Mycosis fungoides

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Mycosis fungoides is rare lymphomas of CD4+ helper T cells. Mycosis Fungoides was first described in 1806 by French dermatologist Jean-Louis-Marc Alibert. On microscopic histopathological analysis, atypical lymphoid cells, polymorphous inflammatory infiltrate in the dermis, and lymphocytes with cerebroid nuclei are characteristic findings of mycosis fungoides. Mycosis fungoides is caused by a mutation in the T cells. Mycosis fungoides must be differentiated from other diseases such as eczema and psoriasis. Mycosis fungoides commonly affects 45 and 55 years. In the United States, males are more commonly affected with Mycosis fungoides than females. In the United States, Mycosis fungoides usually affects individuals of the African American race.The risk factors in the development of mycosis fungoides is environmental, occupationalexposure to long term exposure to chemicals, virainfection. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for cutaneous T cell lymphoma.The staging of mycosis fungoides is based on skin and lymph node involvement.The most common symptoms of mycosis fungoides include fever, weight loss, skin rash, night sweats, itching, chest pain, abdominal pain, and bone pain. Common physical examination findings of mycosis fungoides include fever, rash, pruritus, ulcer, chest tenderness, abdominal tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.Laboratory tests for mycosis fungoides include complete blood count (CBC), blood chemistry studies, flow cytometry, immunohistochemistry, and immunophenotyping. The definitive diagnosis of mycosis fungoides is confirmed by either a skin biopsy or a lymph node biopsy. CT scanning in mycosis fungoides patients evaluated information such as enlarged lymph node (LN) and metastatic involvement. MRI may be helpful in the diagnosis of mycosis fungoides. PET scan may be helpful in the diagnosis of mycosis fungoides.Other diagnostic studies for mycosis fungoides include bone marrow aspiration and bone marrow biopsy.The predominant therapy for mycosis fungoides is PUVA. Adjunctive chemotherapy, radiotherapy, biological therapy, retinoid therapy, and photophoresis may be required.

Mycosis Fungoides was first described in 1806 by French dermatologist Jean-Louis-Marc Alibert. Sézary’s disease was first described by Albert Sézary

There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes.

Cutaneous T cell lymphoma arises from T-cell lymphocytes, which are normally involved in the cell mediated immune response. On microscopic histopathological analysis, atypical lymphoid cells, polymorphous inflammatory infiltrate in the dermis, and lymphocytes with cerebroid nuclei are characteristic findings of mycosis fungoides.

Development of cutaneous T cell lymphoma is the result of multiple genetic mutations.

Cutaneous T cell lymphoma must be differentiated from other diseases such as eczema and psoriasis.

The incidence of mycosis fungoides increases with age; the median age at diagnosis is between 45 and 55 years of age. The median age at diagnosis of Sézary syndrome is between 60 years of age. In the United States, males are more commonly affected with cutaneous T cell lymphoma than females. In the United States, cutaneous T cell lymphoma usually affects individuals of the African American race.^[1]

There are no established risk factors for cutaneous T cell lymphoma.

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for cutaneous T cell lymphoma.^[2]

If left untreated, cutaneous T cell lymphoma may progress to develop cutaneous patches and plaque. Depending on the extent of the lymphoma at the time of diagnosis, the prognosis may vary.

The staging of cutaneous T cell lymphoma is based on the skin and lymph nodes involvement.^[3]

The most common symptoms of cutaneous T cell lymphoma include fever, weight loss, skin rash, night sweats, itching, chest pain, abdominal pain, and bone pain.^[4]

Common physical examination findings of cutaneous T cell lymphoma include fever, rash, pruritus, ulcer, chest tenderness, abdominal tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.^[4]

Laboratory tests for cutaneous T cell lymphoma include complete blood count (CBC), blood chemistry studies, flow cytometry, immunohistochemistry, and immunophenotyping.^[4]

The definitive diagnosis of cutaneous T cell lymphoma is confirmed by either a skin biopsy or a lymph node biopsy.

CT scan may be helpful in the diagnosis of cutaneous T cell lymphoma.^[4]

MRI may be helpful in the diagnosis of cutaneous T cell lymphoma.^[4]

PET scan may be helpful in the diagnosis of cutaneous T cell lymphoma.^[4]

Other diagnostic studies for cutaneous T cell lymphoma include bone marrow aspiration and bone marrow biopsy. ^[4]

The predominant therapy for cutaneous T cell lymphoma is PUVA. Adjunctive chemotherapy, radiotherapy, biological therapy, retinoid therapy, and photophoresis may be required.^[3]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Mycosis Fungoides was first described in 1806 by French dermatologist Jean-Louis-Marc Alibert.

