C96.6

Applicable To

• Eosinophilic granuloma

• Histiocytosis X, unifocal

• Histiocytosis X NOS

• Langerhans-cell histiocytosis NOS

Approximate Synonyms

• Histiocytosis x

• Histiocytosis, langerhans cell, unifocal

• Histiocytosis, unifocal langerhans-cell

• Langerhans cell histiocytosis

• Langerhans cell histiocytosis, unifocal clinical)

Clinical Information

• A clinical variant of langerhans cell histiocytosis characterised by unifocal involvement of a bone most often), skin, or lung. Patients are usually older children or adults usually presenting with a lytic bone lesion. The etiology is unknown. Morphologically, eosinophilic granuloma is characterised by the presence of langerhans cells in a characteristic milieu which includes histiocytes, eosinophiles neutrophiles, and small, mature lymphocytes.

• A group of disorders resulting from the abnormal proliferation of and tissue infiltration by langerhans cells which can be detected by their characteristic birbeck granules x bodies), or by monoclonal antibody staining for their surface cd1 antigens. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder.

• A group of rare disorders in which too many langerhans cells a type of white blood cell) grow in certain tissues and organs including the bones, skin, and lungs, and damage them. Langerhans cell histiocytosis may also affect the pituitary gland which makes hormones that control other glands and many body functions, especially growth). Langerhans cell histiocytosis is most common in children and young adults.

• A multifocal, multisystem form of langerhans-cell histiocytosis. There is involvement of multiple organ systems including the bones, skin, liver, spleen, and lymph nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy, bone and skin lesions, and pancytopenia.

• A multifocal, unisystem form of langerhans-cell histiocytosis. There is involvement of multiple sites in one organ system, most frequently the bone. Patients are usually young children presenting with multiple destructive bone lesions.

• A neoplastic proliferation of langerhans cells which contain birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, letterer-siwe disease, and hand-schuller-christian disease. The clinical course is generally related to the number of organs affected at presentation. who, 2001)

• Group of disorders of histiocyte proliferation which includes letterer-siwe disease; hand-schueller-christian syndrome; and eosinophilic granuloma; langerhans cells are components of the lesions.

• Most benign clinical form of langerhans-cell histiocytosis, which involves localized nodular lesions of the gastric mucosa, small intestine, bones, lungs, or skin, with infiltration by eosinophils; the proliferating cell that appears to be responsible for the clinical manifestations is the langerhans cell.

• 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.

Details

• C96.6 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM C96.6 became effective on October 1, 2022.

• This is the American ICD-10-CM version of C96.6 – other international versions of ICD-10 C96.6 may differ.