2026 ICD-10-CM Diagnosis Code M33.90Dermatopolymyositis, unspecified, organ involvement unspecified

AHRQ’s CCSR groups this code into broader clinical categories.

• Dermatomyositis

  a subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. the illness occurs with approximately equal frequency in children and adults. the skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. the disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. the childhood form of this disease tends to evolve into a systemic vasculitis. dermatomyositis may occur in association with malignant neoplasms. (from adams et al., principles of neurology, 6th ed, pp1405-6)

• Exosome Component 10|Autoantigen PM-SCL|Autoantigen PM/Scl|EC 3.1.13.-|EXOSC10|P100 Polymyositis-Scleroderma Overlap Syndrome-Associated Autoantigen|PM/Scl-100|Polymyositis/Scleroderma Autoantigen 100 kDa|Polymyositis/Scleroderma Autoantigen 2

  exosome component 10 (885 aa, ~101 kda) is encoded by the human exosc10 gene. this protein plays a role in the maturation and degradation of rna.

• Other Overlap Syndromes|Other overlap syndromes

  evidence of other overlap syndromes not specified elsewhere.

• Overlap Syndrome

  an autoimmune, connective tissue disorder in which the patient exhibits features from two or more diseases. these typically include systemic sclerosis, dermatomyositis, polymyositis, rheumatoid arthritis, systemic lupus erythematosus, and sjogren syndrome; in pediatrics the respective pediatric entities are encountered.

• Scleroderma Polymyositis Overlap Syndrome|Scleroderma Polymyositis

  a rare autoimmune disorder in which patients present with overlapping symptoms of systemic scleroderma and polymyositis or dermatomyositis.

• Adult Dermatomyositis

  dermatomyositis in an adult.

• Childhood Dermatomyositis|JDM|JDM|Juvenile Dermatomyositis|Juvenile Dermatomyositis

  an inflammatory myopathy of childhood resulting in muscle weakness, and associated with a characteristic skin rash.

• Cutaneous Dermatomyositis Disease Area and Severity Index|CDASI|CDASI

  an instrument that measures activity and damage in the skin of dermatomyositis patients.

• Dermatomyositis

  inflammation of the skin and muscle.

• Dermatomyositis Skin Severity Index|DSSI|DSSI

  an assessment of disease activity in the skin of patients with dermatomyositis.

• Interferon-Induced Helicase C Domain-Containing Protein 1|CADM-140 Autoantigen|Clinically Amyopathic Dermatomyositis Autoantigen 140 kDa|EC 3.6.4.13|Helicard|Helicase with 2 CARD Domains|IFIH1|Interferon-Induced With Helicase C Domain Protein 1|MDA-5|Melanoma Differentiation Associated Protein-5|Melanoma Differentiation-Associated Protein 5|Murabutide Down-Regulated Protein|RLR-2|RNA Helicase-DEAD Box Protein 116

  interferon-induced helicase c domain-containing protein 1 (1025 aa, ~117 kda) is encoded by the human ifih1 gene. this protein is involved in the positive regulation of antiviral responses.

• Juvenile Dermatomyositis Sine Myositis|Juvenile Amyopathic Dermatomyositis|Juvenile Amyopathic Dermatomyositis|Juvenile Dermatomyositis sine Myositis|Juvenile dermatomyositis without myopathy

  a rare form of juvenile dermatomyositis that manifests with characteristic cutaneous findings for at least six months in the absence of any detectable muscle involvement.

• Juvenile Dermatomyositis with Myopathy|Juvenile dermatomyositis with myopathy

  evidence of juvenile dermatomyositis with myopathy.

• Juvenile Dermatomyositis with Other Organ Involvement|Juvenile dermatomyositis with other organ involvement

  evidence of juvenile dermatomyositis with other organ involvement.

• Juvenile Dermatomyositis with Respiratory Involvement|Juvenile dermatomyositis with respiratory involvement

  evidence of juvenile dermatomyositis with respiratory involvement.

• Juvenile Dermatomyositis, Organ Involvement Unspecified|Juvenile dermatomyositis, organ involvement unspecified

  evidence of juvenile dermatomyositis, organ involvement unspecified.

• Other Dermatomyositis with Myopathy|Other dermatomyositis with myopathy

  evidence of other dermatomyositis with myopathy not specified elsewhere.

