Excludes1 pair

C96.0 vs D76-

C96.0 and D76 are not coded together. ICD-10-CM declares them mutually exclusive with an Excludes1 note — the two conditions are not expected to occur together, so exactly one of them is normally correct for a given encounter. The Official Guidelines carve out one exception: if the two conditions are clearly unrelated to each other, both may be reported.

The official note, declared on D76
(Abt-) Letterer-Siwe disease (C96.0)
C96.0

Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis

Billable
Chapter 2
Neoplasms
Section
C81-C96
Use when the record says
  • Histiocytosis X, multisystemic
  • Letterer-Siwe disease
Indexed under
  • Letterer-Siwe's disease
  • Histiocytosis › acute differentiated progressive
  • Reticuloendotheliosis › acute infantile
  • Reticuloendotheliosis › nonlipid
  • Reticulosis › acute of infancy
D76

Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue

Not billable
Chapter 3
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
Section
D70-D77

These two codes sit in different chapters of the Tabular List, which is usually the clearest signal that they describe different underlying processes rather than different degrees of the same one.

Excludes1 is not always absolute: where two conditions covered by the note are genuinely unrelated, CMS guidance permits reporting both. Document the reasoning rather than assuming it. More on Excludes1 vs Excludes2 · FY2027