Table 3
Proposed ECD classification
Clinical severity and organ system dominance
.
Asymptomatic or minimally symptomatic ECD
Cutaneous-dominant disease
Minimal or asymptomatic bone disease
Symptomatic ECD
CNS dominant
Cardiac dominant
Retroperitoneal dominant
Orbital-craniofacial dominant
Neuroendocrine dominant
Pulmonary dominant
Multisystem
Clinical severity and organ system dominance
.
Asymptomatic or minimally symptomatic ECD
Cutaneous-dominant disease
Minimal or asymptomatic bone disease
Symptomatic ECD
CNS dominant
Cardiac dominant
Retroperitoneal dominant
Orbital-craniofacial dominant
Neuroendocrine dominant
Pulmonary dominant
Multisystem
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