Mikulicz disease and subsequent lupus erythematosus development.

In a 12-year-old boy with clinical and histologic features of Mikulicz disease, systemic lupus erythematosus (SLE) developed two years later. Atrophic plaques and nodules in the skin along with substantial increase in salivary gland enlargement characterized the onset of SLE. There was lack of renal and joint manifestations, and no evidence of keratoconjunctivitis sicca or xerostomia was seen. Histopathologic changes in the skin lesions included mucinous infiltrates, chronic vasculitis, and dermal-epidermal junction findings typical of SLE. Direct immunofluorescene studies of skin lesions and normal-appearing skin showed granular deposition of immunoglobulins and complement at the dermal-epidermal junction zone. Level of anti-DNA antibodies was noticeably elevated, and the serum C3 value was depressed. The patient's response to systemic corticosteroid therapy was dramatic.