Acta Dermato-Venereologica 99-1CompleteContent | Page 24

97 SHORT COMMUNICATION Cytophagic Histiocytic Panniculitis Associated with Myelodysplastic Syndrome Ryoko KIMURA, Kazunari SUGITA, Hiroyuki GOTO and Osamu YAMAMOTO Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University, Yonago, Japan. E-mail: [email protected] Accepted Sep 18, 2018; Epub ahead of print Sep 18, 2018 Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis that usually presents with liver failure and haemorrhage, leading to death in most cases (1). Histo- pathologically, skin lesions consist of benign histiocytes, including phagocytosed erythrocytes, leukocytes and platelets. We report here a rare case of CHP associated with myelodysplastic syndrome (MDS). CASE REPORT An 88-year-old man was admitted to our hospital with a 10-day history of high fever and painful plaques on his extremities. He had been on anti-angina medications for 40 years. Physical examination revealed a fever of 38.7°C and multiple erythematous, tender and indurated plaques on his arms and legs (Fig. 1). He had no evidence of lymphadenopathy. Blood analysis revealed an elevated total white blood cell count (10.3 × 10 3 /μl; normal range 3.3–8.8 × 10 3 ) with elevated level of neutrophils (88%; 40–70%) and myelocytes (1.0%; 0%) and low level of haemoglobin (8.3; 12–17 g/dl). Platelet count was within the normal range (192 × 10 3 ; 125–343 × 10 3 /μl). Chemistry profile results included elevations of alkaline transferase (AST) (41; 10–40 U/l), aspartate transami- nase (AST) (49; normal range 13–30 U/l), C-reactive protein (18.9; normal range 0–0.15 mg/dl), ferritin (567; normal range 23.9–336.2 ng/ml) and α chain of solu- ble IL2 receptor (1,700; normal range 122–496 U/ml), prolonged activated partial thromboplastin time (40.1; normal range 23.3–38.2s), and normal prothrombin ratio (1.20; 0.86–1.21). Triglyceride (85; 40–149 mg/dl) and fibrinogen (294; 170–410 mg/dl) levels were within normal limits. Cytomegalovirus antigenaemia was not detected. Epstein Barr virus (EBV) viral capsid antigen antibody (VCA) was negative for IgM and positive Fig. 1. Erythematous plaques (a) on the arm and (b) on the leg. for IgG. Computed tomography (CT) of the chest and abdomen demonstrated mediastinal lymphadenopathy as well as hepatomegaly. A skin biopsy from the right lower thigh exhibited lobular panniculitis with mixed inflammatory infiltrations of neutrophils, lymphocytes and histiocytes without any atypia (Fig. 2a, b). Some histiocytes had phagocytized erythrocytes (Fig. 2c). A bone marrow biopsy revealed hypercellularity with increased numbers of megakaryocytes and granulocy- tes (Fig. 2d). Chromosomal analyses of bone marrow cells showed 46, XY, t(7; 17) (q36; q21). Bone marrow blasts accounted for 1% of the cells. Based on clinical, histopathological and molecular analyses, the patient was diagnosed as having CHP with very low-grade MDS. He required erythrocyte transfusion and erythropoietin therapy for his low haemoglobin level. He was treated with a low-dose oral corticosteroid (betamethasone 1 mg daily) because he chose not to have aggressive therapy Fig. 2. (a) A biopsy specimen from the leg (haematoxylin and eosin (HE), original magnification ×20). (b) Inflammatory cells in square of Fig. 2a (HE, ×200). (c) Phagocytosed erythrocytes (arrows) (HE, ×400). (d) A bone marrow biopsy finding (HE, ×200). This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica. doi: 10.2340/00015555-3043 Acta Derm Venereol 2019; 99: 97–98