On the diagnosis of erythrodermic cutaneous T‐cell lymphoma

Abstract

Erythrodermic cutaneous T-cell lymphoma (E-CTCL) is the cause of less than 5% of all cases of generalized erythroderma. A methodical evaluation of skin, blood, and lymph node samples using standard histology, immunohistochemistry (IHC), flow cytometry (FC), and molecular analysis for evidence of a dominant T-cell clone has been recommended in a recently published diagnostic algorithm. In this commentary, the author discusses available information regarding the role of these diagnostic methods for the diagnosis of E-CTCL with emphasis on personal observations regarding skin IHC and polymerase chain reaction (PCR)-based molecular studies as adjunct diagnostic studies on a series of 55 patients with erythrodermic mycosis fungoides and 50 patients with Sézary syndrome compared to 50 patients with extensive benign inflammatory skin disease. The conclusions are (1) IHC of the skin does not reliably differentiate E-CTCL from benign simulants, (2) presence of phenotypically abnormal T cells in the blood or expanded subsets of CD4+CD7- or CD4+CD26- cells by FC is particularly helpful as a diagnostic study, (3) the presence of an identical T-cell clone in the skin and blood also is a specific diagnostic criterion for E-CTCL, but exceptions may occur, and (4) the PCRgamma-denaturing gradient gel electrophoresis technique appears to be more reliable than PCRgamma-single-stranded conformational polymorphism for diagnostic purposes.

Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)

“…[13][14][15][16] However, a definitive histopathologic diagnosis by light microscopy alone may be difficult to make in early MF 17,18 or in erythroderma in which inflammatory cells often predominate. 19 The ISCL recently proposed a diagnostic algorithm for early MF (Table 3). 15 No patient with clinically suspect patch-or plaque-stage disease who does not at least fulfill this algorithm nor any patient with tumor-stage disease with histologic findings only "suggestive of MF" should be entered into MF/SS databases or into therapeutic trials for MF/SS.…”

“…In the case of erythrodermic CTCL, multiple skin biopsies may be necessary to establish a firm diagnosis or a definitive diagnosis may be made by blood studies and/or by biopsy of an enlarged lymph node or other tissue. 19,20 For patients presenting with tumors, it is important to differentiate tumor-stage MF from non-MF subtypes of CTCL. In classic MF, the tumor lesions generally develop in the presence of patch or plaque disease and not de novo.…”

A Rare Presentation of Cutaneous Pseudolymphoma as a Preleukaemic State

“…The commonest cause of erythroderma was due to pre-existing inflammatory skin disease with an incidence between 27-68% in various case series [1]. Malignancies were the least common cause of erythroderma in these series, with cutaneous T-cell lymphoma (CTCL) as the most frequent malignant cause of erythroderma with a median incidence of 3% (range, 0-18%) [1].…”

Cutaneous lymphomas: A review

“…Patients may complain of intense pruritus, weight loss, fevers, and chills. These clinical findings are nonspecific, and they are also found in varying frequencies in erythrodermic patients with different etiologies, highlighting the importance of extensive laboratory research seeking to determine the cutaneous and blood involvement of neoplastic cells in erythrodermic patients [44][45][46] . The prognosis of SS is poor, with a median survival of four years after diagnosis (or 42.3% at five years) 45 .…”