Chronic multifocal Langerhans cell histiocytosis

Hans-Schuller-Christian

Chronic multifocal Langerhans cell histiocytosis (CMLCH) is a rare disease characterized by the proliferation of Langerhans cells, a type of dendritic cell that normally helps regulate the immune system. This condition is part of a group of disorders known as Langerhans cell histiocytosis (LCH).

Presentation

CMLCH typically presents in childhood and can affect multiple organs. Common symptoms include bone pain, skin rash, fever, and weight loss. The disease can lead to complications such as diabetes insipidus, lung disease, and liver disease.

Pathophysiology

The exact cause of CMLCH is unknown. It involves the abnormal accumulation and proliferation of Langerhans cells in various tissues, leading to the formation of granulomas and tissue damage.

Diagnosis

Diagnosis of CMLCH is based on clinical presentation, biopsy of affected tissues, and imaging studies such as X-ray, CT scan, and MRI. The biopsy typically shows the presence of Langerhans cells, which can be identified by their characteristic markers.

Differential Diagnosis

The differential diagnosis for CMLCH includes:

• Osteomyelitis
• Ewing sarcoma
• Lymphoma

Treatment

Treatment options for CMLCH include:

• Chemotherapy: Often used to reduce the number of Langerhans cells.
• Radiation therapy: Used in cases where the disease is localized.
• Surgery: May be necessary to remove isolated lesions.

Prognosis

The prognosis for CMLCH is variable and depends on the extent of organ involvement and response to treatment. Some patients may experience a chronic course with periods of remission and relapse.

See also

• Langerhans cell histiocytosis
• Hematology
• Oncology