Current Issues & Opinion
Defining early mycosis fungoides

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This editorial review summarizes the results of 5 meetings sponsored by the International Society for Cutaneous Lymphoma at which the clinicopathologic and ancillary features of early mycosis fungoides were critically examined. Based on this analysis, an algorithm was developed for the diagnosis of early mycosis fungoides involving a holistic integration of clinical, histopathologic, immunopathologic, and molecular biological characteristics. A novel aspect of this algorithm is that it relies on multiple types of criteria rather than just one, for example, histopathology. Before its finalization, the proposed diagnostic algorithm will require validation and possibly further refinement at multiple centers during the next several years. It is anticipated that a more standardized approach to the diagnosis of early mycosis fungoides will have a beneficial impact on the epidemiology, prognostication, treatment, and analysis of clinical trials pertaining to this most common type of cutaneous lymphoma.

Section snippets

Relationship between MF and parapsoriasis en plaques: Historical perspective and current implications

Part of the difficulty in establishing threshold criteria for the diagnosis of early MF stems from the existence of established terminology for reputedly benign dermatoses, such as parapsoriasis en plaques (PEP), that have overlapping clinical and/or histologic features with early MF. By utilizing clinical findings only, PEP was defined by Brocq1 in 1902 as a chronic, recurrent, erythematous, and scaling dermatosis that was part of a larger spectrum of eczematoid, psoriasiform, or lichenoid

Early diagnosis of MF: The role of clinical assessment

The diagnosis of MF relies heavily on clinical assessment, particularly in providing a supportive history, confirming one of several typical or suspect clinical presentations of MF and directing the choice of the critical biopsy site(s). The clinical diagnosis of MF may be difficult to make in patch- or early plaque-phase disease because many of its clinical features may also be found in benign inflammatory diseases. However, certain findings are quite characteristic of early MF and, when

Early diagnosis of MF: The role of histopathology

The definition of histopathologic features to differentiate early MF from benign inflammatory diseases is by far the most difficult, debated, and yet crucial issue. To enhance the chance of establishing a histologic diagnosis of MF, multiple biopsies from a variety of lesions may be required, including the oldest, well-developed, most infiltrated lesions as well as the newest lesions. With the exception of emollients, it is important that all topical treatments, but especially topical

Early diagnosis of MF: The role of ancillary techniques

DNA cytophotometry,33, 34, 35, 36, 37 nuclear morphometry,38, 39, 40, 41 immunohistochemistry,42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57 chromosomal studies,58, 59, 60 and, more recently, molecular genetic analysis of T-cell clonality8, 55, 56, 57, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76, 77, 78 have generated a significant amount of data that suggest their utility not only in the early diagnosis of MF but also potentially in the clinical management of

Diagnostic algorithm: An ISCL proposal

Based on the literature reviewed herein, the proceedings of the ISCL meetings in Zurich, Napa, Bethesda, New Orleans, and Washington, DC, as well as our aggregate experience evaluating early MF, we have proposed an algorithm for the diagnosis of early “classic” MF that incorporates clinical, histopathologic, molecular biologic, and immunopathologic features (Table I). The diagnosis of early MF requires a total of 4 points. Because molecular biologic and immunopathologic criteria represent no

Conclusions

It is hoped that this proposed algorithm will prove useful in the standardization of the diagnosis of early MF. Having such standardization is critical to predicting and tracking prognosis, designing clinical trials, and implementing stage-appropriate treatment. The ISCL has developed this algorithm only as a first step toward establishing firm diagnostic criteria for classic early MF (Table I). The next step will be multicenter testing of this algorithm in order to identify areas of weakness,

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  • Cited by (0)

    Funding sources: Research funding provided by the Department of Veterans Affairs and National Institutes of Health grant AR-02136 (to G. S. W.).

    Conflicts of interest: None identified.

    Parts of this work have been presented at international meetings.

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