IgG4-related disease (RD). Rituximab only had a slight effect. This case demonstrates that overlapping features of IgG4-RD and MCD may present in a single patient, which suggests a shared pathogenesis.

IgG4-related disease presenting with scarring alopecia of the scalp

“… 4 Cutaneous lesions are uncommon, and skin involvement that precedes other lesions as the first sign of disease is especially rare. 5 In this case, a plaque on the scalp associated with progressive scarring alopecia was the initial manifestation of IgG4-RD.…”

“… 4 , 6 , 7 Histopathologically, the dense lymphoplasmacytic infiltrate of IgG4-RD often mimics the infiltrate found in several other inflammatory dermatoses, including Rosai-Dorfman disease, cutaneous multicentric Castleman disease, and B-cell lymphoma. 5 , 8 These conditions also may involve significant increases in IgG4 + cell counts. In our case, negative S100 and CD1a stains excluded Rosai-Dorfman disease and Langerhans cell histiocytosis, respectively.…”

Clinical characteristics of a concurrent condition of IgG4-RD and Castleman’s disease

“…In 2013, Ogoshi et al [12] reported six CD patients achieved IgG4-RD criteria, four responding well to glucocorticoid monotherapy, one patient with mild disease not receiving any treatment, and one having no response to corticosteroids or repetitive worsening of the symptoms during the tapering of corticosteroids, after being added cyclosporine, following 33 months alive. Very recently, Mochizuki et al [21] reported a 59-year-old male who was histopathologically diagnosed as CD from his skin lesion with elevated CRP and IL-6 levels, oral prednisolone (PSL) initiated at 0.5 mg/kg relieving his symptoms, but the laboratory data did not improve. After 4 years, he developed lacrimal and salivary gland swelling; histopathological findings in a dacryoadenectomy specimen showed that IgG4+/IgG+ plasma cell ratio was 70%; finally he was diagnosed as IgG4-RD; and rituximab combined PSL treatment were used.…”

Two cases of generalized pustular psoriasis complicated by IgG4-related disease

“…It is well known that IgG4‐RD causes various skin changes, although less than 2% of all IgG4‐RD cases present skin manifestations . The infiltration of direct IgG4‐positive plasma cells causes cutaneous plasmacytosis, pseudolymphoma, angiolymphoid hyperplasia with eosinophilia, and MD .…”

“…3 It is well known that IgG4-RD causes various skin changes, although less than 2% of all IgG4-RD cases present skin manifestations. 4 The infiltration of direct IgG4-positive plasma cells causes cutaneous plasmacytosis, pseudolymphoma, angiolymphoid hyperplasia with eosinophilia, and MD. 1 IgG4-positive plasma cells or IgG4 can indirectly lead to psoriasis-like eruptions, hypergammaglobulinaemic purpura, unspecified maculopapular or erythematous eruptions, urticarial vasculitis, and ischaemic digit as secondary skin manifestations.…”