Abstract

Chronic Lymphocytic leukemia (CLL) is generally associated with a variety of autoimmune diseases, while the incidence of vasculitis in CLL is very low (0.1-2%).  Most of the cases of CLL associated vasculitis are leukocytoclastic in nature.  Herein, we report a rare case of CLL associated with a low grade lymphocytic vasculitiswith Anti-nuclear antibody (ANA) positivity.

An 89-year-old male with history of CLL since 2006 who presented to dermatology clinic with one-week history of palpable purpuric rash, coalescing into plaques on his lower leg. A punch biopsy was performed, which showedan extensive perivascular and periadnexal inflammatory infiltrate. The inflammatory infiltrate predominantly consisted of lymphocytes and scattered eosinophils. On Immunohistochemical examination, CD3 highlighted small reactive T lymphocytes. CD4: CD8 ratio was preserved with no loss of CD5 and CD7.  CD20 highlighted rare B cells. CD10, BCL-6, CD23, CyclinD1 and MUM1 highlighted rare cells. These combined findings rule out a cutaneous manifestation of chronic lymphocytic leukemia. The vessels showed protuberant endothelial lining and narrowing of lumen. Combining clinicopathologic findings, diagnosis of an early low grade lymphocytic vasculitis was rendered.

To this date, only one case of CLL associated lymphocytic vasculitis has been reported in literature. In our case, ANA screen and rheumatic factor were positive, while C3 and C4 complements were decreased. We aim to hypothesis,that CD5-positive clonal B cells might play an important role in CLL associated lymphocytic vasculitis. Pathologists/ dermatopathologists should be aware of this rare association of CLL with lymphocytic vasculitis with possible autoimmune etiology.