Mycosis fungoides
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| Mycosis fungoides Classification and external resources | |
| ICD-10 | C84.0 |
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| ICD-9 | 202.1 |
| ICD-O: | 9700/3 |
| OMIM | 254400 |
| DiseasesDB | 8595 |
| eMedicine | med/1541 |
| MeSH | D009182 |
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Most recent articles on Mycosis fungoides Most cited articles on Mycosis fungoides | |
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Cochrane Collaboration on Mycosis fungoides | |
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Ongoing Trials on Mycosis fungoides at Clinical Trials.gov Trial results on Mycosis fungoides Clinical Trials on Mycosis fungoides at Google
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US National Guidelines Clearinghouse on Mycosis fungoides NICE Guidance on Mycosis fungoides
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Overview
Mycosis Fungoides (also known as Alibert-Bazin syndrome[1] or granuloma fungoides), is a rare form of cutaneous T-cell non-Hodgkin's lymphoma. It generally affects the skin, but may progress internally over time.
Mycosis Fungoides was first described in 1806 by French dermatologist Jean-Louis-Marc Alibert. The name mycosis fungoides is somewhat confusing because it sounds, to the uninitiated, like a type of fungal infection. In reality, mycosis fungoides is unrelated to fungus and the fungoides portion derives from a patient with a severe case whom Alibert described as having mushroom-like skin tumors.
Origins and causes
The cause of mycosis fungoides is unknown, and is not believed to be hereditary or genetic. It is not contagious.
It is rare for the disease to appear before age 20, and it appears to be noticeably more common in males than females, especially over the age of 50, where the incidence of the disease (the risk per person in the population) does increase. The average age of onset is between 45 and 55 years of age for patients with patch and plaque disease only, but is over 60 for patients who present with tumours, erythroderma (red skin) or a leukemic form (the Sézary syndrome).
The disease is an unusual expression of T-cells, a part of the immune system. These T-cells are skin-associated, meaning that they biochemically and biologically are most related to the skin, in a dynamic manner. Mycosis Fungoides is the most common type of 'Cutaneous T cell lymphoma' (CTCL), but there are many other types of CTCL that have nothing to do with Mycosis Fungoides and these disorders are treated differently.
Symptoms, diagnosis, and stages
Typical visible symptoms include rashlike patches, tumors, or lesions. Itching (pruritus) is common, perhaps in 20% of patients, and is not universal.
Diagnosis is sometimes difficult because the early phases of the disease often resemble eczema or even psoriasis. As with any serious disease, it is advisable to pursue the opinion of a medical professional if a case is suspected. Diagnosis is generally accomplished through a skin biopsy. Several biopsies are recommended, to be more certain of the diagnosis. The diagnosis is made through a combination of the clinical picture and examination, and is confirmed by biopsy.
To stage the disease, various tests may be ordered, to assess nodes, blood and internal organs, but most patients present with disease apparently confined to the skin, as patches (flat spots) and plaques(slightly raised or 'wrinkled' spots).
Treatments and cures
Mycosis fungoides can be treated in a variety of ways.
If treatment is successful the disease can go into a non-progressing state with clinically clear examination and various tests. This is called remission; it can last indefinitely. Treatments may also cause disease not to progress, while still present, and this is called stable disease; it may last indefinitely but is a more serious situation. Disease may also progress, to involve nodes, blood and internal organs, or transform into a higher-grade lymphoma.
Common treatments include simple sunlight, ultraviolet light, topical steroids, topical and systemic chemotherapies, local superficial radiotherapy, total skin electron beam radiation, and biological therapies (e.g. interferons, retinoids, rexinoids).Vorinostat (Zolinza®) is a second-line drug for CTCL. Application of organic (Manuka) honey to skin affected by erythorderma (red skin) has also proved to be effective in reducing inflammation. Treatments are often used in combination.
Selection of treatments typically depends on patient preference and access to therapies, as well as recommendations by physicians, the stage of the disease, established resistance to prior therapies, allergies of the patient, clinical evidence of a positive benefit:risk ratio, and so on.
