Mycosis Fungoides

Doorn, Remco van; Haselen, Christian W. van; Vader, Pieter; Geerts, ML; Heule, Freerk; Rie, Menno A. de; Steijlen, P.M.; Dekker, Sybren K.; Vloten, W. A. van; Willemze, Rein

doi:10.1001/archderm.136.4.504

Cited by 313 publications

(90 citation statements)

References 70 publications

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“…Reported cases of syringotropic MF, with only skin involvement, usually had a good prognosis. The 10-year survival rates was 83% to 97%, similar to that of the chronic forms of syringotropic MF13. Therefore, the stable course of Case 1 might be due to the syringeal involvement observed.…”

Section: Discussionsupporting

confidence: 59%

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Two Clinically Unusual Cases of Folliculotropic Mycosis Fungoides: One with and the Other without Syringotropism

et al. 2014

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Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, and it rarely exhibits predilection for hair follicle and eccrine gland infiltration. Here, we present 2 similar cases that display folliculotropism with varying amounts of follicular mucinosis, with and without syringotropism. The features observed in both cases were cystic, comedo-like, acneiform lesions; generalized involvement with loss of body hair; pruritus; and hidradenitis suppurativa-like lesions. Hypohidrosis as well as nail and palmoplantar involvement with lichen planopilaris-like clinical features were unique characteristics of the first case. Despite the well-known aggressive behavior of follicular mycosis fungoides, the presented cases had a subtle, slowly progressive, but persistent, clinical course. Folliculotropic and syringotropic mycosis fungoides are variants of cutaneous T-cell lymphoma. Clinical presentations might be challenging, and multiple, deep biopsy specimens containing adnexal structures are required for this critical diagnosis. Aggressive treatment may not be necessary in cases having an indolent course, especially in those with syringotropism.

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Section: Discussionsupporting

confidence: 59%

“…Generalized hypohidrosis is not a commonly reported feature of MF in any form; focal anhydrotic patches have been defined in syringotropic MF cases10,11,12,13. The prominent syringeal involvement observed may alter eccrine gland function, and it might be the reason for the generalized hypohidrosis observed.…”

Section: Discussionmentioning

confidence: 98%

Two Clinically Unusual Cases of Folliculotropic Mycosis Fungoides: One with and the Other without Syringotropism

et al. 2014

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“…In these patients, definitive diagnosis is often difficult and the institution of definitive and appropriate therapy is delayed, often until disease worsens to the point where the diagnosis is clear but effective treatment is more difficult. On average, it takes six years after the initial onset of skin lesions to make a definitive diagnosis of CTCL ( 7 ).…”

Section: Discussionmentioning

confidence: 99%

“…The most commonly used clinical test, multiplex/heteroduplex PCR amplification of the TCR Vγ chain followed by GeneScan capillary electrophoresis analysis, detects clones in a subset of patients with CTCL but has a significant false negative rate ( 5, 6 ). Definitive diagnosis of MF is often delayed and is made on average six years after the first development of skin lesions ( 7 ). A more reliable method of discriminating between CTCL and benign inflammatory skin disease would both facilitate timely diagnosis of the disease and help to discriminate CTCL recurrences from unrelated benign inflammatory reactions in the skin.…”

Section: Introductionmentioning

confidence: 99%

TCR sequencing facilitates diagnosis and identifies mature T cells as the cell of origin in CTCL

et al. 2015

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Early diagnosis of CTCL is difficult and takes on average six years after presentation, in part because the clinical appearance and histopathology of CTCL can resemble that of benign inflammatory skin diseases. Detection of a malignant T cell clone is critical in making the diagnosis of CTCL but the TCRγ PCR analysis in current clinical use detect clones in only a subset of patients. High-throughput TCR sequencing (HTS) detected T cell clones in 46/46 CTCL patients, was more sensitive and specific than TCRγ PCR, and successfully discriminated CTCL from benign inflammatory diseases. HTS also accurately assessed responses to therapy and facilitated diagnosis of disease recurrence. In patients with new skin lesions and no involvement of blood by flow cytometry, HTS demonstrated hematogenous spread of small numbers of malignant T cells. Analysis of CTCL TCRγ genes demonstrated that CTCL is a malignancy derived from mature T cells. There was a maximal T cell density in skin in benign inflammatory diseases that was exceeded in CTCL, suggesting a niche of finite size may exist for benign T cells in skin. Lastly, immunostaining demonstrated that the malignant T cell clones in mycosis fungoides and leukemic CTCL localized to different anatomic compartments in the skin. In summary, HTS accurately diagnosed CTCL in all stages, discriminated CTCL from benign inflammatory skin diseases and provided insights into the cell of origin and location of malignant CTCL cells in skin.

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“…4,5,7,11,13 Most of our 525 patients had limited or generalized patch and/or plaque (T1-T2) disease. None of our patients with limited skin involvement (T1) presented with extracutaneous (stage IV) disease.…”

Section: Commentmentioning

confidence: 98%