Weber Christian Disease Presenting With Pacytopenia and Anticardiolipin Antibodies

Mehmet Yılmaz; Simten Dagdaş; Nuran Allı; Sibel Erdoğan; S. Zeynep Akı; Gülsüm Özet

doi:10.58600/eurjther.2007-13-2-1382-arch

Authors

Mehmet Yılmaz Gaziantep University School of Medicine Department of Hematology
Simten Dagdaş Ankara Numune Education and Research Hospital, Department of Hematology
Nuran Allı Ankara Numune Education and Research Hospital, Department of Dermatology
Sibel Erdoğan Ankara Numune Education and Research Hospital, Department of Pathology
S. Zeynep Akı Gaziantep University School of Medicine Department of Hematology
Gülsüm Özet Gaziantep University School of Medicine Department of Hematology

DOI:

https://doi.org/10.58600/eurjther.2007-13-2-1382-arch

Keywords:

Weber-Christian disease, Pancytopenia, Anticardiolipin antibody

Abstract

Weber-Christian disease is a controversial entity that represents reccurent fever, inflamation of adipose tissue and tender subcutaneus nodules. The etiolgy of WCD remains unknown. However, ıt has been related to an immunologically mediated reaction to diverse antigenic stimuli. We present a 50 year-old man with Weber-Christian disease presented with fever, reccurent pancytopenia attacks, IgG and IgM anticardiolipin antibody positivitiy.. The patient showed fever and characteristic redish subcutaneous nodules. Biopsy taken from the lesion showed areas of fat necrosis, accumulation of leucocytes and macrophages with foamy cytoplasm. and no evidence of vasculitis. Histopathologic findings were consistent with Weber-Christian panniculitis. Bone marrow aspirate and biopsy disclosed mild hypercellularitiy with concomitant mild increasing lympocyte and plasma cells. Patients with pancytopenia, cutaneous lesions and ACA positivity must be searched for WCD.

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