Q81.0 vs Q87.19

Q81.0 (Epidermolysis bullosa simplex) compared with Q87.19 (Other congenital malformation syndromes predominantly associated with short stature), from the official CMS tabular data. Do not report these codes together: the official tabular list marks them Excludes1.

Relationship

Conflict

Excludes1 conflict

Q81.0

Epidermolysis bullosa simplex

↔

Q87.19

Other congenital malformation syndromes predominantly associated with short stature

Not coded here — these two are mutually exclusive.

“Cockayne's syndrome (Q87.19)”

An Excludes1 note means the two conditions cannot occur together, so the pair should not be reported for the same encounter. The sole exception is when the conditions are documented as unrelated to each other.

Source: CMS ICD-10-CM tabular instructional notes; ICD-10-CM Official Guidelines, Section I.A.12.a

Can these codes be reported together?

No — do not report together. Q81.0 carries an Excludes1 note covering Q87.19: “Cockayne's syndrome (Q87.19)”

Sole exception: when the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

Side by side

	Q81.0Epidermolysis bullosa simplex	Q87.19Other congenital malformation syndromes predominantly associated with short stature
Billing status	Billable	Billable
Classification	Q00-Q99 — Congenital Malformations, Deformations and Chromosomal Abnormalities (Q00-Q99)	Q00-Q99 — Congenital Malformations, Deformations and Chromosomal Abnormalities (Q00-Q99)
Definition	Epidermolysis bullosa simplex is a billable ICD-10-CM diagnosis code (Q81.0).	Other congenital malformation syndromes predominantly associated with short stature is a billable ICD-10-CM diagnosis code (Q87.19).
Includes	—	Aarskog syndrome Cockayne syndrome De Lange syndrome Dubowitz syndrome
Use additional code	—	code(s) to identify all associated manifestations

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Derived from the official CMS ICD-10-CM tabular data (FY2026). Not billing advice; verify sequencing rules for the encounter. All data sources