Q82.1 Xeroderma pigmentosum

Billable ICD-10-CM code, FY2026. Exempt from present-on-admission (POA) reporting.

Applies from Q82 Other congenital malformations of skin

Excludes1 (never code together)

• acrodermatitis enteropathica (E83.2)
• congenital erythropoietic porphyria (E80.0)
• pilonidal cyst or sinus (L05.-)
• Sturge-Weber (-Dimitri) syndrome (Q85.89)

MS-DRG index

Defines the logic of DRG(s) 606-607 in MDC 09.

Approximate ICD-9-CM (GEMs)

• 757.33 Congenital pigmentary anomalies of skin (approximate)

Code annotations containing back-references to Q82.1

Back-references to Q82.1

• Type 1 Excludes: E79 Disorders of purine and pyrimidine metabolism ("xeroderma pigmentosum (Q82.1)")
• Type 2 Excludes: G11.3 Cerebellar ataxia with defective DNA repair ("xeroderma pigmentosum (Q82.1)")

Back-references to Q82

• Type 1 Excludes: P83 Other conditions of integument specific to newborn ("congenital malformations of skin and integument (Q80-Q84)")

Diagnosis Index entries for Q82.1

• Atrophoderma, atrophodermia (of) › pigmentosum
• Epitheliomatosis pigmented
• Kaposi's › dermatosis (xeroderma pigmentosum)
• Melanosis › lenticularis progressiva
• Xeroderma › pigmentosum

Nearby codes

• Q82 Other congenital malformations of skin
• Q82.0 Hereditary lymphedema
• Q82.1 Xeroderma pigmentosum (this code)
• Q82.2 Congenital cutaneous mastocytosis
• Q82.3 Incontinentia pigmenti
• Q82.4 Ectodermal dysplasia (anhidrotic)
• Q82.5 Congenital non-neoplastic nevus
• Q82.6 Congenital sacral dimple