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Mikulicz disease and subsequent lupus erythematosus development
JAMA
|June 6, 1977
Summary
Systemic lupus erythematosus (SLE) developed in a boy with Mikulicz disease, presenting with skin lesions and salivary gland enlargement. Treatment with corticosteroids proved highly effective, indicating a potential link between these conditions.
Area of Science:
- Immunodermatology
- Rheumatology
- Pediatric Autoimmunity
Background:
- Mikulicz disease, characterized by salivary and lacrimal gland enlargement, can present with autoimmune features.
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical manifestations.
Observation:
- A 12-year-old boy initially diagnosed with Mikulicz disease later developed SLE.
- SLE onset manifested as atrophic skin plaques, nodules, and significant salivary gland enlargement.
- Notably absent were renal, joint, and ocular/oral sicca symptoms.
Findings:
- Histopathology revealed mucinous infiltrates, chronic vasculitis, and SLE-typical dermal-epidermal junction changes in skin lesions.
- Direct immunofluorescence showed granular immunoglobulin and complement deposition at the dermal-epidermal junction.
- Elevated anti-DNA antibodies and depressed serum C3 levels were observed.
Implications:
- This case suggests a potential association between Mikulicz disease and the subsequent development of SLE in pediatric patients.
- The distinct presentation highlights the variability of SLE, emphasizing skin and salivary gland involvement.
- Prompt corticosteroid therapy demonstrated significant efficacy, underscoring its role in managing such cases.