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< Current issue
Dermatopathology: Practical & Conceptual July - September 2003
>
Alopecia Mucinosa is Mycosis Fungoides
Almut Böer, M.D.
Ying Guo, M.D.
A .Bernard Ackerman, M.D.
Abstract
Contents
Quotations from Contemporary Sources
Conventional Terminology
Historical perspective
Our observations
Pitfall in diagnosis histopathologically of mycosis fungoides with epithelial mucinosis
Our concept of alopecia mucinosa
Conclusion
Acknowledgements
References
SEE ALSO
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alopecia mucinosa expression of mycosis fungoides
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mycosis fungoides
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Conclusion
In conclusion, we regard "alopecia mucinosa" to be mycosis fungoides from the outset, not a paraneoplasia, not a precursor, not an "abortive" lymphoma, and not a "comparatively benign" variant of lymphoma. It is that particular expression of mycosis fungoides morphologically typified clinically at first as patches punctuated by "follicular" papules and histopathologically by involvement prominently of infundibula, of sebaceous lobules, and of follicles by lymphocytes disposed as solitary units, the effects of those neoplastic cells resulting in infundibular, sebaceous, and follicular mucinosis. Subtle, and not so subtle, changes in surface epidermis are typical of the patch stage of mycosis fungoides with epithelial mucinosis, but even in the absence of such changes, a diagnosis of mycosis fungoides with epithelial mucinosis can be made with confidence by virtue of findings detailed repeatedly in this work. Atypical lymphocytes and clonal rearrangement of T-cell receptors may be helpful in substantiating a diagnosis of mycosis fungoides with epithelial mucinosis but, in most instances, a diagnosis of mycosis fungoides of that particular type can be made with confidence without any aid other than hematoxylin and eosin.
The course of a patient with mycosis fungoides with epithelial mucinosis cannot be predicted accurately based on assessment either of features clinically or findings histopathologically, or of both of them together. In this regard, mycosis fungoides with epithelial mucinosis is no different from other manifestations of mycosis fungoides as they express themselves in patches and "early" plaques. Most patients with mycosis fungoides, including those with epithelial mucinosis, have only patches and plaques for a lifetime; they do not develop nodules and tumors and they do not die from the effects of their lymphoma. Nonetheless, mycosis fungoides, from the outset, is a systemic lymphoma and at the beginning of the process, no judgment of worth can be made about prognosis. How macules and patches will evolve is mere supposition and will remain that until much more is learned about the roles of genetics, the neoplastic lymphocytes themselves, and the host who combats them. What is not supposition to us is that understanding of this subject is enhanced by relinquishing the terms "follicular mucinosis" and "alopecia mucinosa" in favor of "epithelial mucinosis" and "mycosis fungoides with epithelial mucinosis," respectively.
In Figs.
38
through
44
that follow, we engage in clinico-pathologic correlation of mycosis fungoides with epithelial mucinosis, seeking to convey in the exercise the essential message of this work, namely, that so-called alopecia mucinosa, clinically and histopathologically, is mycosis fungoides.
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Figs. 38AH Mycosis fungoides with epithelial mucinosis. In the patient pictured in Fig. 38 A, a slightly raised erythematous plaque is present on the left cheek. Taken out of context, that lesion could pose a quandary in diagnosis clinically, one that can be resolved only by scrutiny of sections of tissue of a biopsy specimen taken from it. At scanning magnification (B), a moderately dense infiltrate of lymphocytes is observed to be present in infundibular epidermis and in sebaceous lobules, both of which are bloated by deposits of mucin. At high magnification, the lymphocytes are seen better (F) being disposed also as solitary units in the lower part of the epidermis, which is virtually devoid of spongiosis (H). When the clinical lesion is revisited in the context of the histopathologic findings, it becomes obvious that the features are consonant with those of mycosis fungoides.
