Abstract
Mycosis fungoides is the most common form of cutaneous T-cell lymphoma. In the western population there are around 0.3 cases of Sezary syndrome per 100,000 people. Sézary disease is more common in males with a ratio of 2:1, and the mean age of diagnosis is between 55 and 60 years of age.
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Mycosis fungoides is the most common form of cutaneous T-cell lymphoma. In the western population there are around 0.3 cases of Sezary syndrome per 100,000 people. Sézary disease is more common in males with a ratio of 2:1, and the mean age of diagnosis is between 55 and 60 years of age.
Clinical Description
The curious name “mycosis fungoides” refers to the initial clinical description of mushroom-like tumors evolving from a desquamating rash. Four clinical stages are seen:
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1.
Patch stage-persistent, pruritic, red, pink, or brown patches, with or without scale
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2.
Plaque stage-persistent, pruritic, red, pink, or brown plaques
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3.
Tumor stage-persistent red, brown, or violet papules, nodules, and/or tumors (d’ emblee type refers to sudden appearance of tumors without previous patches or plaques)
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4.
Sezary syndrome-exfoliative erythroderma with numerous, bloodborne Sezary cells (convoluted T lymphocytes) [1–6]
Etiology
The cause of mycosis fungoides is still unknown.
Theories include chronic, low-grade contact dermatitis and/or retrovirus (HIV III, HTL V I) infection. (HIV III, also called HTLV I, is different from the AIDS virus, which is HlV I, and is also called HTLV III). The result is a malignant clone of helper T cells [7–10].
Histopathology
Cutaneous T-cell lymphoma shows a lichenoid (band-like) lymphocytic infiltrate with Pautrier microabscesses consisting of collections of atypical cerebriform or hyperconvoluted T lymphocytes in the epidermis, with no or at most minimal spongiosis and a mixed lymphohistiocytic perivascular dermal infiltrate, with variable eosinophils and plasma cells. The lack of spongiosis is one clue to distinguishing cutaneous T-cell lymphoma from eczematous diseases.
Differential Diagnosis
The differential diagnosis includes the following:
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1.
Patch stage-eczema, tinea, pityriasis rosea, pityriasis lichenoides chronica, secondary syphilis, other papulosquamous disorders
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2.
Plaque stage-psoriasis, parapsoriasis en plaques (large plaque parapsoriasis may represent a precursor lesion to mycosis fungoides)
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3.
Tumor stage-squamous cell carcinoma (usually single, not multiple), other lymphomas (cutaneous nodules of Hodgkin’s disease and leukemic infiltrates), postscabetic nodules, Kaposi’s sarcoma
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4.
Sezary syndrome-other causes of exfoliative erythroderma, including psoriasis, generalized eczema, drug eruptions, tinea, erythema multiforme (toxic epidermal necrolysis)
Therapy
Treatment includes the following:
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1.
Electron beam therapy, orthovoltage radiotherapy
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2.
Topical chemotherapy-topical nitrogen mustard (mechlorethamine), topical carmustine (BCNU, bischloroethyl nitrosurea)
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3.
Systemic chemotherapy-methotrexate, cyclosporine
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4.
Photochemotherapy–PUVA (psoralen plus UV A light)
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5.
Extracorporeal photochemotherapy-plasmapheresis and PUVA
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6.
Vorinostat is a second-line drug for cutaneous T-cell lymphoma. Treatments are often used in combination with phototherapy and chemotherapy
No single treatment type has revealed clear-cut benefits in comparison to others, and treatment for all cases remains problematic.
Prognosis
The prognosis is poor, with a gradual but inexorable progression from patch to plaque to tumor stage, which may take over 20 years. Patients often succumb to other diseases, infections, or complications of therapy for mycosis fungoides. Patients with Sézary disease have a median survival of 5 years.
References
Burg G, Kempf W, Cozzio A, et al. WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects. J Cutan Pathol. 2005;32(10):647–74.
Burg G, Jaffe ES. WHO/EORTC classification of cutaneous lymphomas. In: LeBoit P, Burg G, Weedon D, Sarasin A, editors. Tumors of the skin. 10th ed. Lyon: WHO Books; 2006. p. 166–8.
Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105(10):3768–85.
Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: IARC Press; 2008.
Olsen E, Vonderheid E, Pimpinelli N, et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110(6):1713–22.
Pimpinelli N, Olsen EA, Santucci M, et al. Defining early mycosis fungoides. J Am Acad Dermatol. 2005;53(6):1053–63.
Abrams JT, Balin BJ, Vonderheid EC. Association between Sezary T cell-activating factor, Chlamydia pneumoniae, and cutaneous T cell lymphoma. Ann N Y Acad Sci. 2001;941:69–85.
Boni R, Davis-Daneshfar A, Burg G, Fuchs D, Wood GS. No detection of HTLV-I proviral DNA in lesional skin biopsies from Swiss and German patients with cutaneous T-cell lymphoma. Br J Dermatol. 1996;134:282–4.
Imai S, Burg G, Braun-Falco O. Mycosis fungoides and Sezary’s syndrome show distinct histomorphological features. Dermatologica. 1986;173(3):131–5.
Kamarashev J, Burg G, Kempf W, Hess Schmid M, Dummer R. Comparative analysis of histological and immunohistological features in mycosis fungoides and Sézary syndrome. J Cutan Pathol. 1998;25(8):407–12.
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Norman, R.A., Young, E.M. (2014). Mycosis Fungoides (Cutaneous T-Cell Lymphoma). In: Atlas of Geriatric Dermatology. Springer, London. https://doi.org/10.1007/978-1-4471-4579-0_47
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DOI: https://doi.org/10.1007/978-1-4471-4579-0_47
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