Presentation
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.
Entire Body System
- Fever
If the lymphoma involves internal organs, people may feel tired or experience weight loss, night sweats, or fevers. How is mycosis fungoides diagnosed ? Diagnosis is made by skin biopsy. [mountsinai.org]
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]
Other symptoms include: Swollen lymph nodes Night sweats Fever Fatigue Loss of appetite Weight loss A biopsy, usually of an infected lymph node, is required for an accurate diagnosis if Burkitt lymphoma is suspected. [cancercenter.com]
- Weight Loss
Common symptoms of follicular lymphoma include swollen lymph nodes, fatigue, shortness of breath, night sweats and weight loss. [cancercenter.com]
If the lymphoma involves internal organs, people may feel tired or experience weight loss, night sweats, or fevers. How is mycosis fungoides diagnosed ? Diagnosis is made by skin biopsy. [mountsinai.org]
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]
Symptoms are related mostly to the skin, with fevers, night sweats, and unintentional weight loss coming later in the disease course. [merckmanuals.com]
- Inflammation
Biopsy of an indurated mammary plaque initially indicated sarcoid granulomatous inflammation. Biopsy review suggested GMF. [ncbi.nlm.nih.gov]
MF is understood to arise through longstanding chronic inflammation, and can be considered as a state of longstanding low-grade systemic inflammation (3). [medicaljournals.se]
Normally, inflammation is a bodily response to any sort of wound or infection. Imagine when you cut your finger: within a short time, the tissue around the cut swells up and turns red, which are classic signs of inflammation. [healthline.com]
- Swelling
Symptoms of DLBCL may include swelling caused by enlarged lymph nodes in the neck, underarms or groin. The swelling may or may not be painless. [cancercenter.com]
Some treatments help control symptoms like skin soreness, swelling, and itching. [healthline.com]
Transformed MF occurs when a specific subset of MF tumor An abnormal mass or swelling of tissue, that can occur anywhere in the body. cells undergo molecular and/or genetic changes that cause them to become larger. [lymphoma.org]
Symptoms of mycosis fungoides (MF) The symptoms of mycosis fungoides (MF) can include: Painless swelling or lump, mainly in the neck, groin or armpit Irregularly shaped, oval or ring-like, dry or scaly patches most common on chest, back and buttocks. [lymphoma.org.au]
- Disability
Please enable JS and disable any ad blocker [aocd.org]
Immune System
- 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]
Gastrointestinal
- Diarrhea
The most common adverse reactions (reported in ≥20%) were rash, infusion-related reactions, fatigue, diarrhea, musculoskeletal pain, and upper respiratory tract infection. [asco.org]
Adverse Reactions The most common adverse reactions (reported in ≥10% of patients) with POTELIGEO in the clinical trial were rash, including drug eruption (35%), infusion reaction (33%), fatigue (31%), diarrhea (28%), drug eruption (24%), upper respiratory [poteligeohcp.com]
If MAC goes all over your body, you may have: High fever or chills Night sweats Belly pain Diarrhea Weight loss Fatigue Swollen glands Fewer red blood cells (anemia) You could also have more serious symptoms like: Blood infections Hepatitis Pneumonia [webmd.com]
Safety Profile The most common adverse events of any grade in patients receiving mogamulizumab-kpkc in the phase III trial (≥ 20% of patients) were rash including drug eruption, infusion-related reactions, fatigue, diarrhea, upper respiratory tract infection [ascopost.com]
- Failure to Thrive
However, the patient received only 10 fractions for a total dose of 18 Gy due to severe mucositis, which was not relieved by sucralfate, and eventually required hospitalization due to significant decreased oral intake and failure to thrive. [appliedradiationoncology.com]
Liver, Gall & Pancreas
- Hepatosplenomegaly
In the later stages of the disease, mushroom-shaped tumors develop within the plaque lesions, and ultimately lymphadenopathy and hepatosplenomegaly can develop. [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]
Musculoskeletal
- Muscle Spasm
SLIDESHOW Muscle Cramps (Charley Horse) and Muscle Spasm Remedies See Slideshow Medically Reviewed on 10/22/2018 References Medically reviewed by Robert J. [medicinenet.com]
Skin
- Skin Lesion
