REVIEW ARTICLE DOI 10.1111/j.1365-2133.2006.07526.x Mycosis fungoides: a dermatological masquerader D. Nashan, D. Faulhaber,* S. Sta¨nder,* T.A. Luger* and R. Stadler Department of Dermatology, University of Freiburg, Hautstr. 7, 79104 Freiburg, Germany *Department of Dermatology, University of Mu ¨nster, Mu ¨nster, Germany Department of Dermatology, Klinikum Minden, Minden, Germany Correspondence D. Nashan. E-mail: [email protected] Accepted for publication 8 June 2006 Key words clinical subtypes, differential diagnoses, mycosis fungoides, overview Conflicts of interest None declared. Summary Mycosis fungoides (MF), a low-grade lymphoproliferative disorder, is the most common type of cutaneous T-cell lymphoma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumours or erythroderma. Diagnosis of MF can be difficult due to highly variable presentations and the sometimes non- specific nature of histological findings. Molecular biology has improved the diag- nostic accuracy. Nevertheless, clinical experience is of substantial importance as MF can resemble a wide variety of skin diseases. We performed a literature review and found that MF can mimic >50 different clinical entities. We present a structured framework of clinical variations of classical, unusual and distinct forms of MF. Distinct subforms such as ichthyotic MF, adnexotropic (including syringotropic and folliculotropic) MF, MF with follicular mucinosis, granuloma- tous MF with granulomatous slack skin and papuloerythroderma of Ofuji are delineated in more detail. Mycosis fungoides (MF), a low-grade lymphoproliferative dis- order, is the most common type of cutaneous T-cell lymph- oma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumours or erythroderma. Diagnosis of MF can be difficult due to highly variable presentations and the sometimes nonspecific nature of histological findings. Molecular biology has improved the diagnostic accuracy. Nev- ertheless, clinical experience is of substantial importance as MF can resemble a wide variety of skin diseases. Diagnosis of MF is based on a combination of clinical pre- sentation, histopathology and gene rearrangement. 1 None of these factors exclusively determines the diagnosis. Histologi- cally, MF is characterized by the presence of large atypical lymphocytes, a lymphocytic infiltrate in the papillary dermis and thickened collagen fibres. However, in early MF not all of these pathological findings are present and distinction from an inflammatory infiltrate is often difficult. 2 Detection of a mono- clonal T-cell infiltrate is not lymphoma specific. Positive poly- merase chain reaction (PCR) results are also found in diseases such as psoriasis, pityriasis lichenoides et varioliformis acuta (PLEVA) and lichen ruber. 3,4 Thus clinical presentation is a major factor determining the diagnosis. 5 Search criteria This article emerged from Medline searches, manual searches in dermatological journals and textbooks, and from personal experiences. Electronic key word searches included ‘mycosis fungoides’ with ‘differential diagnosis’ and ‘clinical picture’, and ‘mycosis fungoides’ and ‘cutaneous T-cell lymphoma’ in conjunction with clinically descriptive adjectives. From extrac- ted articles the related articles and publishing authors were also screened. Further original articles were extracted from ref- erence lists. We thereby present a review of all currently published clin- ical pictures of MF simulating other dermatoses and distinctive clinicopathological features which are in part considered sep- arately in the new World Health Organization (WHO)—Euro- pean Organization for Research and Treatment of Cancer (EORTC) classification. 6 The illustration of MF variants follows in Tables according to clinical signs and under the headings of more distinct subtypes. Clinically and morphologically unusual variations of mycosis fungoides In early stages of MF (T1N0M0 or T2N0M0) characteristic le- sions consist of erythematous macules or papules, which are primarily superficial and resemble an ‘eczema’ with sharply defined borders. Often some degree of scaling is observed, similar to psoriasis. The edges of the lesions might exhibit in- creased scaling, corresponding to a growing infiltrate. The configuration can be arciform, annular, semiannular, serpingi- nous or polycyclic. 7 The skin surface can be atrophic, exhibit- ing wrinkles. The colour can be orange to bright red or can present livid or brown-red components. Spontaneous Ó 2006 British Association of Dermatologists • British Journal of Dermatology 2007 156, pp1–10 1
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