Mikulicz's disease: a new perspective and literature review.

PURPOSE: To report the clinical and pathophysiologic features of two patients with Mikulicz's disease and to further characterize recommendations for diagnosis and management with a review of the literature.

METHODS: Retrospective nonrandomized consecutive case series, Jules Stein Eye Institute, David Geffen School of Medicine at UCLA.

RESULTS: Mikulicz's disease is characterized by symmetric lacrimal, parotid, and submandibular gland enlargement with associated lymphocytic infiltrations. The authors noted two cases of Mikulicz's disease. The diagnosis of Mikulicz's disease was based on the following criteria: 1) symmetric and persistent swelling of the lacrimal glands and either or both of the major salivary glands (parotid and submandibular); and 2) the exclusion of other diseases that may mimic this presentation, such as sarcoidosis, viral infection, or lymphoproliferative disorders.

CONCLUSIONS: Mikulicz's disease is a condition in which there is bilateral lacrimal and salivary gland swelling that is not associated with other systemic conditions. The condition is self-limiting and most often, the diagnosis is a clinical one. Previously, Mikulicz's disease was often considered as a subtype of Sjögren's syndrome (SS). Clinical and immunologic differences between Mikulicz's disease and SS may warrant further consideration of Mikulicz's disease as a specific autoimmune phenomenon separate from SS, and Mikulicz's disease may be amenable to different treatment modalities than those employed in patients with SS.