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  2. ICD-10-CM Codes List
  3. D50–D89
  4. D80-D89
  5. Other immunodeficiencies (D84)

Other immunodeficiencies (D84)

The ICD-10 code section D84 covers various other immunodeficiencies, which are disorders where parts of the immune system are impaired but do not fall into more common categories. These codes are used to document rare or specific immune system defects not classified elsewhere.

This section includes codes like D84.0 for lymphocyte function antigen-1 (LFA-1) defect, which affects white blood cell interactions, and D84.1 for complement system defects, linked to various conditions such as hereditary angioedema and atypical hemolytic uremic syndrome. These codes clarify diagnoses when terms like complement deficiency disease or hereditary angioedema with C1 esterase inhibitor deficiency are encountered. Other codes, including D84.81 and D84.82, address immunodeficiency caused by external factors like drugs or radiation. Additionally, D84.89 contains a wide range of specific immunodeficiencies with complex syndromes, helping coders connect uncommon names such as Mendelian susceptibility to mycobacterial disease or immune dysregulation with recurrent infection to this classification. Finally, D84.9 is used when the immunodeficiency is unspecified, providing flexibility for diverse clinical presentations.

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

Atypical Hemolytic Uremic Syndrome

An hereditary hemolytic uremic syndrome associated with variations in the gene that encodes COMPLEMENT FACTOR H, or the related proteins CFHR1 and CFHR3. Disease often progresses to CHRONIC KIDNEY FAILURE without the prodromal symptoms of ENTEROCOLITIS and DIARRHEA that characterize typical hemolytic uremic syndrome.

Livedo Reticularis

A condition characterized by a reticular or fishnet pattern on the skin of lower extremities and other parts of the body. This red and blue pattern is due to deoxygenated blood in unstable dermal blood vessels. The condition is intensified by cold exposure and relieved by rewarming.

Livedoid Vasculopathy

A rare cutaneous thrombotic disease due to occlusion of dermal vessels. It is characterized by purpuric maculae and ulcerations especially during summer which form scars called atrophie blanche. It is more associated with other syndromes (e.g., PROTEIN C DEFICIENCY; HYPERHOMOCYSTEINEMIA). Livedo reticularis with systemic involvement and stroke is SNEDDON SYNDROME.

Sneddon Syndrome

A systemic non-inflammatory arteriopathy primarily of middle-aged females characterized by the association of LIVEDO RETICULARIS, multiple thrombotic CEREBRAL INFARCTION; CORONARY DISEASE, and HYPERTENSION. Elevation of antiphospholipid antibody titers (see also ANTIPHOSPHOLIPID SYNDROME), cardiac valvulopathy, ISCHEMIC ATTACK, TRANSIENT; SEIZURES; DEMENTIA; and chronic ischemia of the extremities may also occur. Pathologic examination of affected arteries reveals non-inflammatory adventitial fibrosis, thrombosis, and changes in the media (From Jablonski, Dictionary of Syndromes & Eponymic Diseases, 2d ed; Adams et al., Principles of Neurology, 6th ed, p861; Arch Neurol 1997 Jan;54(1):53-60). Mutations in the CECR1 gene (ADA2 protein, human) are associated with Sneddon syndrome.