M04.8
BillableOther autoinflammatory syndromes
Other autoinflammatory syndromes
Coding Notes
Inclusion Terms
Alternative clinical terms for this condition
- Blau syndrome
- Deficiency of interleukin 1 receptor antagonist [DIRA]
- Majeed syndrome
- Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome [PFAPA]
- Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome [PAPA]
Excludes 2
Conditions not included here, but the patient may have both
- arthropathic psoriasis (L40.5-)
- certain conditions originating in the perinatal period (P04-P96)
- certain infectious and parasitic diseases (A00-B99)
- compartment syndrome (traumatic) (T79.A-)
- complications of pregnancy, childbirth and the puerperium (O00-O9A)
- congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
- endocrine, nutritional and metabolic diseases (E00-E88)
- injury, poisoning and certain other consequences of external causes (S00-T88)
- neoplasms (C00-D49)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
- Crohn's disease (K50.-)
Also Known As / Clinical Terms
SNOMED CT
- Acquired pancytopenia5876000
- Pancytopenia - acquired5876000
- CDA - congenital dyserythropoietic anaemia52951008
- CDA - congenital dyserythropoietic anemia52951008
- Congenital dyserythropoietic anaemia52951008
- Congenital dyserythropoietic anemia52951008
- Vesicular eruption79893008
- Vesicular rash79893008
- Neonatal purpura82835005
- Neonatal thrombocytopenia82835005
- Purpura of newborn82835005
- Neonatal anaemia234350007
- Neonatal anemia234350007
- Haemophagocytic lymphohistiocytosis234437005
- Haemophagocytic syndrome234437005
- Hemophagocytic lymphohistiocytosis234437005
- Hemophagocytic syndrome234437005
- Hyperinflammatory lymphohistiocytosis234437005
- Acute generalised pustular psoriasis238612002
- Acute generalized pustular psoriasis238612002
- Generalised pustular psoriasis238612002
- Generalised pustular psoriasis of von Zumbusch238612002
- Generalized pustular psoriasis238612002
- Generalized pustular psoriasis of von Zumbusch238612002
- Bullous eruption271759003
- Disorder of haematopoietic system in newborn414028007
- Disorder of hematopoietic system in newborn414028007
- Arthrocutaneouveal granulamotosis699861000
- Familial granulomatosis, Blau type699861000
- Familial granulomatous inflammatory arthritis, dermatitis and uveitis699861000
- Familial juvenile systemic granulomatosis699861000
- Granulomatous inflammatory arthritis, dermatitis and uveitis, familial699861000
- Paediatric granulomatous arthritis699861000
- Pediatric granulomatous arthritis699861000
- Chronic recurrent multifocal osteomyelitis, congenital dyserythropoietic anaemia and neutrophilic dermatosis703540008
- Chronic recurrent multifocal osteomyelitis, congenital dyserythropoietic anemia and neutrophilic dermatosis703540008
- Majeed syndrome703540008
- Acute blistering eruption of skin721542002
- Acute blistering skin eruption721542002
- Mendelian susceptibility to mycobacterial disease due to complete IL12B deficiency721877008
- Mendelian susceptibility to mycobacterial disease due to complete interleukin 12 subunit beta deficiency721877008
- Monogenic autoinflammatory syndrome724593005
- Early onset sarcoidosis726078000
- Sporadic Blau syndrome726078000
- Infantile-onset periodic fever, panniculitis, dermatosis syndrome765435009
- OTU deubiquitinase with linear linkage specificity related autoinflammatory syndrome765435009
- OTULIN (OTU deubiquitinase with linear linkage specificity) related autoinflammatory syndrome765435009
- OTULIN-related autoinflammatory syndrome765435009
