2026 ICD-10-CM Diagnosis Code C96.6Unifocal Langerhans-cell histiocytosis

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

• Churg-Strauss Syndrome

  widespread necrotizing angiitis with granulomas. pulmonary involvement is frequent. asthma or other respiratory infection may precede evidence of vasculitis. eosinophilia and lung involvement differentiate this disease from polyarteritis nodosa.

• Eosinophilic Granuloma

  the most benign and common form of langerhans-cell histiocytosis which involves localized nodular lesions predominantly of the bones but also of the gastric mucosa, small intestine, lungs, or skin, with infiltration by eosinophils.

• Kimura Disease

  a chronic inflammatory disease characterized by benign enlargement of cervical lymph node and salivary glands with increased levels of immunoglobulin e. unlike angiolymphoid hyperplasia with eosinophilia it involves eosinophil infiltrates in lymph node and salivary glands and mostly found in asian males.

• Polyarteritis Nodosa

  a form of necrotizing non-granulomatous inflammation occurring primarily in medium-sized arteries, often with microaneurysms. it is characterized by muscle, joint, and abdominal pain resulting from arterial infarction and scarring in affected organs. polyarteritis nodosa with lung involvement is called churg-strauss syndrome.

• Eosinophils

  granular leukocytes with a nucleus that usually has two lobes connected by a slender thread of chromatin, and cytoplasm containing coarse, round granules that are uniform in size and stainable by eosin.

Langerhans cell histiocytosis

Langerhans cell histiocytosis is a disorder in which excess immune system cells called Langerhans cells build up in the body. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin, lymph nodes, spleen, lungs, liver, and bone marrow.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.