Dermatology Book

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Mycosis Fungoides

Aka: Mycosis Fungoides, Cutaneous T-Cell Lymphoma, Sezary Syndrome, Sezary Cell, Sezary Erythroderma
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  1. Pathophysiology
    1. T-Cell Lymphoma that begins in skin
    2. Sezary Syndrome is leukemic form of T-Cell Lymphoma
  2. Epidemiology
    1. Incidence: 0.42 cases per 100,000 (rare)
    2. Most common after age 50 years
    3. Gender: Twice as common in men
    4. Black patients are more commonly affected
  3. Symptoms
    1. Pruritus
  4. Signs (four stages of development)
    1. Eczematous patches and Plaques
    2. Distributed over non-sun-exposed skin
      1. Buttocks
      2. Thighs
    3. Tumors develop later in course
      1. Associated with superficial and deep Lymphadenopathy
      2. Associated with metastases to Spleen, lung, GI tract
  5. Complications
    1. Exfoliative dermatitis
  6. Course
    1. Survival <3 years after tumors develop
  7. References
    1. Habif (1996) Clinical Dermatology, Mosby, p. 674-5
    2. Pujol (2000) J Am Acad Dermatol 42:324-8

Sezary Syndrome (C0036920)

Definition (NCI) A form of cutaneous T-cell lymphoma, a cancerous disease that affects the skin.
Definition (NCI) A generalized peripheral (mature) T-cell neoplasm characterized by the presence of erythroderma, lymphadenopathy, and neoplastic, cerebriform T-lymphocytes in the blood. Sezary syndrome is an aggressive disease. (WHO, 2001)
Definition (MSH) A form of cutaneous T-cell lymphoma manifested by generalized exfoliative ERYTHRODERMA; PRURITUS; peripheral lymphadenopathy, and abnormal hyperchromatic mononuclear (cerebriform) cells in the skin, LYMPH NODES, and peripheral blood (Sezary cells).
Concepts Neoplastic Process (T191)
MSH D012751
ICD9 202.2
ICD10 C84.1, M9701/3, C84.10
SnomedCT 4950009, 118611004, 188638002, 188629004
English Erythroderma, Sezary, Sezary Erythroderma, Sezary Syndrome, Syndrome, Sezary, RETICULOSIS SYNDROME, SEZARY, SEZARY SYNDROME, Sezary's disease NOS, Sezary's disease of unspecified site, Sezary's disease-unspec. site, Sezary's syndrome, [M]Sezary's disease, Sezary's disease (clinical), Sezary's disease (disorder), Sezary's disease (morphologic abnormality), Sezary's disease NOS (disorder), Sezary's disease of unspecified site (disorder), Sezary syndrome (diagnosis), Lymphoma, Sezary's, Sezarys Lymphoma, Sezary Lymphoma, Sezary's Lymphoma, Sezary disease, Sézary's disease of unspecified site (disorder), Sézary's disease NOS (disorder), Sézary disease, Sézary's disease of unspecified site, Sézary's disease NOS, Sézary disease, unspecified site, Sezary Syndrome [Disease/Finding], sezary syndrome, sezary's disease, sezary's syndrome, Sézary's disease (clinical), Sézary's disease (disorder), Sezary; reticulosis, Sezary, Sézary; reticulosis, Sézary, reticulosis; Sezary, reticulosis; Sézary, Sezary's Disease, Sezary's disease, Sezary syndrome, Sézary's disease (morphologic abnormality), Sézary's disease, [M]Sézary's disease, Sézary's syndrome
Spanish [M]enfermedad de Sézary, Enfermedad de Sezary, Eritroderma de Sezary, Eritroderma de Sézary, (M)enfermedad de Sézary, enfermedad de Sézary (anomalía morfológica), enfermedad de Sézary (clínica) (trastorno), enfermedad de Sézary (clínica), enfermedad de Sézary (trastorno), enfermedad de Sézary de localización no especificada (trastorno), enfermedad de Sézary de localización no especificada, enfermedad de Sézary, SAI (trastorno), enfermedad de Sézary, SAI, enfermedad de Sézary, síndrome de Sézary, Sindrome de Sezary, Síndrome de Sézary
Dutch Sézary-ziekte, Sézary; reticulose, reticulose; Sézary, Szary-syndroomd, Sézary-reticulose, Sézary-syndroom, Syndroom, Sézary-
French Maladie de Sezary, Réticulose de Sézary, Syndrome de Sézary, Érythrodermie de Sézary
German Sezary-Syndrom, Sezary-Krankheit, Erythrodermie, Sézary, Sézary-Syndrom
Italian Malattia di Sézary, Eritroderma di Sezary, Sindrome di Sezary
Portuguese Síndrome de Sezary, Doença de Sezary, Eritroderma de Sézary, Síndrome de Sézary
Japanese セザリー病, ｾｻﾞﾘｰﾋﾞｮｳ, ｾｻﾞﾘｰｼｮｳｺｳｸﾞﾝ, Sezary紅皮症, Sezary症候群, シーゼリィ病, シーゼリー症候群, セザリー症候群, セザール症候群, セザリー紅皮症, 紅皮症-セザリー
Swedish Sezarys syndrom
Czech Sézaryho syndrom, Sézaryho choroba
Finnish Sézaryn oireyhtymä
Russian ERITRODERMIIA SEZARI, SEZARI SINDROM, СЕЗАРИ СИНДРОМ, ЭРИТРОДЕРМИЯ СЕЗАРИ
Korean 세자리병
Polish Zespół Sezary'ego, Erytrodermia Sezary'ego
Hungarian Sézary-syndroma, Sézary-betegség, Sézary-szindróma
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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