Q82.2 Congenital cutaneous mastocytosis

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

Applicable to

• Congenital diffuse cutaneous mastocytosis
• Congenital maculopapular cutaneous mastocytosis
• Congenital urticaria pigmentosa

Excludes1 (never code together)

• cutaneous mastocytosis NOS (D47.01)
• diffuse cutaneous mastocytosis (with onset after newborn period) (D47.01)
• malignant mastocytosis (C96.2-)
• systemic mastocytosis (D47.02)
• urticaria pigmentosa (non-congenital) (with onset after newborn period) (D47.01)

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 Q82.2

Back-references to Q82.2

• Type 1 Excludes: C96.2 Malignant mast cell neoplasm ("mastocytosis (congenital) (cutaneous) (Q82.2)")
• Type 1 Excludes: D47.0 Mast cell neoplasms of uncertain behavior ("congenital cutaneous mastocytosis (Q82.2)")
• Type 1 Excludes: D47.01 Cutaneous mastocytosis ("congenital (diffuse) (maculopapular) cutaneous mastocytosis (Q82.2)")
• Type 1 Excludes: D47.01 Cutaneous mastocytosis ("congenital urticaria pigmentosa (Q82.2)")
• Type 1 Excludes: D89.4 Mast cell activation syndrome and related disorders ("congenital cutaneous mastocytosis (Q82.2)")

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 Q82.2

• Mastocytosis › cutaneous (diffuse) (maculopapular) › congenital
• Mastocytosis › cutaneous (diffuse) (maculopapular) › of neonatal onset
• Mastocytosis › cutaneous (diffuse) (maculopapular) › of newborn onset
• Urticaria › pigmentosa › congenital
• Urticaria › pigmentosa › of neonatal onset
• Urticaria › pigmentosa › of newborn onset

Nearby codes

• Q82 Other congenital malformations of skin
• Q82.0 Hereditary lymphedema
• Q82.1 Xeroderma pigmentosum
• Q82.2 Congenital cutaneous mastocytosis (this code)
• Q82.3 Incontinentia pigmenti
• Q82.4 Ectodermal dysplasia (anhidrotic)
• Q82.5 Congenital non-neoplastic nevus
• Q82.6 Congenital sacral dimple
• Q82.8 Other specified congenital malformations of skin