We report a case of
malignant histiocytosis which began with the skin lesions of
panniculitis. A 32-year-old woman presented with recurrent erythematous plaques, subcutaneous nodules, and
ulcers on the trunk and the extremities and intermittent
fever for 7 months. The cutaneous lesions consisted of erythematous and brownish irregular-shaped patches and tender cutaneous nodules 0.5-1.0 cm in diameter. Central
necrosis and shallow
ulcers were seen in the lesions. The patient also suffered from general
fatigue,
arthralgia, and
weight loss. She was anemic and thrombocytopenic and had progressive impairment of liver function with coagulation defect. Histopathological study of skin lesions showed lobular
panniculitis without
vasculitis in the subcutaneous fat tissue. In the
panniculitis lesion, moderate mixed cell infiltration consisting of lymphocytes and histiocytes was observed. Bone marrow aspiration revealed an increase in the number of histiocytes, mostly immature with active phagocytosis of erythroid cells, myeloid cells, and platelets. She was diagnosed as having
malignant histiocytosis and treated with
cyclophosphamide,
vincristine, and
prednisolone which she responded well; her
fever subsided and the lesions healed with
hyperpigmentation. In this patient, benign histiocytes with hemophagocytosis without immature forms were found in the skin lesions. According to our knowledge, this is the first Thai report of
malignant histiocytosis with clinical features of
panniculitis.