Development of indolent skin lesions is the major characteristic of mycosis fungoides [3]. The lesions most commonly appear in the areas of buttocks, groin, breast, lower trunk, hips and axillae [4]. The skin lesions usually develop in three stages:

• Patch phase: The patches measure about 15 cm and are flat with pink–brown macules [5]. They may appear as single or multiple patches. Following this, the patches evolve as plaques.
• Plaque phase: Plaques are raised having irregular borders. These are well demarcated with erythematosus shapes [6].
• Tumor phase: In this stage, the plaques turn malignant and evolve as ulcerating and necrotic tumors.

• Splenomegaly

  Na kůži je svědící erytrodermie, hyperkeratóza, pigmentace, může být lymfadenopatie či splenomegalie. Objevují se Sézaryho-Lutznerovy buňky. Vyskytuje se obv. u starších osob s určitou převahou mužů, průběh bývá větš. chronický. [fpnotebook.com]

  However, if lung abnormalities or other suggestions of extracutaneous lymphomatous involvement besides splenomegaly are seen on imaging, biopsy confirmation is usually recommended before categorising this as visceral involvement of MF or SS. [emjreviews.com]

  Splenomegaly in MF patients is generally proven to be either diffuse or nodular involvement with MF 14 and is uncommon in healthy persons or in those with benign skin disease. [doi.org]

• Fever

  Hospital Discharge [ORANGE] Unknown status NCT00445497 Phase 3 amoxicillin-clavulanate potassium;ciprofloxacin 8 Multicenter, Double-Blind, Randomized Study to Compare the Safety and Efficacy of Levofloxacin With That of Cefepime in the Treatment of Fever [malacards.org]

  One year later, he presented with fever, more than 30 infiltrated cutaneous plaques, and 2 ulcers resembling pyoderma gangrenosum (PG). His neutrophil count was normal, but his ESR and CRP level (108 mg/L) were increased. [jamanetwork.com]

  Symptoms are related mostly to the skin, with fevers, night sweats, and unintentional weight loss coming later in the disease course. [merckmanuals.com]

  Patients with myocarditis may present with the following signs and symptoms: Mild symptoms of chest pain (in concurrent pericarditis), fever, sweats, chills, dyspnea In viral myocarditis: Recent history (≤1-2 wk) of flulike symptoms of fevers, arthralgias [emedicine.medscape.com]

• Weight Loss

  In addition, individuals often experience high temperatures, weight loss, anemia, and symptoms of malaise and weakness, too. Gastrointestinal symptoms (and possible ulcerations of the intestines), coughing, and difficulty swallowing may develop. [everydayhealth.com]

  If you notice swollen lymph nodes, unexplained weight loss and extreme fatigue, visit your doctor. Early detection and treatment is key to fighting lymphoma, which can be detected through blood work or a biopsy. [sharecare.com]

  Common symptoms of follicular lymphoma include swollen lymph nodes, fatigue, shortness of breath, night sweats and weight loss. [cancercenter.com]

  Symptoms are related mostly to the skin, with fevers, night sweats, and unintentional weight loss coming later in the disease course. [merckmanuals.com]

• Malaise

  Infiltrating Stage): Small circular, purple-ish, raised plaques that slowly grow and coalesce with one another Lymph node inflammation Stage III (Fungoid or Tumor Stage): Bluish or red-brown tumors that resemble mushrooms on the skin Large nodules Stage IV: Malaise [patientworthy.com]

  In addition, individuals often experience high temperatures, weight loss, anemia, and symptoms of malaise and weakness, too. Gastrointestinal symptoms (and possible ulcerations of the intestines), coughing, and difficulty swallowing may develop. [everydayhealth.com]

  Symptoms may include a general feeling of ill health (malaise) and weakness, elevated temperatures, weight loss, and anemia. There may be gastrointestinal involvement with or without ulceration of the intestines. [rarediseases.org]

• Varicella-Zoster Virus Infection

  RESULTS: Four herpes simplex virus type-I (HSV-I), 2 staphylococcal (S. aureus) impetiginizations and 2 Malassezia infections were detected in single isolated plaque/patch stage MF as well as 1 varicella zoster virus infection (herpes zoster, HZ) and [ncbi.nlm.nih.gov]

• Throat Tightness

  Herein, we report the case of a patient with a 7-year history of MF who presented to the emergency department with hoarseness, throat tightness and cough, as well as erythroderma and skin tumours. [ncbi.nlm.nih.gov]

• Hepatosplenomegaly

  Subsequently, systemic spread occurs, including lymphadenopathy and hepatosplenomegaly. [amboss.com]

