Cutane manifestatie van IgG4-gerelateerde ziekte

IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory condition characterized by tissue infiltration with IgG4 positive plasma cells in the affected organs and, often but not always, elevated serum IgG4- concentrations. The exact pathogenesis of the disease is still not clear. The disease has been described in virtually every organ system. Clinical symptoms depend on which organs are affected. Described skin lesions in IgG4-RD are erythematous papules, plaques and nodules, especially located on the head and neck area. Skin lesions can be the first manifestation of the disease. The histopathological characteristics are the basis for the diagnosis. Elevated serum IgG4 can be supportive in diagnosing IgG4-RD. Imaging studies, with preference for PET/CT scan, play an important role to evaluate the extension and activity of the disease and for follow-up reasons. Systemic glucocoticosteroids are the first-line treatment. The aim of treatment is to reduce irreversible damage to the affected organs caused by chronic inflammation and fibrosis.