Sometimes, these skin lesions progress to infiltrated plaques followed by ulcerative lesions or tumerous growths. Patients can also present with skin patches, plaques, tumors and ulceration at the same time. [clinicaladvisor.com]
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]
- Ulcer
In advanced cases, ulcerated tumors and infiltration of lymph nodes by diseased cells may occur. The disorder may spread to other parts of the body including the gastrointestinal system, liver, spleen, or brain. (NORD/G. [dermis.net]
Abstract Methotrexate-induced cutaneous ulceration is a rare but potentially serious drug adverse reaction. [ncbi.nlm.nih.gov]
[…] de novo with ulceration without any history of ulcerative colitis or taking methotrexate. [journals.lww.com]
Figure 1 Clinical manifestation of the ulcerative lesion with necrotic center localized in the upper eyelid of the right eye. [mayoclinicproceedings.org]
- 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]
In its early stages, it can look like eczema. [healthline.com]
- Papule
[…] mimicking rosacea (n = 1), as well as white dome-shaped asymptomatic papules/nodules filled with mucin (on histology) (n = 2) that overlay other disease-related lesions. [ncbi.nlm.nih.gov]
Physicians of patients with transformed MF may see signs that include the presences of a new solitary nodule within a classic MF patch or plaque, an abrupt onset of multiple scattered papules and/or nodules that do not go away on their own, a new or enlarging [lymphoma.org]
A 68-year-old man presented to primary care with a rash consisting of polycyclic (overlapping circles) erythematous scaly papules and plaques with raised borders ( Figure 1A ) that was localized to his feet. [cmaj.ca]
Stage IB: Ten percent or more of the skin surface is covered with patches, papules, and/or plaques. There may be abnormal lymphocytes in the blood but they are not cancerous. [uofmhealth.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]
Neurologic
- 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]
Although brentuximab vedotin was well tolerated overall, peripheral sensory neuropathy was reported in 67% of patients (44/66: 17 with grade 1 neuropathy, 21 with grade 2 neuropathy, and 6 with grade 3 neuropathy). [hematologyandoncology.net]
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]
- Dizziness
[…] kidney damage Occasionally, Folliculotropic Mycosis Fungoides can convert into a more destructive form of lymphoma There may be complications related to chemotherapy used in treating the condition, which may include the following: Side effects such as dizziness [dovemed.com]
Tachycardia, chest pain, headache, pain, nervousness, dizziness, hypocalcemia, and weight loss may occur. Severe fatigue can occur after the first cycle. [clinicaladvisor.com]
Workup
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].
Serum
- 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]
Treatment
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.
Prognosis
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
Etiology
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.
Epidemiology
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.
Pathophysiology
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].
Prevention
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.
Summary
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.
Patient Information
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.
References
- Willemze R, Meijer CJ. Rationale of a new classification for the group of primary cutaneous lymphomas. Semin Cutan Med Surg 2000; 19:71-7.
- 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.
- Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. May 15 2005; 105(10):3768-85.
- Wain EM, Setterfield J, Judge MR et al, Mycosis Fungoides involving the oral mucosa of a child. Clin Exp Dermatol 2003; 28:499-501.
- Saada D, Lami MD, Vabres P et al. Mycoses Fungoides presenting as annular erythema. Annal Dermatol Venereol 2005; 132:35-7.
- Burg G, Schmockel C. Syringolymphoid hyperplasia with alopecia- a syringotropic T-cell lymphoma? Dermatology 1992; 184:306-7.
- Burg G, Kemppf W, Dummer R. Diagnostic signs of cutaneous lymphomas. J Eur Acad Dermatol Venereol 2001; 15:358-9
- Willemze R, Meijer CJ. Rationale of a new classification for the group of primary cutaneous lymphomas. Semin Cutan Med Surg 2000; 19:71-7.
- 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.
- Willemze R, Meijer CJ. Rationale of a new classification for the group of primary cutaneous lymphomas. Semin Cutan Med Surg 2000; 19:71-7.