  […] usually helper, less often, suppressor subtypes that is 2-fold more common in older blacks Clinical Skin involvement precedes Sx by up to 2 yrs; the leukemic phase–Sezary syndrome–occurs in 80% and is accompanied by fever, weight loss, lymphadenopathy, hepatosplenomegaly [medical-dictionary.thefreedictionary.com]

  It is characterised by erythroderma, leukaemia, generalised lymphadenopathy and hepatosplenomegaly. It occurs most frequently in middle-aged males. Patients have a median survival of less than five years. [patient.info]

  […] condition can affect people of all ages, it is commonly diagnosed in adults over age 60. [6] [2] The dominant symptoms of the disease are: Generalized erythroderma [2] Lymphadenopathy [2] Atypical T-cells ("Sézary cells") in the peripheral blood [2] Hepatosplenomegaly [en.wikipedia.org]

• Hepatomegaly

  Liver involvement may be suggested by clinical hepatomegaly, abnormal liver function tests, or radiologic tests (CT, fludeoxyglucose-positron emission tomography, and liver or spleen scan) and should be confirmed by liver biopsy. 3 Cerebral lesions may [emjreviews.com]

• Skin Lesion

  Development of indolent skin lesions is the major characteristic of mycosis fungoides. The lesions most commonly appear in the areas of buttocks, groin, breast, lower trunk, hips and axillae. [symptoma.com]

  The patient continued to receive topical steroids and maintenance phototherapy, and her skin lesions completely resolved after 14 weeks of therapy. [ncbi.nlm.nih.gov]

  It is usually initially seen by dermatologists with patients presenting with skin lesions. Patients often have a history of several years of eczematous or dermatitic skin lesions before the diagnosis is finally established. [radiopaedia.org]

  T categories T1: There is only a single skin lesion. T1a: The skin lesion is less than 5 cm (about 2 inches) across. T1b: The skin lesion is at least 5 cm across. T2: There are 2 or more lesions on the skin. [cancer.org]

  It generally affects the skin, but may progress internally over time. Symptoms include rash, tumors, skin lesions, and itchy skin. While the cause remains unclear, most cases are not hereditary. [dbpedia.org]

• Ulcer

  Abstract Methotrexate-induced cutaneous ulceration is a rare but potentially serious drug adverse reaction. [ncbi.nlm.nih.gov]

  Figure 1 Clinical manifestation of the ulcerative lesion with necrotic center localized in the upper eyelid of the right eye. [mayoclinicproceedings.org]

  […] divided generally into three successive stages: premycotic, associated with intensely pruritic eruptions; infiltrated plaques, or mycotic, characterized by the presence of abnormal mononuclear cells ( Sézary cells ); and mushroom-like tumors that often ulcerate [medical-dictionary.thefreedictionary.com]

• Eczema

  Without effective therapy a systemic infection of this type can be fatal. my·co·sis fun·goi·'des [MIM*254400] a chronic progressive lymphoma arising in the skin that initially simulates eczema or other inflammatory dermatoses; the appearance of plaques [medical-dictionary.thefreedictionary.com]

  It can be seen in children and is usually mistaken for eczema, vitiligo, or progressive macular hypomelanosis, clinically and histopathologically. We present a boy with HMF confirmed by histopathology. [ncbi.nlm.nih.gov]

  This stage may be very subtle histopathologically and mimic other dermatoses such as eczema or lichenoid dermatoses, as the atypia may be difficult to appreciate. [dermnetnz.org]

• Pruritus

  KEYWORDS: cutaneous T-cell lymphoma; epidermal keratinocytes; pruritus; thymic stromal lymphopoietin; vascular endothelial growth factor A [ncbi.nlm.nih.gov]

  Chronic intractable pruritus has a significant detrimental impact on quality of life, and for individuals with CTCL, it has been estimated to double the mortality rate compared to those without pruritus. [web.archive.org]

  Elmariah, Pruritus in Chronic Kidney Disease, Dermatological Manifestations of Kidney Disease, 10.1007/978-1-4939-2395-3_7, (81-89), (2015). Timur A. Galperin, Antonia J. Cronin and Kieron S. [doi.org]

• Alopecia

  Clinically, this area resembled alopecia areata (AA) and was without hypopigmentation or erythema. A scalp biopsy revealed a non-scarring inflammatory alopecia and a superficial band-like atypical lymphoid infiltrate with prominent epidermotropism. [ncbi.nlm.nih.gov]