• Mycosis Fungoides was first described in 1806 by French dermatologist Jean-Louis-Marc Alibert.^[1][2][3]
• Mycosis fungoides is unrelated to any fungal infection. The term fungoides was first used by Dr. Jean-Louis-Marc Alibert to describe the tumor as having a mushroom-like appearance.^[4]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes. Cutaneous T cell lymphoma may be classified into several subtypes based on WHO-EORTC classification.

According to world Health Organization (WHO) and European Organization for Research and Treatment of Cancer (EORTC) classification, mycosis fungoides may be classified into the following types:^[1][2]

• Mycosis fungoides
• Mycosis fungoides variants and subtypes
  • Folliculotropic mycosis fungoides
  • Pagetoid reticulosis
  • Granulomatous slack skin

• The staging of cutaneous T cell lymphoma is based on skin and lymph node involvement.^[3]
• Staging for cutaneous T cell lymphoma(Mycosis fungoides (MF) and Sezary syndrome have same critera for staging) is provided in the following table:^[4][5]

The staging of Sezary syndrome is based on the clinical stages:^[4][6]

• [5-year disease free survival (DSS)]
• Cancer has spread to other organs in the body, including the blood and bone marrow
• Lymph nodes may be enlarged and may contain cancer

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Mycosis fungoides arises from T-cell lymphocytes, which are normally involved in the cell mediated immune response. On microscopic histopathological analysis, atypical lymphoid cells, polymorphous inflammatory infiltrate in the dermis, and lymphocytes with cerebroid nuclei are characteristic findings of mycosis fungoides.

• Mycosis fungoides arises from T-cell lymphocytes, which are normally involved in the cell mediated immune response.^[1]
• The tumor cells originate from memory T cells or skin homing CD4+ T cells expressing cutaneous lymphocyte antigen (CLA) and chemokine receptors CCR4 and CCR7.^[2]
• It is understood that maycosis fungoides is the result of malignant T cell that derived from a mature CD41 CD45RO1 memory T cells.^[3]
• Malignant T cell express adhesion molecules such as CCR4 and CLA.^[3]
• Immunohistochemistry shows expression T-cell antigens such as CD7, CD5, CD26, CD2 and CD3.^[3]
• Mycosis fungoides is T-cell lymphomas that primary manifest as multiple cutaneous lesions.^[4]
• Mycosis Fungoides is the most common type of cutaneous T cell lymphoma.^[5]

• Mycosis fungoides pathological course may be divided into three main stages:^[6][7][8]

• Premycotic stage
• Mycotic stage
• Tumoros stage

• The premycotic stage

• Non-diagnostic and represented by chronic nonspecific dermatisis associated with psoriasiform changes in epidermis

• The mycotic stage

• Shows a polymorphous inflammatory infiltrate in the dermis that contains small numbers of frankly atypical lymphoid cells
• These cells may line up individually along the epidermal basal layer
• The presence of spongiosis is highly suggestive of mycosis fungoides

• Tumoros stage

• Expansion of the dermis due to dense infiltration by medium sized lymphocytes that are typically characterized by a [[[cerebroid]] nuclei.

• Development of mycosis fungoides disease is the result of multiple genetic mutations.^{[9][10][11][12][13]}

• Genes involved in the pathogenesis of mycosis fungoides include:^[14]
  • Deletions or translocations involving a gene, NAV3, at 12q2 (helicaselike activity)
  • Deletion in 42 regions and amplification in 21 observed with meaningful amplifications of 8q (MYC) and 17q (STAT3) and deletions of 17p (TP53) and 10 (PTEN, FAS)^[15]

• Other deletion such as: ZEB1, ARID1A, DNMT3A, CDKN2A, FAS, ATM, CTCF, STAT5B, PRKCQ and somatic mutations (NFKB2, CD28, RHOA) observed in gene sequencing.^[16]

• The development of cutaneous T cell lymphoma is the result of multiple genetic mutations such as (There is not a classic chromosomal translocation in cutaeous T cell lymphoma( MF and SS ) significant):^{[17][18][19][20]}
• chromosomal instability has been noted. Losses on 1p, 10q, 13q, and 17p and gains of 4, 17q, and 18 have been identified ^[21]
  • Deletions and translocations in different chromosomes or chromosomal segments
  • Chromosomal amplification of JunB at 19p12 observed in mycosis fungoides and Sezary syndrome.^[21]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2], Sogand Goudarzi, MD [3]

Development of cutaneous T cell lymphoma is the result of multiple genetic mutations.

• The cause of mycosis fungoides has not been identified. However, there are certain risk factors that predisposes to risk of mycosis fungoides.
• For more information on risk factors, click here.
• Development of cutaneous T cell lymphoma is the result of multiple genetic mutations.^{[1][2][3][4][5]}

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2], Sowminya Arikapudi, M.B,B.S. [3]

Cutaneous T cell lymphoma must be differentiated from other diseases such as eczema and psoriasis.

