Acta Dermato-Venereologica 99-1CompleteContent | Page 24
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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