CR(E)ST syndrome

This code represents the CREST syndrome, a limited form of systemic sclerosis (scleroderma) characterized by Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasias. It is a chronic autoimmune connective tissue disease primarily affecting the skin and internal organs.

Use this code when documentation clearly indicates a diagnosis of CREST syndrome, either explicitly stated or when all five characteristic components are present and attributed to this specific syndrome. This code is appropriate for both initial diagnoses and subsequent encounters for managing the condition.

• Ensure documentation specifies "CREST syndrome" or explicitly lists all five components (Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasias) within the context of limited systemic sclerosis.
• Do not confuse with generalized systemic sclerosis (M34.0), which involves more widespread skin and organ involvement.
• Code associated manifestations (e.g., specific esophageal disorders, pulmonary hypertension) separately as secondary diagnoses.