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.
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].
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.