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Pooya Khan Mohammad Beigi - Clinicians Guide to Mycosis Fungoides

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Clinicians Guide to Mycosis Fungoides
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Pooya Khan Mohammad Beigi
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Springer International Publishing, Cham
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2017
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Part I
Overview of Disorder

Springer International Publishing AG 2017

Pooya Khan Mohammad Beigi Clinician's Guide to Mycosis Fungoides 10.1007/978-3-319-47907-1_1

1. Introduction and History

Pooya Khan Mohammad Beigi 1, 2, 3

(1)

Department of Health Management, New York Medical College, Valhalla, New York, USA

(2)

University of British Columbia, Vancouver, British Columbia, Canada

(3)

Misdiagnosis Association, Seattle, Washington, USA

Keywords

Mycosis fungoides History Skin Lymphoma Alibert-Bazin disease Patch Plaque Tumor

Mycosis fungoides (MF) is a mature non-Hodgkin lymphoma of T cell origin, and a type of cutaneous T cell lymphoma (CTCL). MF was first recognized in 1806 by a French dermatologist, Jean-Louis-Marc Alibert at the Lhpital St. Louis in France [].

In 1876, MF became known as Alibert-Bazin disease , named after Alibert and Pierre-Antoine-Ernest Bazin who contributed toward an early comprehensive characterization of the disease []. Taken together, these descriptions formed the early characterizations of MF, which were later followed by descriptions of Szarys syndrome (SS), the leukemic variant of CTCL.

Today we know that CTCL comprise a diverse group of non-Hodgkin lymphomas (NHL) caused by malignant skin-trafficking T cells. MF is the most common type of CTCL. Primary cutaneous lymphomas are classified by the European Organization for Research and Treatment of Cancer (EORTC) and the World Health Organization (WHO) (Table ).

Table 1.1

Frequency of disease and 5-year survival rate obtained from the European Organization for Research and Treatment of Cancer (EORTC) and the World Health Organization (WHO)

Disease classification	Frequency (%)	5-Year survival rate (%)
Mycosis fungoides (MF)
Folliculotropic MF
Pagetoid reticulosis
Granulomatous slack skin	<1
Primary cutaneous anaplastic large cell lymphoma (C-ALCL)
Lymphomatoid papulosis (LyP)
Szary syndrome (SS)
Extranodal NK/T-cell lymphoma, nasal type		<5

MF is a mature T cell NHL that primarily develops in the skin, but can also involve lymph nodes, blood, and other organs. Clinically, MF typically begins as variably sized, pruritic, erythematous patches with a fine scale []. Patients with MF typically have a prolonged clinical course.

Early lesions of MF may show variable degrees of epidermal atrophy, telangiectasia, and/or mottled hyper- or hypopigmentation. The term poikoderma refers to a combination of these features, the presence of which is highly suggestive of MF. These initial skin lesions have a tendency to asymmetrically affect the buttocks, trunks, and limbs. Patients often have nonspecific dermatitis and/or psoriasiform skin lesions for many years that are frequently misdiagnosed as atopic, contact, nummular, or photo-induced dermatitis (see Chap. Diagnosis and Differential Diagnosis section). For these reasons and more, diagnosing early MF proves to be challenging.

Contributors to This Chapter

Pooya Khan Mohammad Beigi, MD, University of British Columbia, BC, Canada
Elizabeth Alice Wang, BS, University of California Davis, Sacramento, CA, USA
Seyed Sajad Niyyati, BS, University of British Columbia, Vancouver, BC, Canada
Emanual Maverakis, MD, University of California Davis, Sacramento, CA, USA

References

Karamanou M, Psaltopoulou T, Tsoucalas G, Androutsos G. Baron Jean-Louis Alibert (1768-1837) and the first description of mycosis fungoides. J BUON. 2014;19(2):585. PubMed

Wiernik PH. Neoplastic diseases of the blood. Cambridge: Cambridge University Press; 2003.

Vonderheid EC, Bernengo MG, Burg G, Duvic M, Heald P, Laroche L, et al. Update on erythrodermic cutaneous t-cell lymphoma: Report of the international society for cutaneous lymphomas. J Am Acad Dermatol. 2002;46(1):95106. CrossRef PubMed

Besnier E, Hallopeau H. On the erythrodermia of mycosis fungoides. J Cutan Genitourin Dis. 1892;10:453.

Ashton R, Leppard B. Differential diagnosis in dermatology. Oxford: Radcliffe; 2005.

Springer International Publishing AG 2017

Pooya Khan Mohammad Beigi Clinician's Guide to Mycosis Fungoides 10.1007/978-3-319-47907-1_2

2. Epidemiology

Pooya Khan Mohammad Beigi 1, 2, 3

(1)

Department of Health Management, New York Medical College, Valhalla, New York, USA

(2)

University of British Columbia, Vancouver, British Columbia, Canada

(3)

Misdiagnosis Association, Seattle, Washington, USA

Keywords

Epidemiology Age Ethnicity Sex Income Education

The overall age-adjusted incidence of mycosis fungoides (MF) worldwide is around 67 cases per 1 million [].

In the United States, the incidence of MF, up until the year 2000, was reported to have increased over time [].

Contributors to This Chapter

Pooya Khan Mohammad Beigi, MD, University of British Columbia, BC, Canada
Elizabeth Alice Wang, BS, University of California Davis, Sacramento, CA, USA
Seyed Sajad Niyyati, BS, University of British Columbia, Vancouver, BC, Canada
Emanual Maverakis, MD, University of California Davis, Sacramento, CA, USA

References

Hall JC, Hall BJ. Cutaneous lymphoma: diagnosis and treatment. Shelton: PMPH; 2012.

Fatemi Nf, Najafian J, Salehi M, Azimi Z, Nilforoushzadeh Ma, Rajabi P. Mycosis fungoides: epidemiology in Isfahan, Iran; 2014.

Crowley JJ, Nikko A, Varghese A, Hoppe RT, Kim YH. Mycosis fungoides in young patients: clinical characteristics and outcome. J Am Acad Dermatol. 1998;38(5):696701. CrossRef PubMed

Kim ST, Sim HJ, Jeon YS, Lee JW, Roh HJ, Choi SY, et al. Clinicopathological features and t-cell receptor gene rearrangement findings of mycosis fungoides in patients younger than age 20 years. J Dermatol. 2009;36(7):392402. CrossRef PubMed

Weinstock MA, Horm JW. Mycosis fungoides in the United States: increasing incidence and descriptive epidemiology. JAMA. 1988;260(1):426. CrossRef PubMed

Agnarsson BA, Kadin ME, editors. Peripheral t-cell lymphomas in children. Seminars in diagnostic pathology; 1995.

Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105(10):376885. CrossRef PubMed

Morales Suarez-Varela MM, Llopis Gonzalez A, Marquina Vila A, Bell J. Mycosis fungoides: review of epidemiological observations. Dermatology (Basel, Switzerland). 2000;201(1):218.

Korgavkar K, Xiong M, Weinstock M. Changing incidence trends of cutaneous T-cell lymphoma. JAMA Dermatol. 2013;149(11):12959. CrossRef PubMed

Springer International Publishing AG 2017

Pooya Khan Mohammad Beigi Clinician's Guide to Mycosis Fungoides 10.1007/978-3-319-47907-1_3

3. Etiology

Pooya Khan Mohammad Beigi 1, 2, 3

(1)

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