D82.4 Hyperimmunoglobulin E [IgE] syndrome

ICD-10-CM D82.4: Hyperimmunoglobulin E [IgE] syndrome

ICD-10-CM code D82.4 represents Hyperimmunoglobulin E [IgE] syndrome, an active diagnosis concept classified under immunodeficiency associated with other major defects. The condition involves recurrent infections, infantile dermatitis, and elevated IgE levels. This reference summarizes official terminology and clinical characteristics without providing personal medical advice. 1 2

Plain-language overview

Hyperimmunoglobulin E syndromes are rare immunologic disorders defined by recurrent skin and sinopulmonary infections, dermatitis beginning in infancy, and markedly elevated immunoglobulin E levels. 2

Patients with these syndromes may experience variable skeletal, dental, vascular, neurologic, and malignant complications, including an elevated risk for hematologic malignancies such as Hodgkin and non-Hodgkin lymphoma. 2

What this code represents

ICD-10-CM code D82.4 is an active terminology record with the preferred name Hyperimmunoglobulin E [IgE] syndrome, and its semantic type is classified as a Disease or Syndrome. 1

Code D82.4 has D82 as its parent concept, which represents immunodeficiency associated with other major defects, and the D82.4 record itself contains narrower concepts within the terminology. 1

Coding details

The D82.4 code for Hyperimmunoglobulin E [IgE] syndrome maps to the legacy ICD-9-CM code 279.8, while the related D82.8 code maps to the same legacy numerical code. 3

Documentation considerations

Clinical documentation for this condition may need to distinguish between autosomal dominant STAT3-HIES and autosomal recessive DOCK8 deficiency due to different complication profiles and mortality risks. 2

Diagnostic accuracy requires differentiating hyperimmunoglobulin E syndromes from atopic dermatitis, Wiskott-Aldrich syndrome, Netherton syndrome, Omenn syndrome, DiGeorge syndrome, and other disorders with elevated immunoglobulin E levels. 2

What the sources add

While the official terminology record establishes the active code hierarchy and preferred name, the clinical reference details the specific genetic variants, diagnostic triad, and differential diagnosis necessary for clinical understanding. 1 2

The clinical reference indicates autosomal dominant HIES is associated with high morbidity, while autosomal recessive DOCK8 deficiency carries markedly increased mortality without hematopoietic stem cell transplant. 2

Sources

1. ICD-10-CM D82.4 terminology record — National Cancer Institute Enterprise Vocabulary Services; accessed 2026-07-27.
2. Bookshelf — ncbi.nlm.nih.gov; accessed 2026-07-27.
3. immunodeficiencies disease codes icd-10 cm icd-9 cm — aaaai.org; accessed 2026-07-27.
4. D82.4 - Hyperimmunoglobulin E [IgE] syndrome | ICD-10-CM — unboundmedicine.com; accessed 2026-07-27.