- Usually young adult onset, usually normolipemic (lesions look xanthomatous)
- Many yellow, red or brown papules on face (especially eyelids), flexural, intertriginous, progress to disfiguring plaques
- Self-healing, persistent, or progressive
- Mucosal lesions in 40%, oral/airway > eye
- CNS involvement in 40%, mild diabetes insipidus, sometimes gammopathies
- Pathology
- Similar to JXG, “scalloped” macrophages, hemosiderin
- Organ-specific morbidity/mortality
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