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