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Images in Dermatology
January 8, 2020

Mikulicz Disease

Author Affiliations
  • 1Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
JAMA Dermatol. 2020;156(4):451. doi:10.1001/jamadermatol.2019.4260

A man in his 60s had a 12-year history of bilateral palpebral swelling. He also had dryness of the eyes and mouth. A physical examination showed bilateral swelling of the upper and lower eyelids (Figure). Spindle-shaped subcutaneous masses were found by palpation of the eyelids. Results of laboratory tests showed high levels of serum IgG4 (1240 mg/dL; to convert to grams per liter, multiply by 0.01) and IgE (401 μg/L; to convert to milligrams per liter, multiply by 0.001). Serum levels of high-sensitivity C-reactive protein (10.08 mg/L; to convert to nanomoles per liter, multiply by 9.524) and erythrocyte sedimentation rate (30 mm/h) were both elevated. Test results were negative for serum antinuclear antibodies and extractable nuclear antigens. Magnetic resonance imaging revealed several hypoechoic lymph nodes in the right parotid gland and submandibular region and diffuse lesions involving the bilateral orbits, eyelids, lacrimal glands, and subcutaneous tissues of the temporal regions. Cutaneous biopsy results from the right upper eyelid were nondiagnostic. Histopathologic examination of the left lacrimal gland revealed a massive infiltration of lymphocytes and plasma cells. Immunostaining showed infiltration of IgG4-positive cells. A diagnosis of Mikulicz disease was made. The patient was treated with 30 mg/d of oral prednisolone, which was tapered for 2 months. The patient had partial remission of the palpebral swelling at last follow-up.

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