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Figs. 39AG Mycosis fungoides with epithelial mucinosis. At different anatomic sites, the patient pictured in Fig. 39 A has several slightly erythematous patches punctuated by papules that are equidistant from one another, a phenomenon indicative of infundibulocentricity. Even at scanning magnification (B), a superficial and deep perivascular and peri-infundibular infiltrate of lymphocytes is as apparent as is plentiful mucin in infundibular epithelium. At higher magnification (C and D), lymphocytes are recognizable as solitary units in infundibular and surface epidermis (E), in the latter site in the near absence of spongiosis.
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Figs. 40AG Mycosis fungoides with epithelial mucinosis. The patient shown in Fig. 40 A presented herself clinically with alopecia devoid of inflammation. On the trunk were many nummular lesions (B), most of which were elevated slightly, and some of which were studded with papules, whereas others displayed comedo-like plugs. The papules and the comedo-like structures are equidistant from one another, from which can be drawn the inference that each of them is infundibulocentric. A moderately dense infiltrate of lymphocytes envelops infundibula and follicles, and surrounds venules of the superficial plexus (C). Infundibular epidermis houses deposits of mucin (E). Both surface and infundibular epidermis are pierced by solitary lymphocytes in league with paltry spongiosis (F).
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Figs. 41AE Mycosis fungoides with epithelial mucinosis. A second specimen taken from the same patient pictured in Fig. 40A and B shows, at scanning magnification, a moderately dense infiltrate in the immediate vicinity of infundibula and follicles especially. Infundibula are dilated and plugged by orthokeratotic cells. At higher magnifications, mucin is seen to accompany lymphocytes in infundibular epidermis and in follicular epithelium. Only uncommonly in mycosis fungoides with epithelial mucinosis is involvement of the stem and the bulb encountered, but it is mucin in the bulb that accounts for inability of matrical cells to generate a hair, which, in turn, is responsible for the appearance clinically of alopecia, from whence derived the name given to it by H. Pinkus, to wit, alopecia mucinosa.
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Figs. 42AE Mycosis fungoides with epithelial mucinosis. This patient presented herself with alopecia in a manner reminiscent of that of alopecia areata (A and B). Some alopecic patches, however, were slightly erythematous and scaly, indications that this condition is not truly alopecia areata. At scanning magnification (C), mucin is observed in infundibular epidermis, which is showered with lymphocytes (E). Lymphocytes arrayed as solitary units also are found within surface epidermis (not pictured here). Although one hesitates to make a diagnosis clinically of mycosis fungoides in a patient whose only sign is alopecia, the findings histopathologically are those of mycosis fungoides with epithelial mucinosis.
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Figs. 43AI Mycosis fungoides with epithelial mucinosis. This patient presented himself with skin colored, slightly scaly patches on the trunk, the forehead, and the scalp. Lesions on the scalp were alopecic and studded in infundibulocentric arrangement with tiny keratotic papules. At scanning magnification, a moderately dense infiltrate is observable around venules of both plexuses, around infundibula, and around follicles, as well as in patchy lichenoid fashion in company with wiry bundles of collagen arranged haphazardly. An infundibulum sports abundant mucin. A dense infiltrate of lymphocytes joined by a few eosinophils is present in follicular epithelium. Within surface epidermis, lymphocytes are disposed as solitary units and in small collections, and there spongiosis is slight.
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Figs. 44AG Mycosis fungoides with epithelial mucinosis and involvement of eccrine ducts. This patient has numerous red patches and plaques on the face and neck (A). Sections from a biopsy specimen obtained from one of those lesions shows, at scanning magnification (B), a lake of mucin in an infundibulum swollen enormously by it, the epithelium also containing many lymphocytes (D). The epidermis is psoriasiform and houses many lymphocytes disposed as solitary units in conjunction with hardly any spongiosis (E). Eccrine ducts are involved in the process, too (F), lymphocytes being situated within and around hyperplastic ductal epithelium (G). In short, the lymphomatous process affects all of the epithelial structures pictured in this section.
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