• Mycosis fangoides must be differentiated from any diseases with cutaneous patch or plaque that not respond to first- and second-line treatment ssuch as:^{[1][2][3][4][5][6]}
  • Sezaruy syndrome
    • Sezaruy syndrome(SS) is more symptoI contrast to patch or plaque mycosis fungoides, SS is much more symptomatic. Sezary syndrome patients tend to present with diffuse skin involvement,not like mycosis fungoides usually evolve through patches and plaques to erythroderma ^[7]
    • In Sezary syndrome infiltration of skin is generally much less dense than plaque in mycosis fungoides (MF)
  • Eczema
  • Adult T cell leukemia/lymphma
  • Psoriasis
  • Pityriasis rubra pilaris
  • dermatitis
  • Hypereosinophilic syndrome
  • Adult T-cell leukemia
  • Atopic dermatitis
  • Contact dermatitis ( Allergic, irritant)
  • Chronic actinic dermatitis
  • Scabies
  • Subcutaneous panniculitis like T cell lymphoma (SPTCL)
  • Drug eruption
  • Graft versus host disease
  • Lichen planus
  • Pediatric atopic dermatitis
  • Tinea corporis
  • Primary cutaneous anaplastic large cell lymphoma (ALCL)
  • Cutaneous gamma/delta T cell lymphoma (G/D TCL)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

The incidence of mycosis fungoides increases with age; the median age at diagnosis is between 45 and 55 years of age. In the United States, males are more commonly affected with mycosis fungoides than females. In the United States, mycosis fungoides usually affects individuals of the African American race.

• The incidence of mycosis fungoides increases with age; the median age at diagnosis is between 40 and 60 years of age.^[1]
• Mycosis fungoidesaffects individuals younger than majority of patients and this diseases are reported in children.^[1]

• In the United States, males are more commonly affected with mycosis fungoides than females.^[1][2]

• In the United States, mycosis fungoides affects individuals of the African American race.^[1][2]

• The majority of mycosis fungoides (primary and secondary) cases are reported in geographical variances folllowing viral-induced lymphomas might show partial geographical restriction.^[3]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

• There are no established risk factors for cutaneous T cell lymphoma.The risk factors in the development of mycosis fungoides is environmental, occupational exposure to long term exposure to chemicals, virainfection.

• There are no established risk factors for cutaneous T cell lymphoma.
• The risk factors in the development of mycosis fungoides is environmental and occupational exposure.^[1][2][3]
• Other risk factors for cutaneous T cell lymphoma include :
  • Bacterial infection of the skin
  • Long term exposure to chemicals
  • Smoking
  • Medications
  • Long term sun exposure
  • Viral infections (Human T lymphotropic virus type I)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for cutaneous T cell lymphoma.

• According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for mycosis fungoiseades.
• Visual skin examination to screen for skin cancer in adults should be helpful.^[1]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

If left untreated, mycosis fungoides may progress to develop cutaneous patches, plaque, and tumors. Depending on the extent of the lymphoma at the time of diagnosis, the prognosis may vary.

• The symptoms of mycosis fungoides usually develop as non-specific, indolent inflammation such as etopic dermatitis, nonspecific chronic dermatitis, or parapsoriasis ( large-plaque parapsoriasis).^[1]
• The majority of patients have early stage (77.9% : IA stage IA 38.8%, stage IB 39.1% ), and patients with stage IIB diagnosed in 5.5% and stage III observed in 6.6%. The majority of patients of mycosis fungoides are not diagnosed and end stage not be observed.^[1]
• Mycosis fungoides in early stage is characterized by non-specific dermatitis or consistent patches observed on the lower trunk and buttocks.^[1]
• Mycosis fungoides initiates as an indolent lymphoma that may later develop peripheral lymphadenopathy and may finally progress to widespread visceral involvement.^[2]
• Patients often have a history of several years of eczematous or dermatitis skin lesions before the diagnosis is finally established.^[1]
• The skin lesions then progress from the patch stage to the plaque stage to cutaneous tumors.^[1]

• Common complications of mycosis fungoides include:^{[3][4][5][6][7]}
  • Mycosis Fungoides increases risk of secondary malignancies such as primary malignancy, especially Hodgkin lymphoma, chronic leukemia, and lung cancer.
  • Neurologic involvement (intracerebral mycosis fungoides)
  • Oral involvement
  • Hypersensitivity reaction with topical HN2 therapy
  • Hypopigmentation or hyperpigmentation of treated areas

• Cutaneous T cell lymphoma is usually a slow-growing (indolent) lymphoma.^[8]
• The prognosis for people with cutaneous T cell lymphoma is based on the extent of disease and how the person responds to treatment.
• Although more advanced stages of cutaneous T cell lymphoma may not be cured, the lymphoma can still be controlled with treatment

• Early stage disease
• Lymphoma is confined to the skin

• More advanced disease
• Lymphoma has spread to lymph nodes
• Lymphoma has spread to other organs

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