IgG4-Related Sialoadenitis with a Skin Lesion and Multiple Mononeuropathies Suggesting Coexistent Cryoglobulinemic Vasculitis

IgG4-related skin lesions in a patient with IgG4-related chronic sclerosing dacryoadenitis and sialoadenitis.

We describe a 60-year-old man with IgG4-related chronic sclerosing dacryoadenitis and sialoadenitis associated with lymphoplasmacytic and eosinophilic infiltration in erythematous nodules. Physical examination revealed left eye extrusion and small itchy nodules on the scalp and neck. The serum IgG level was 1,570 mg/dL, IgG4 463 mg/dL (29.5%), and IgE 4,554 IU/mL. Lacrimal gland biopsy disclose...

A case of marginal zone B cell lymphoma mimicking IgG4-related dacryoadenitis and sialoadenitis

BACKGROUND IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS), so-called Mikulicz's disease, is characterized by elevated serum IgG4 and infiltration of IgG4-positive plasma cells in glandular tissues. Recently, several studies reported both malignant lymphoma developed on the background of IgG4-associated conditions and IgG4-producing malignant lymphoma (non-IgG4-related disease). CASE ...

Managing refractory cryoglobulinemic vasculitis: challenges and solutions

Cryoglobulinemia is thought to be a rare condition. It may be an isolated disorder or secondary to a particular disease. According to immunoglobulin composition, cryoglobulinemia is classified into three types. In mixed cryoglobulinemia (types II and III), vascular deposition of cryoglobulin-containing immune complexes and complement may induce a clinical syndrome, characterized by systemic vas...

Therapy for hepatitis C virus-related cryoglobulinemic vasculitis.

Copyright © 2013 Massachusetts Medical Society. Cryoglobulins are immunoglobulins characterized by their insolubility at low temperatures and their dissolution after rewarming.1,2 On the basis of their immunochemical composition, cryoglobulins are classified as either single (type I), consisting of a monoclonal immunoglobulin, or mixed, comprising two or more immunoglobulin isotypes, with (type...

Pregnancy in patient with cirrhosis and cryoglobulinemic vasculitis.