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Dermatopathology: Practical & Conceptual October - December 2003
New Heights: “Hypopigmented mycosis fungoides” is not always mycosis fungoides!
Betina Werner, M.D.
Sonya Brown, M.D.
A. Bernard Ackerman, M.D.
The issue of hypopigmentation in mycosis fungoides
Our method for attempting to assess authenticity of a hypopigmented expression of mycosis fungoides
Definition of mycosis fungoides and criteria morphologically (clinical and histopathologic) for diagnosis of it
“Hypopigmented mycosis fungoides” in historical perspective
Our conclusions about “hypopigmented mycosis fungoides”
Summary of attributes of the patients who surely had “hypopigmented mycosis fungoides”
Photographs of clinical lesions and photomicrographs of the 19 patients with unquestionable hypopigmented mycosis fungoides
Summary of attributes of the patients who did not have “hypopigmented mycosis fungoides”
Photographs of clinical lesions and photomicrographs of the four patients who did not have hypopigmented mycosis fungoides:
Reasons we were unable to make a specific diagnosis of mycosis fungoides in some patients reputed to have it
Conclusions in the form of five questions and our own answers to them
How to differentiate, clinically and histopathologically, pityriasis alba and vitiligo from hypopigmented mycosis fungoides
A patient with stereotypical mycosis fungoides associated with hypopigmentation
A patient with stereotypical mycosis fungoides associated with both hypo- and hyperpigmentation
The subject under discussion here ostensibly is "hypopigmented mycosis fungoides," but our concerns transcend that matter. First, the term "hypopigmented" mycosis fungoides is just one more variation on an old theme (first sounded in 1806 when Alibert described mycosis fungoides unidimensionally, requiring as he did the presence of tumors for diagnosis of it), namely, still another morphologic manifestation of mycosis fungoides, in this case it being that lymphoma as if presents itself as hypopigmentation in patients with dark skin. The reasons for hypopigmentation in lesions of mycosis fungoides is not known, but it is not analogous to "post-inflammatory hypopigmentation." The hypopigmented expression of mycosis fungoides displays the same fundamental findings histopathologically as do all other patch/subtle plaque variants of mycosis fungoides (large and small plaque parapsoriasis) and portends prognosis no worse than any of them. The benefit of calling attention of clinicians to mycosis fungoides that manifests itself as hypopigmented patches and plaques pertains to enabling differentiation of it, clinically and histopathologically, from pityriasis alba and from vitiligo. To establish a special category of mycosis fungoides based entirely on hypopigmentation, however, would be as inefficacious as constructing one predicated solely on hyperpigmentation. In actuality, there are few reports of hyperpigmentation as a consequence of mycosis fungoides,
but no effort has been made to create a separate niche for it, i.e., "hyperpigmented mycosis fungoides" (analogous to "hypopigmented mycosis fungoides"). In short, irrespective of whether lesions are hypopigmented or hyperpigmented, the diagnosis, morphologically, is mycosis fungoides and no modification further is necessary.
Last, we are struck by how difficult was the struggle for us to come to a decision about whether or not a particular patient reported on did or did not have hypopigmented mycosis fungoides, the reason for that inability, so frustratingly, being the ineptness of the authors of the articles, the forfeiture of responsibility of reviewers of those articles, and the irresponsibility of editors of the journals in which those articles were published. All failed readers by serving up fare that was neither palatable nor edible. If a serious student of a subject cannot make "heads or tails" of a particular published work because of egregious flaws in it, such as no pictures being presented or clinical photographs and photomicrographs that defy interpretation because of impediments in them technically, what is the point of having forged that work in the first place and having it disseminated in the second? The matter is not merely one of professionalism and of competence; it is one of social conscience or, better put, the absence of it. For an author, a reviewer, and an editor to throw slop at a colleague-reader is telling; not only is there lack of caring about the quality of a professional life, there is unabashed contempt for colleagues. That situation is untenable and unacceptable, and that is among the most important messages we seek to convey through this work about hypopigmented mycosis fungoides our hope being to jolt socially conscionable colleagues to act to rectify it.
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