- Otulipenia765435009
- NOD2 (nucleotide binding oligomerization domain containing 2) associated autoinflammatory disease768667002
- NOD2-associated autoinflammatory disease768667002
- Nucleotide binding oligomerization domain containing 2-associated autoinflammatory disease768667002
- Yao syndrome768667002
- APLAID - autoinflammation phospholipase C gamma 2 associated antibody deficiency and immune dysregulation778004006
- Autoinflammation phospholipase C gamma 2 associated antibody deficiency and immune dysregulation778004006
- Autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation778004006
- DITRA - deficiency of interleukin 36 receptor antagonist784339002
- Deficiency of IL-36R antagonist784339002
- Deficiency of IL-36Ra (interleukin 36 receptor antagonist)784339002
- Deficiency of interleukin 36 receptor antagonist784339002
- Blau syndrome818950005
- Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis829973009
- Behçet-like disease due to HA201187119002
- Behçet-like disease due to haploinsufficiency of A201187119002
- Hereditary paediatric Behçet-like disease1187119002
- Hereditary pediatric Behçet-like disease1187119002
- Acute pustular eruption of skin1251376007
- Acute pustular skin eruption1251376007
- Acute pustular skin rash1251376007
- USP18 deficiency1251449006
- Ubiquitin specific peptidase 18 deficiency1251449006
- VEXAS syndrome1290093003
- Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome1290093003
- A20 haploinsufficiency1290916005
- HA20 (haploinsufficiency A20)1290916005
- Haploinsufficiency of A201290916005
- Complex multigenic autoinflammatory syndrome1295181006
- Type 1 interferonopathy1295193002
- Type I interferon-associated autoinflammatory disease1295193002
- Type I interferonopathy1295193002
- NOCARH syndrome1360083000
- Neonatal onset cytopenia, autoinflammation, rash, episodes of hemophagocytic lymphohistiocytosis syndrome1360083000
- SAMD9L-SAAD - SAMD9L-associated autoinflammatory disease1365682007
- SAMD9L-associated autoinflammatory syndrome1365682007
- Sterile alpha motif domain containing 9 like-associated autoinflammatory syndrome1365682007
- Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis due to HOIL-1 deficiency1365687001
- Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis due to HOIP deficiency1365687001
- Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis due to RNF31 mutation1365687001
UMLS
- Arthrocutaneouveal granulomatosisC5201146
- BLAU SYNDROMEC5201146
- BLAUSC5201146
- Blau SyndromeC5201146
- Blau syndromeC5201146
- Blau syndrome (disorder)C5201146
- Pediatric Granulomatous ArthritisC5201146
- Autoinflammatory Disease due to Interleukin-1 Receptor Antagonist DeficiencyC2748507
- Autoinflammatory disease due to interleukin-1 receptor antagonist deficiencyC2748507
- CHRONIC RECURRENT MULTIFOCAL OSTEOMYELITIS 2, WITH PERIOSTITIS AND PUSTULOSISC2748507
- CRMO2C2748507
- DIRAC2748507
- Deficiency Of Interleukin 1 Receptor AntagonistC2748507
- Deficiency of interleukin 1 receptor antagonist [DIRA]C2748507
- Deficiency of interleukin(IL)-1 receptor antagonistC2748507
- Deficiency of interleukin-1 receptor antagonistC2748507
- Deficiency of interleukin-1 receptor antagonist (DIRA)C2748507
- Deficiency of the Interleukin-1 Receptor AntagonistC2748507
- Deficiency of the interleukin-1 receptor antagonistC2748507
- INTERLEUKIN 1 RECEPTOR ANTAGONIST DEFICIENCYC2748507
- Interleukin 1 receptor antagonist deficiencyC2748507
- Interleukin-1 receptor antagonist deficiencyC2748507
- OMPPC2748507
