Mikulicz disease and subsequent lupus erythematosus development
R. W. Romero, L. T. Nesbitt Jr and H. Ichinose
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.
