A Case of Erythema Nodosum and Serositis Associated with Myelodysplastic Syndrome
A 34-year-old woman was diagnosed with MDS French-American-British (FAB) subtype refractory anemia in March, 2000. The patient did not receive any special treatment except intermittent transfusion of packed red cell.\nIn May, 2003 the patient presented with fatigue, myalgia, fever as well as multiple edematous, tender, erythematous to brownish subcutaneous nodular lesions on both upper and lower extremities and trunk (). Laboratory finding indicated hemoglobin 5.8 g/dL, white blood cell count 6.8×109/L, platelet 4.8×1010/L, and normal liver and kidney function test. Biopsy of skin lesion revealed lobular panniculitis consistent with erythema nodosum (). The skin lesion had improved with 20 mg of prednisone per day.\nOne month later, dyspnea, chest pain and fever developed and the skin lesions were aggravated. She was taking a tapering dose of prednisone (10 mg). The laboratory tests showed hemoglobin 6.0 g/dL, white blood cell count 4.8×109/L, platelet 8.0×109/L, uric acid 2.3 mg/dL, LDH 474 U/L (normal 211-423), serum ferritin 1831 ng/mL, serum iron/TIBC 145/162 µ g/dL. Antinuclear antibody, rheumatoid factor and ANCA were all negative. The levels of C3 and C4 were normal. Blood and urine culture were sterile. Chest X-ray and chest CT scan showed cardiomegaly with pulmonary edema (). Echocardiography revealed normal ejection fraction with large amounts of pericardial effusion. After the dose of prednisone was increased to 40 mg/day, the pericardial effusion and skin lesions had remarkably improved, suggesting that these newly developed symptoms might be attributed to the immune-mediated complications of MDS. The repeated bone marrow biopsy performed one month later revealed no evidence of progression to acute leukemia. However, the dysplastic changes in the myeloid, erythroid and megakaryocytes lineages had progressed compared to the examination in the prior month. While gradually tapering prednisone until July 2004, she was taking 5 mg/day of prednisone without pericardial effusion. Platelet count slowly increased and maintained above 2.0×1010/L.