SPTCLSubcutaneous Panniculitis-Like T-Cell Lymphoma (rheumatology)
SPTCLSouth Park Township Community Library (Bethel Park, PA)
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Diagnosis of SPTCL is a challenge, especially in the early stages when the symptoms mimic other more common conditions such as benign panniculitis, eczema, dermatitis, cellulitis, and other skin and soft tissue infections.
Diagnosis of SPTCL is based on pathological examination of skin and subcutaneous tissue, clinical characteristics, immunohistochemistry staining patterns, and molecular analysis.
Lesions may show overlapping features with LP and early lesions may closely mimic lobular lymphocytic panniculitis, but whether benign lobular lymphocytic panniculitis precedes SPTCL in patients without systemic LE is unclear.
(10) While histiocytes are present in both LP and SPTCL, other inflammatory cells, particularly plasma cells (which are commonly seen in LP), are absent.
Distinguishing SPTCL from cutaneous [delta][gamma] T-cell lymphoma is important, as the latter has a much poorer prognosis.
Given the overlap among LP, atypical lymphocytic lobular panniculitis, and SPTCL, some authors believe that these disorders represent a spectrum of subcuticular T-cell lymphoid dyscrasia.
The most important distinction is between LP and SPTCL. Cases of atypical lymphocytic lobular panniculitis with overlapping features between LP and SPTCL require long-term follow-up, as future subcutaneous lymphoma cannot be ruled out.
Ulceration of nodules in SPTCL is rare, and only 6% of the cases may show ulceration at some stage of the clinical course.
Hemophagocytic syndrome is present in both TCR[alpha][beta] SPTCL (17%) and TCR[gamma][delta] phenotype (45%) cases, but the disease is more aggressive in the TCR[gamma][delta] phenotype.
The diagnosis of SPTCL is based on the combination of clinical presentation, pathologic examination of skin/subcutaneous tissue, immunohistochemical staining pattern, and molecular analysis.
(15) In SPTCL, the cells that rim the fat spaces are CD[8.sup.-], and immunohistochemistry is very useful in identifying these cells (Figure 8, a and b).
(9) In benign panniculitis, in contrast to SPTCL, aggregates of CD[20.sup.+] B cells are present, mixed with CD[3.sup.+] cells that are both CD[4.sup.+] and CD[8.sup.-] in equal proportions and with a slight predominance of CD[4.sup.+] cells; cells that are TIA-[1.sup.+] are only occasionally seen.