Subcutaneous Panniculitis-Like T-Cell Lymphoma With Overlapping Clinicopathologic Features of Lupus Erythematosus: Coexistence of 2 Entities?

Abstract

We observed 5 patients with subcutaneous panniculitis-like T-cell lymphoma (SPTCL) who were unusual, in that they also exhibited features of lupus erythematosus (LE). This observation is in keeping with a recent study that reported an increased rate of autoimmune disease, including systemic lupus erythematosus (SLE), among patients with SPTCL. In all cases, attributes indicating SPTCL included an infiltrate of lymphocytes with pleomorphic nuclei involving subcutaneous lobules exhibiting a cytotoxic T-cell (CD3/CD8/betaF1) immunophenotype. Additionally, a high proliferation rate and a monoclonal T-cell receptor-gamma gene rearrangement were observed in most cases. The manifestations of LE in these patients included a spectrum of clinical and histopathological abnormalities. Clinical manifestations of LE included, in some patients, morphologic evidence of lupus erythematosus panniculitis (LEP) with subcutaneous nodules that healed with lipoatrophy on the face. In addition, all the patients exhibited serologic and/or extracutaneous end-organ abnormalities seen in patients with SLE, with 2 patients having sufficient findings to meet American College of Rheumatology criteria for SLE. Histopathological evidence of LE included vacuolar change at the dermal-epidermal interface in 3 patients, 2 of whom also showed interstitial deposition of mucin in the reticular dermis. One of these patients also had findings of LEP in the subcutaneous lobules with clusters of CD20 B cells partially arranged within germinal centers. In 2 patients in which neither the epidermis nor dermis was available for review, histopathological features of LE included, in one patient, a few small clusters of CD123 plasmacytoid dendritic cells within the adipose tissue and, in the other patient, a positive direct immunofluorescence test (lupus band) on clinically uninvolved and lesional skin. Our study shows that some patients show overlap between SPTCL and LE. We suspect that these patients may suffer from both diseases concomitantly. Furthermore, patients with LE, particularly LEP, should be monitored for evolution into SPTCL with biopsy of any subcutaneous lesion that is not typical of LEP. Additionally, screening for cutaneous LE and SLE could be considered in patients with SPTCL.

Pathologic Diagnosis of Cutaneous Lymphomas

“…In both entities, plasma cells can be observed. 51,52 Useful histopathological criteria for distinguishing lupus panniculitis from SPLTCL include epidermal changes, lymphoid follicles with reactive germinal centers, clusters of B cells, and mixed cell infiltrate with prominent plasma cells in lupus panniculitis. 53 In addition, clusters of CD123 1 plasmacytoid dendritic cells are found in lupus panniculitis.…”

Lymphome T sous-cutané à type de panniculite

“…Elles sont retrouvées dans 19 % des cas : lupus érythémateux le plus fré-quemment [15,29], mais aussi polyarthrite rhumatoïde [15,30], et beaucoup plus rarement syndrome de Sjögren, diabète de type 1, thrombocyté-mie idiopathique, sclérose en plaques, syndrome de Raynaud, maladie de Kikuchi [15] et sarcoïdose [31].…”

“…Dans un certain nombre de cas, le diagnostic de panniculite lupique (LP) a été évoqué avant celui de LPST [15,29,53,54]. La présentation clinique proche et des aspects histologiques communs font du LP un des principaux diagnostics diff érentiels du LTSP.…”

“…La présentation clinique proche et des aspects histologiques communs font du LP un des principaux diagnostics diff érentiels du LTSP. La diffi culté diagnostique est accrue par l'existence d'authentiques associations entre LES et LTSP [15,29] [29]. Il n'est pas établi que les LP puissent progresser vers un LTSP, mais l'existence d'association entre ces deux entités et d'éléments de chevauchement entre LP et LTSP suggère que le diagnostic de LTSP soit envisagé devant un LP et inversement qu'un LE soit recherché chez les patients atteints de LTSP.…”

Lymphoproliferative Disorders in Patients with Systemic Lupus Erythematosus

“…These subtypes include lymphoplasmacytic lymphoma (Papadaki et al, 2003), intravascular lymphoma (Sanchez-Cano et al, 2007), Franklin´s disease (García-Muñoz et al, 2008. ), subcutaneous panniculitis-like T cell lymphoma (Pincus et al, 2009. ), ALKnegative T cell anaplastic large cell lymphoma (Suvajdizc et al,2003), peripheral T cell lymphoma (Löfström et al, 2007) and T cell leukemia/lymphoma (Frisch Stork et al,2009).…”