  Pinkus noted 3 distinct patterns: an idiopathic form of alopecia mucinosa, lymphoblastoma with associated FM, and alopecia mucinosa that later transformed into lymphoblastoma. 4 In 1983, however, Pinkus 4 described uncertainty if alopecia mucinosa represented [mdedge.com]

• Peripheral Neuropathy

  neuropathy, and periarteritis nodosa. [medical-dictionary.thefreedictionary.com]

  After a median 22.9 months of follow-up, 82% of patients with peripheral neuropathy experienced improvement or resolution. [frontiersin.org]

  The main limiting AE is neurosensitive peripheral neuropathy. Mogamulizumab is a humanized monoclonal antibody that targets the CC chemokine receptor 4, modified by glycoengineering to enhance its antibody-dependent cell-mediated cytotoxicity. [dovepress.com]

• Responsiveness Decreasing

  A large proportion of these patients may remain disease-free. 37 The likelihood of achieving a CR and the durability of those responses decreases with increasing stage of disease; patients with T1 disease have a more than 80% CR rate with radiotherapy [doi.org]

• Neurologic Manifestation

  In the patient in this report, MF had been diagnosed 2 years before neurologic manifestations appeared; MR images showed brainstem abnormalities. [ajnr.org]

Diagnosis of mycosis fungoides is often misleading and difficult. In the initial stages, the lesions mimic eczema or psoriasis. In such cases, more in depth diagnosis needs to be done to reach a definitive conclusion. Following are various methods for diagnosing mycosis fungoides:

• Blood work to determine RBCs, WBCs, platelets and hemoglobin.
• Peripheral blood smear test to analyze the structure of the blood components.
• Skin biopsy to check for nature of the lesions.
• T–cell receptor gene rearrangement test to determine changes in the genetic functioning.
• Flow cytometry to determine the number of cells in the sample taken, the number of live cells and is also used to analyze the presence of tumor markers by polymerase chain reaction on the surface of the cells [7].
• Immunophenotyping test to identify the cancer cells [8].

• Sezary Cell

  cells ( B0a - Clone negative B0b - Clone positive B1 - Circulating Sezary cells (≥5% of lymphocytes) B1a - Clone negative B1b - Clone positive B2 - High blood tumor burden (≥ 1000/uL Sezary cells with positive clone) Notes : Patch indicates any size [en.wikibooks.org]

  Sezary cells have a type 2 helper T cell cytokine profile. [omim.org]

  B1: Low numbers of Sezary cells in the blood (more than in B0 but less than in B2). B2: High number of Sezary cells in the blood. [cancer.org]

  […] title=Category:Mycosis_fungoides&oldid=324846893 " Categorie : T-cell lymphomas Cutaneous T-cell lymphoma Categoria nascosta: Uses of Wikidata Infobox [commons.wikimedia.org]

  Quantitation of neoplastic T lymphocytes within the peripheral blood (Sezary cells) is also performed, either by manual differential, flow cytometry, or both. A count of 1000 cells per micrometer is the cutoff between clinical stages III and IV. [clinicaladvisor.com]

• Human Herpesvirus 8

  The presence of human herpesvirus 8 (HHV-8) - associated with all forms of Kaposi's sarcoma - has also been recently identified in mycosis fungoides lesions. [ncbi.nlm.nih.gov]

Treatment regime involves achieving any of the 2 stages, either the remission state or the stable state. The following are various methods involved to treat mycosis fungoides:

• Use of topical chemotherapy to clear off the skin lesions and patches. This is usually the first line of treatment for mycosis fungoides.
• Topical retinoid is yet another therapy meant for local application of the medicated ointment on the skin plaques.
• Topical mechlorethamine may be uses to treat mycosis fungoides [9].
• Topical and systemic treatment combination for mycosis fungoides may be indicated for stages II B or higher [10].
• Electron beam radiation is a method that employs exposing the skin to high beam radiation. The radiation does not penetrate the skin and does not harm the internal organs. 
• Oral retinoids are drugs that work by changing the growth pattern of mycosis fungoides cells.
• Interferon alpha is meant for treating patches that are widespread.
• Extracorporal photopheresis involves delivering PUVA therapy to the skin, lymph nodes and the blood.

The prognosis of the disease condition greatly depends on the stage of the disease and type of lesions present. Individuals who have the disease diagnosed on time with early initiation of treatment can live healthy lifes for several years. As the disease progresses, the cancer spreads to other body parts making treatment of the condition difficult.