- OMPP - sterile osteomyelitis, multifocal with periostitis and pustulosisC2748507
- OSTEOMYELITIS, STERILE MULTIFOCAL, WITH PERIOSTITIS AND PUSTULOSISC2748507
- Osteomyelitis, sterile multifocal, with periostitis and pustulosisC2748507
- Sterile multifocal osteomyelitis with periostitis and pustulosisC2748507
- Sterile multifocal osteomyelitis with periostitis and pustulosis (disorder)C2748507
- CHRONIC RECURRENT MULTIFOCAL OSTEOMYELITIS 1, WITH CONGENITAL DYSERYTHROPOIETIC ANEMIA, WITH OR WITHOUT NEUTROPHILIC DERMATOSISC1864997
- CRM01C1864997
- CRMO1C1864997
- Chronic Recurrent Multifocal Osteomyelitis, Congenital Dyserythropoietic Anemia, and Neutrophilic DermatosisC1864997
- Chronic recurrent multifocal osteomyelitis 1, with congenital dyserythropoietic anemia, with or without neutrophilic dermatosisC1864997
- Chronic recurrent multifocal osteomyelitis, congenitalC1864997
- Chronic recurrent multifocal osteomyelitis, congenital dyserythropoietic anaemia and neutrophilic dermatosisC1864997
- Chronic recurrent multifocal osteomyelitis, congenital dyserythropoietic anemia and neutrophilic dermatosisC1864997
- Chronic recurrent multifocal osteomyelitis, congenital dyserythropoietic anemia and neutrophilic dermatosis (disorder)C1864997
- Chronic recurrent multifocal osteomyelitis-congenital dyserythropoietic anemia-neutrophilic dermatosis syndromeC1864997
- Congenital dyserythropoietic anemia and chronic recurrent multifocal osteomyelitisC1864997
- Dyserythropoietic anemia, and neutrophilic dermatosisC1864997
- MAJEED SYNDROMEC1864997
- MJDSC1864997
- Majeed SyndromeC1864997
- Majeed syndromeC1864997
- Majeed's syndromeC1864997
- FAMILIAL RECURRENT ARTHRITISC1858361
- FRAC1858361
- Familial recurrent arthritisC1858361
- PAPAC1858361
- PAPA (pyogenic arthritis, pyoderma gangrenosum, acne) syndromeC1858361
- PAPA SYNDROMEC1858361
- PAPA syndromeC1858361
- PAPASC1858361
- PYOGENIC STERILE ARTHRITIS, PYODERMA GANGRENOSUM, AND ACNEC1858361
- Pyogenic Arthritis, Pyoderma Gangrenosum and AcneC1858361
- Pyogenic Sterile Arthritis, Pyoderma Gangrenosum, and AcneC1858361
- Pyogenic arthritis, pyoderma gangrenosum, acne syndromeC1858361
- Pyogenic arthritis, pyoderma gangrenosum, acne syndrome (disorder)C1858361
- Pyogenic arthritis, pyoderma gangrenosum, and acneC1858361
- Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome [PAPA]C1858361
- Pyogenic arthritis, pyoderma gangrenosum, and severe cystic acneC1858361
- Pyogenic sterile arthritis pyoderma gangrenosum and acne syndromeC1858361
- Other autoinflammatory syndromesC4268693
- Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome [PFAPA]C4268694
Frequently Asked Questions
What is ICD-10 code M04.8?
ICD-10-CM code M04.8 represents "Other autoinflammatory syndromes". It is a billable/specific code that can be used on a claim.
Is M04.8 a billable code?
Yes, M04.8 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.
What chapter is M04.8 in?
M04.8 is in Chapter 13: Diseases of the Musculoskeletal System and Connective Tissue (codes M00-M99).
What SNOMED CT codes does M04.8 map to?
M04.8 maps to 31 SNOMED CT concepts: 1290916005, 778004006, 5876000, 721542002, 238612002, and 26 more. SNOMED CT is a clinical terminology used in electronic health records.
What are the UMLS CUIs for M04.8?
M04.8 is linked to 6 UMLS Concept Unique Identifiers: C5201146, C2748507, C1864997, C1858361, C4268693, and 1 more. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.
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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.