• Other Dermatomyositis with Other Organ Involvement|Other dermatomyositis with other organ involvement

  evidence of other dermatomyositis with other organ involvement not specified elsewhere.

• Other Dermatomyositis with Respiratory Involvement|Other dermatomyositis with respiratory involvement

  evidence of other dermatomyositis with respiratory involvement not specified elsewhere.

• Other Dermatomyositis without Myopathy|Other dermatomyositis without myopathy

  evidence of other dermatomyositis without myopathy not specified elsewhere.

• Other Dermatomyositis, Organ Involvement Unspecified|Other dermatomyositis, organ involvement unspecified

  evidence of other dermatomyositis, organ involvement unspecified not specified elsewhere.

• Exosome Complex Component RRP45|AMPA RECEPTORS|Autoantigen PM/Scl 1|EXOSC9|Exosome Component 9|GLuRs|P75 Polymyositis-Scleroderma Autoantigen|P75 Polymyositis-Scleroderma Overlap Syndrome Associated Autoantigen|P75 Polymyositis-Scleroderma Overlap Syndrome-Associated Autoantigen|PM/Scl-75|Polymyositis/Scleroderma Autoantigen 1|Polymyositis/Scleroderma Autoantigen 75 kDa

  exosome complex component rrp45 (439 aa, ~49 kda) is encoded by the human exosc9 gene. this protein is involved in the regulation of the exoribonuclease activity of the exosome.

• Exosome Component 10|Autoantigen PM-SCL|Autoantigen PM/Scl|EC 3.1.13.-|EXOSC10|EXOSC10|P100 POLYMYOSITIS-SCLERODERMA AUTOANTIGEN|P100 Polymyositis-Scleroderma Overlap Syndrome-Associated Autoantigen|PM/Scl 2|PM/Scl-100|PM/Scl-100|Polymyositis/Scleroderma Autoantigen 100 kDa|Polymyositis/Scleroderma Autoantigen 2|Polymyositis/Scleroderma Autoantigen 2

  exosome component 10 (885 aa, ~101 kda) is encoded by the human exosc10 gene. this protein plays a role in the maturation and degradation of rna.

• Chromodomain-Helicase-DNA-Binding Protein 3|ATP-Dependent Helicase CHD3|CHD-3|CHD3|Chromodomain Helicase DNA Binding Protein 3|Chromodomain Helicase DNA-Binding Protein 3|Dermatomyositis Autoantigen Mi-2 Alpha|EC 3.6.4.12|MI-2 ALPHA ANTIGEN|MI-2 Alpha|MI-2a|Mi-2 Alpha|Mi-2 Autoantigen 240 kDa Protein|Mi2-Alpha|Zinc Finger Helicase|hZFH

  chromodomain-helicase-dna-binding protein 3 (2000 aa, ~227 kda) is encoded by the human chd3 gene. this protein is involved in chromatin remodeling, spindle organization during mitosis and transcriptional repression.

• Chromodomain-Helicase-DNA-Binding Protein 4|ATP-Dependent Helicase CHD4|CHD-4|CHD4|Chromodomain Helicase DNA-Binding Protein 4|Dermatomyositis Autoantigen Mi-2 Beta|EC 3.6.4.12|MI-2 BETA ANTIGEN|MI-2 Beta|MI-2b|Mi-2 Autoantigen 218 kDa Protein|Mi-2 Beta|Mi2-Beta

  chromodomain-helicase-dna-binding protein 4 (1912 aa, ~218 kda) is encoded by the human chd4 gene. this protein is involved in chromatin remodeling.

• MI-2 Antigens|Dermatomyositis Autoantigen Mi-2|MI-2 ANTIGENS|Major Dermatomyositis-specific Mi-2 Autoantigen

  antigens derived from certain helicases in the nucleosome remodeling-deacetylase complex (nurd) involved in transcription regulation that may be comprised of (but not limited to) two isoforms mi-2 alpha and mi-2 beta. they can be considered as autoantigens and are commonly associated with systemic autoimmune myopathy dermatomyositis.

Myositis

Myositis means inflammation of the muscles that you use to move your body. An injury, infection, or autoimmune disease can cause it. Two specific kinds are polymyositis and dermatomyositis. Polymyositis causes muscle weakness, usually in the muscles closest to the trunk of your body.

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