It is debatable whether cures are reliably obtained through different types of treatments, but many patients experience prolonged periods of disease-control and at least half of all patients do not die from this disease, even if not truly cured. Quality of life is a major objective, in addition to cure, and maximizing periods of remission or stable disease, while minimizing treatments and toxicities, are two central concerns in clinical care.
See also
References
External links
- DermAtlas 197
- The Lymphoma Information Network - Cutaneous T-Cell Lymphomas
- The Doctor's Doctor (detailed information)
- Cutaneous Lymphoma Foundation (formerly MFF)
- Clinical trial NCT00106431 - "A Single Agent Phase II Study of Romidepsin (Depsipeptide, FK228) in the Treatment of Cutaneous T-Cell Lymphoma"
WikiDoc Research Resources for Mycosis fungoides (Click show to right to view) | |
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| Articles on Mycosis fungoides | Most recent articles on Mycosis fungoides • Most cited articles on Mycosis fungoides • Review articles on Mycosis fungoides • Articles on Mycosis fungoides in N Eng J Med, Lancet, BMJ |
| Media (Slides, Video, Images, MP3) on Mycosis fungoides | Powerpoint slides on Mycosis fungoides • Images of Mycosis fungoides • Photos of Mycosis fungoides • Podcasts & MP3s on Mycosis fungoides • Videos on Mycosis fungoides |
| Evidence Based Medicine Regarding Mycosis fungoides | Cochrane Collaboration on Mycosis fungoides • Bandolier on Mycosis fungoides • TRIP on Mycosis fungoides |
| Cost Effectiveness of Mycosis fungoides | Cost Effectiveness of Mycosis fungoides |
| Clinical Trials Involving Mycosis fungoides | Ongoing Trials on Mycosis fungoides at Clinical Trials.gov • Trial results on Mycosis fungoides • Clinical Trials on Mycosis fungoides at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Mycosis fungoides | US National Guidelines Clearinghouse on Mycosis fungoides • NICE Guidance on Mycosis fungoides • NHS PRODIGY Guidance • FDA on Mycosis fungoides • CDC on Mycosis fungoides |
| Textbook Information on Mycosis fungoides | Books and Textbook Information on Mycosis fungoides |
| Pharmacology Resources on Mycosis fungoides | Dosing of Mycosis fungoides • Drug interactions with Mycosis fungoides • Side effects of Mycosis fungoides • Allergic reactions to Mycosis fungoides • Overdose information on Mycosis fungoides • Carcinogenicity information on Mycosis fungoides • Mycosis fungoides in pregnancy • Pharmacokinetics of Mycosis fungoides • |
| Genetics, Pharmacogenomics, and Proteinomics of Mycosis fungoides | Genetics of Mycosis fungoides • Pharmacogenomics of Mycosis fungoides • Proteomics of Mycosis fungoides |
| Newstories on Mycosis fungoides | Mycosis fungoides in the news • Be alerted to news on Mycosis fungoides • News trends on Mycosis fungoides |
| Commentary on Mycosis fungoides | Blogs on Mycosis fungoides |
| Patient Resources on Mycosis fungoides | Patient resources on Mycosis fungoides • Discussion groups on Mycosis fungoides • Patient Handouts on Mycosis fungoides • Directions to Hospitals Treating Mycosis fungoides • Risk calculators and risk factors for Mycosis fungoides |
| Healthcare Provider Resources on Mycosis fungoides | Symptoms of Mycosis fungoides • Causes & Risk Factors for Mycosis fungoides • Diagnostic studies for Mycosis fungoides • Treatment of Mycosis fungoides |
| Continuing Medical Education (CME) Programs on Mycosis fungoides | CME Programs on Mycosis fungoides |
| International Resources on Mycosis fungoides | Mycosis fungoides en Espanol • Mycosis fungoides en Francais |
| Business Resources on Mycosis fungoides | Mycosis fungoides in the Marketplace • Patents on Mycosis fungoides |
| Informatics Resources on Mycosis fungoides | List of terms related to Mycosis fungoides |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
