Langerhansfruma

Langerhansfruma, also known as Langerhans cell histiocytosis (LCH), is a rare disease characterized by an overproduction of Langerhans cells, a type of immune cell. These cells normally help the body fight infection, but in LCH, they accumulate in various parts of the body, forming tumors or lesions. The exact cause of LCH is not fully understood, but it is not contagious and is not considered hereditary.

LCH can affect people of all ages, though it is most common in young children. The symptoms can vary widely depending on where the Langerhans cells accumulate. Common sites include bones, skin, lymph nodes, and the pituitary gland. Bone lesions can cause pain, swelling, and fractures. Skin rashes are also frequent. Involvement of the pituitary gland can lead to hormonal imbalances. In more severe cases, LCH can affect multiple organ systems.

Diagnosis typically involves a biopsy of the affected tissue to examine the cells. Imaging tests like X-rays, CT scans, or MRIs may be used to assess the extent of the disease. Treatment approaches depend on the severity and location of LCH. For localized disease, observation or surgical removal may be sufficient. More widespread or aggressive forms may require chemotherapy, radiation therapy, or steroid treatments. While LCH can be serious, many patients, especially children, respond well to treatment and can achieve long-term remission.