Complications

The most common complication of mycosis fungoides is the spread of the disease to other organs of the body. In addition, the following are some of the complications:

• Cardiac failure
• Development of melanoma
• Colon cancer
• Edema
• Secondary malignancies
• Hodgkin lymphoma
• Neurofibromatosis [2]
• Non–Hodgkin lymphoma

The exact cause that triggers the development of mycosis fungoides is not yet known. However, several other factors have been known to play foul such as environment, viral infection, chronic lymphocyte stimulation and exposure to chemicals. The association of these factors in development of mycosis fungoides has not been extensively explained.

Certain theories point towards the fact that such a type of skin cancer develops as a result of chromosomal abnormalities. Individuals affected by this disease condition have an additional DNA in chromosomes 7 and 17 or have less DNA on chromosomes 9 and 10. Even though such a pattern has been noticed, there is still lack of evidence suggesting chromosomal abnormalities as the cause of mycosis fungoides.

1 in 100,000 to 350,000 individuals are known to be affected by mycosis fungoides. It has been estimated that about 3.6 million individuals of US are affected by this cancerous disease. Mycosis fungoides accounts for about 70% of T–cell lymphomas. Males are more commonly affected by this condition as compared to females.

Mycosis fungoides is a type of cutaneous T–cell lymphoma. In this disease, the T-cells turn cancerous in nature affecting the skin which in turn projects as skin lesions with different characteristics [1].

Preventing mycosis fungoides is difficult. However, steps can be taken to prevent the onset of complications and secondary disease conditions. Timely diagnosis of the disease and initiation of treatment can help in disease progression.

Mycosis fungoides is a type of cutaneous T-cell lymphoma which is also known as Alibert–Bazin syndrome named after the French dermatologist who described it. It is a type of non-Hodgkin lymphoma and presents with serious life threatening complications. Adults aged 20 years and above fall prey to this disease.

The exact etiology remains a unknown; however heredity and genetic factors have no involvement. The male population is more affected in comparison to women. Skin is the most affected organ; and if treatment is not initiated then cancer can spread to rest of the body parts.

Definition

Mycosis fungoides is a type of T–cell lymphoma that causes development of skin lesion and patches. The common areas of the body affected by this disease include groin, buttocks, hips, axillae and breasts. Men are more affected than women and this disease usually strikes the adult population with very rare incidence amongst the children. With early diagnosis, the disease progression can be arrested and individuals can live a quality life.

Cause

The exact cause that triggers mycosis fungoides is yet to be figured out. However, factors such as chemical exposure, environment, viral infection and chromosomal abnormalities are known to play foul.

Symptoms

Development of skin lesions on the body is the primary symptom of mycosis fungoides. The lesions initially appear as patches and as the disease progresses, the patches then evolve as plaques which turn into necrotic tumors later on.

Diagnosis

Physical examination of the skin lesions forms the primary diagnostic regime. In addition, blood work, peripheral blood smear test, skin biopsy, flow cytometry, immunophenotyping and T-cell receptor gene rearrangement test are also done.

Treatment

Treatment of mycosis fungoides is geared towards management of the skin lesion by application of topical agents and through radiation therapy. Topical agents such as topical chemotherapy, topical retinoids, oral retinoids are employed for clearing off the skin lesion. Electron beam radiation is a method that involves exposing the skin to high beam radiation to destroy the cancer cells.

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2. Gelfand TM, Berling J, van Vorhees A, Margolis DJ. Lymphoma are low but increased in patients with psoriasis. Arch with Dermatol 2003; 139:1425-9.
3. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. May 15 2005; 105(10):3768-85.
4. Wain EM, Setterfield J, Judge MR et al, Mycosis Fungoides involving the oral mucosa of a child. Clin Exp Dermatol 2003; 28:499-501.
5. Saada D, Lami MD, Vabres P et al. Mycoses Fungoides presenting as annular erythema. Annal Dermatol Venereol 2005; 132:35-7.
6. Burg G, Schmockel C. Syringolymphoid hyperplasia with alopecia- a syringotropic T-cell lymphoma? Dermatology 1992; 184:306-7.
7. Burg G, Kemppf W, Dummer R. Diagnostic signs of cutaneous lymphomas. J Eur Acad Dermatol Venereol 2001; 15:358-9
8. Willemze R, Meijer CJ. Rationale of a new classification for the group of primary cutaneous lymphomas. Semin Cutan Med Surg 2000; 19:71-7.
9. Ceptaris Therapeutics Inc. Ceptaris receives FDA approval for Valchlor (mechlorethamine) gel for the treatment of stage IA and IB mycosis fungoides-type cutaneous T-cell lymphoma in patients who have received prior skin-directed therapy.
10. Willemze R, Meijer CJ. Rationale of a new classification for the group of primary cutaneous lymphomas. Semin Cutan Med Surg 2000; 19:71-7.