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Mycosis Fungoides

Folliculotropic Mycosis Fungoides

Mycosis fungoides is a rare cutaneous T-cell lymphoma. The cause of mycosis fungoides is unknown.


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.
Fever
  • Symptoms are related mostly to the skin, with fevers, night sweats, and unintentional weight loss coming later in the disease course.[merckmanuals.com]
  • Compared With Epoetin Alfa in Treating Anemia in Patients Receiving Chemotherapy for Cancer Completed NCT00070382 Phase 3 darbepoetin alfa;epoetin alfa 20 Caspofungin Acetate Compared With Amphotericin B Liposomal in Treating Patients With Persistent Fever[malacards.org]
  • 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]
Weight Loss
  • 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]
  • 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 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]
Shivering
  • […] blood, muscle cells, and bones (SGOT) Asthenia Lack of energy or physical weakness or both Cardiomyopathy Structural or functional disease of the cardiac muscle Cardiopulmonary syndrome Adverse effect where the heart and the lung are involved Chill Shivering[doi.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]
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]
Vascular Disease
  • Follicular mucinosis (FM) is a primary or secondary finding in FMF, lupus, or collagen vascular diseases that is only a histological process of mucin deposition surrounding the hair follicles.[ncbi.nlm.nih.gov]
  • Coronary Artery Disease or Peripheral Vascular Disease. No change in standard management. E. Diabetes or other Endocrine issues. No change in standard management. F. Malignancy. No change in standard management. G.[cancertherapyadvisor.com]
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]
  • Systemic examination was normal and there was no hepatosplenomegaly. Investigations revealed a hemoglobin level of 8.6% gm and the Erythrocyte sedimentation rate (ESR) of 110 mm/hour.[idoj.in]
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
  • Treatment Treatment of mycosis fungoides is geared towards management of the skin lesion by application of topical agents and through radiation therapy.[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]
  • A case of difficult histopathological diagnosis with florid and unusual skin lesions mainly on the face is reported.[ncbi.nlm.nih.gov]
  • And it turned out that these patients responded well to ALA-PDT: the skin lesions of 2 patients subsided completely, and the last one had more than 75% of the lesions eliminated.[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]
Ulcer
  • Abstract Methotrexate-induced cutaneous ulceration is a rare but potentially serious drug adverse reaction.[ncbi.nlm.nih.gov]
  • A 64-year-old man presented with a 2 week history of progressive phimosis and painful ulcer on his penile meatus.[ncbi.nlm.nih.gov]
  • We describe a patient with a 30-year history of mycosis fungoides (MF) and renal transplantation performed 3 years before he presented with an ulcerated tumour in the lumbosacral area.[ncbi.nlm.nih.gov]
  • Unexplained exacerbation or the sudden appearance of oozing or ulcerations in MF lesions should initiate a search for viral or bacterial agents. Cellulitis and HZ can be severer and prolonged in MF patients. 2017 S. Karger AG, Basel.[ncbi.nlm.nih.gov]
  • In presumed cases of psoriasis that are unresponsive to treatment, progressive, or ulcerative, biopsy should be considered to rule out MF, particularly before starting a potent immunosuppressive agent.[ncbi.nlm.nih.gov]
Eczema
  • 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]
  • 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]
  • Importantly, serum IL-36γ levels in AD patients positively correlated with Eczema Area and Severity Index and those of MF/SS patients positively correlated with serum soluble IL-2 receptor levels.[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]
  • Nodular prurigo (NP) is still a condition of unknown etiology characterized by papulonodular eruption and intense pruritus. Multiple diseases, including dermatological, systemic, and psychiatric diseases, have been assumed to cause NP.[ncbi.nlm.nih.gov]
  • Other important considerations are symptom severity, including pruritus and patient age/comorbidities.[ncbi.nlm.nih.gov]
  • Median scores for pruritus were decreased from 3.43 before TSEBT to 1.88 after low dose TSEBT and a decreased in quality of life score median from 6.60 to a median of 2.75.[ncbi.nlm.nih.gov]
  • Signs and symptoms [ edit ] Plaque of mycosis fungoides Typical visible symptoms include rash-like patches, tumors, or lesions. [2] Itching ( pruritus ) is common, perhaps in 20 percent of patients, but is not universal.[en.wikipedia.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]
  • RESULTS: HLA-G expression was lower in FMF (34%: 14/41) and CMF (18%: 2/11) groups compared to alopecia areata (92%:11/12) and normal scalp skin group (100%: 12/12).[ncbi.nlm.nih.gov]
  • We report a case of an 83-year-old woman with folliculotropic MF characterized by erythematous, scaly plaques on the forehead along with poliosis and alopecia of the right medial eyebrow.[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]
  • The infiltrate involved the lower isthmic part of the follicle, and in 1 case, there was an alopecia areata-like neoplastic lymphomatoid bulbitis.[ncbi.nlm.nih.gov]
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]

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].
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 skin[en.wikibooks.org]
  • Sezary cells have a type 2 helper T cell cytokine profile.[omim.org]
  • Cartamo.jpg 2 448 3 264; 1,69 MB Mycosis Fungoides Before Sun and Cartamo.jpg 2 448 3 264; 1,16 MB Mycosis fungoides knee.JPG 3 472 2 604; 1,75 MB Neck of a woman suffering from mycosis fungoides of the skin Wellcome L0061976.jpg 4 897 3 793; 2,14 MB Sezary[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:

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.

Sex distribution
Age distribution

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

Article

  1. Willemze R, Meijer CJ. Rationale of a new classification for the group of primary cutaneous lymphomas. Semin Cutan Med Surg 2000; 19:71-7.
  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.

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Last updated: 2019-07-11 21:34