Case Number : Case 3063 - 04 April 2022 Posted By: Dr. Mona Abdel-Halim

Subcutaneous panniculitis-like T cell lymphoma and lupus panniculitis are the differentials. While the constitutional symptoms suggest the former, it's difficult to rule out LE panniculitis without IHC for T cell markers and serological tests for LE. A considerable overlap between the two has been described. LE profundus may progress to SPTCL and it's likely that the two belong to a spectrum of subcutaneous T cell dyscrasias.

Gamma delta TCL would be another consideration given the clinical picture is alarming. Otherwise agree with Anil.

I don’t see any fat hyalinization or plasma cells, as expected in lupus panniculitis, so favor lymphoma...but lymphocytic rimming around adipocytes isn’t enough prominent. And I’m afraid there is some hemophagocytic activity. Only 20% of SPTCL are associated with HLH. So favor SPTCL, but agree with Shawn: it need to rule out PCGDTCL.

CD8? BetaF1? CD56?

This was an interesting case. The condition started 1 year prior to presentation with slowly progressive appearance of subcutaneous indurated plaques over the lower limbs.  1 month prior to presentation, progressive appearance of new lesions started to occur associated with fever. Upon admission, investigations were done and reveled pancytopenia, and hepatosplenomegaly. Bone marrow biopsy revealed hemophagocytosis. Two skin biopsies revealed a process restricted only to subcutaneous fat with no dermal or subepidermal infiltrates. The immunophenotype was: CD3+, CD8+, CD56- and Ki-67 was high with rimming of fat cells with Ki-67+ cells. Unfortunately, we do not have BetaF1 or TCRgamma IHC but clonal rearrangement of the delta chain was negative. We diagnosed this case as SCPTCL associated with hemophagocytosis. SCPTCL usually runs an indolent course with good prognosis unless hemophagocytic syndrome develops. Thank you all! 

Thanks Mona for sharing this interesting case!