ICD-10-CM 2027
ICD-10 code for Herlitz' syndrome
From the official ICD-10-CM alphabetic index entry “Herlitz' syndrome”. Page updated September 29, 2026.
About coding Herlitz' syndrome
The ICD-10-CM code for Herlitz' syndrome is Q81.1 (Epidermolysis bullosa letalis).
Within Q81 (epidermolysis bullosa), Q81.1 is specifically for epidermolysis bullosa letalis. Related codes cover simplex (Q81.0), dystrophica (Q81.2), other epidermolysis bullosa (Q81.8) and unspecified (Q81.9).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 595 (Major Skin Disorders with MCC, relative weight 2.0239) and DRG 596 (Major Skin Disorders without MCC, relative weight 1.1071), in MDC 09 (Diseases and Disorders of the Skin, Subcutaneous Tissue and Breast), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about Herlitz' syndrome ICD-10 codes
What is the ICD-10 code for Herlitz' syndrome?
- Q81.1 — Epidermolysis bullosa letalis.
Is Q81.1 billable?
- Yes. Q81.1 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can Q81.1 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict Q81.1 as a principal diagnosis.
Is Q81.1 a CC or MCC?
- No. Q81.1 is neither a CC nor an MCC under MS-DRG v44.0.