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Self-healing Langerhans cell histiocytosis (LCH) of the skin
This is a clonal neoplastic proliferation of Langerhans type cells that express CD1a, langerin and S100 protein, and shows Birbeck granules by ultrastructural examination.
(This neoplasm is reportable for cases diagnosed 2010 and later. Some of the terms were also reportable for earlier years.)
Langerhans cell histiocytosis presents in several different forms
1. SOLITARY form: bone and adjacent soft tissue (skull, femur, vertebra, pelvic bones, and ribs) and less commonly lymph nodes, skin, and lung.
2. MULTIFOCAL lesions: largely confined to bone and adjacent soft tissue.
3. MULTISYSTEM disease: skin, bone, and bone marrow are preferential sites of involvement. The liver and spleen are also common sites; however they are typically considered metastases and not the primary site.
TYPES of Langerhans disease:
1. UNIFOCAL disease-usually older children or adults who most commonly present with a lyticbonelesion, eroding the cortex. Solitary lesions at other sites present as masslesions or enlarged lymph nodes.
2. UNISYSTEM multifocal disease-usually young children who present with multiple or sequential destructive bonelesions often associated with adjacent soft tissuemasses. Skull and mandibular involvement is common. Diabetes insipidus follows cranial involvement.
3. MULTISYSTEM disease: infants who present with fever, cytopenia, skin and bonelesions, and hepatosplenomegaly. Pulmonary disease in childhood is clinically variable.
Involvement of bone marrow, liver, and lung are regarded as high-risk factors.
Self-healing Langerhans cell histiocytosis (LCH) of the skin means that the disease regressed without treatment. This is a known phenomenon and is reportable as 9751/3.
NOTE: Langerhans histiocytosis, unifocal (9752/1 in ICD-O-3 for 2001-2009) Langerhans cell histiocytosis, multifocal (9753/1), and Langerhans cell histiocytosis, disseminated (9754/3) are now grouped with the newly reportable Langerhans cell histiocytosis (9751/3).