4 yrs boy. 20002/12 skin biopsy with clinical diagnosis M.Darier. Histological dgn.: Langerhans' cell histiocytosis in skin(low quality of pictures).2005/03 BM biopsy is performed. Histological diagnosis: compatible with Langerhans' cell histiocytosis in BM (low quality of pictures). X-ray multiple defects in bones, compatible with diagnosis Langerhans' cell histiocytosis (Eo granuloma). Appropriate treatment was applied. 2005/06 spleen and liver biopsies and 2005/09 splenectomy with lymhonodectomy and liver biopsy. Spleen: architecture is partially destroyed by fibroblastic proliferation with deposition of siderofages. There are multiple multinucleated histiocytes with emperipolesis/ phagocytosis of erythrocytes and granuliocytes. Some giant cells with lobulated nuclei and amphophilic nucleoli. There is vague nodularity in parenchima. There are single CD1a+ cells. Lymph node in hilus: identical findings.  
Biopsy of liver: pigmented multinucleated histiocytes in sinusoids (2005/06 Liver tissue appears normal). PROPOSED DIAGNOSIS: Langerhans' cell histiocytosis combined with Rosai- Dorfman disease? Or secondary phenomenon of macrophageal reaction?  
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