Multisystem Langerhans Cell Histiocytosis in Adult
نویسندگان
چکیده
Langerhans cell histiocytosis (LCH), is a rare disorder, clinically presents with heterogeneous manifestations, and has an unpredictable outcome. Commonly seen in infancy or early childhood, the disorder is characterized by proliferation of abnormal and clonal Langerhans cell in skin, bone, lymph nodes, lungs, liver, spleen, and bone marrow. Occurrence of LCH in adults is rare. Here, we report the case of an adult with acute onset of polymorphic eruptions all over the body, which on biopsy showed features of multisystem LCH, and was confirmed by immunohistochemistry. Although multisystem LCH has a poor prognosis, our patient responded well to chemotherapy.
منابع مشابه
Current Treatment Strategy in Langerhans Cell Histiocytosis
Langerhans cell histiocytosis (LCH) is a rare disorder described as three different entities including eosinophilic granulomaof bone, the Hand-Schuller-Christian syndrome, and Letterer-Siwe disease. LCH is currently classified into singlesystem LCH, and multisystem LCH. Patients with single system LCH have an excellent prognosis, and are mostlytreated with local therapy. Multisystem LCH is subd...
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