ICD-10 Code D76.3 – Other histiocytosis syndromes (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code D76.3 – Other histiocytosis syndromes
What it is
D76.3 identifies other histiocytosis syndromes, a group of uncommon disorders involving abnormal accumulation of histiocytes in tissues. Use it when the record names this category and no more specific histiocytic disorder is documented.
Clinical signs
Clinical features vary; refer to documentation. Findings may include tissue masses, skin lesions, lymph node enlargement, or organ involvement, depending on the specific histiocytosis syndrome described.
When to use this code
Use D76.3 when the clinician documents “other histiocytosis syndromes” or a comparable nonspecific histiocytic disorder that does not map to a more precise code. It is appropriate when pathology, imaging, or specialist notes support the diagnosis but do not identify a named subtype. Check documentation if the condition is described only as histiocytosis.
Do not use for
Do not use this code for a specific histiocytic disorder that has its own ICD-10-CM code. Avoid it when the record describes only a suspected condition or a different disease process. Check documentation.
Coding tip
Code the most specific histiocytosis documented; if the subtype is unclear, use D76.3 only when the provider explicitly supports the diagnosis.