Mycosis fungoides

Medically reviewed by The Dermatologists and written by Dr. Alexander Börve

Common
3000 cases in the US per year.

  • Requires medical diagnosis – Clinical picture, Histology, immuno-histochemistry and PCR
  • Symptoms: Itchy eczema-free plaque 
  • Location: The lower abdomen and gluteal, areas that are rarely exposed to sunlight
  • Treatment: PUVA, Chemotherapy
 
Ages Affected

Men over 60 are twice as likely to have it. It is very rare in younger people

Death Rate 

Untreated, the disease undergoes the stages (eczema-free plaque – infiltrate / tumor – lymphadenopathy / metastasis) and leads to death within 5-20 years.

Incident Rate

0.3 per 100 000

Mycosis fungoides, also known as Alibert-Bazin syndrome or granuloma fungoides, is a skin cancer where a form of white blood cells called T lymphocytes becomes malignant. The disease belongs to the group of cancers called lymphoma. Mycosis fungoides occurs very rarely, only about 3000 new cases annually in the US (0,3/100 000). Men make up two-thirds of patients and the condition is more common among dark-skinned people.

 

Symptoms and signs

Dry, slightly scaly, well-defined skin changes.

The skin changes are usually localized buttock region. involve the patches evolving into plaques distributed over the entire body. The later stages eventually occur in other areas, and sometimes thickenings of the skin are formed in palms and soles of the feet. In some, it can develop alternating pigmentation with dry skin, uncharacteristic eczema changes or blushing skin all over the body. An unusual variant attacks the scalp and causes hair loss. In some cases, the changes are thicker or form tumors. Enlarged lymph nodes may also occur.

Causes

The cause behind mycosis fungoides is unknown. Microbes, professional exposure and genetic mutations have been proposed as causative factors, but documentation is lacking.

 

Diagnosis

The disease usually affects older men (ratio 2:1 to women). It takes an average of about five years before a diagnosis is reached. The condition can be accompanied by itching, which often improves during the summer months. A skin biopsy show typical weaving changes of the dermis under the microscope, with special immunological staining, the lymphocytes can be demonstrated.

  • Clinical picture
  • Histology
  • Immunohistochemistry
  • PCR

 

Treatment 

During the first phase of the disease, cortisone ointments or UV treatment are used to limit symptoms. At a later stage, strong cortisone ointments and PUVA – the oral drug psoralen and then UV radiation can also be used.

It may also be necessary to provide strong vitamin A-like tablets and injections of the substance interferon, which affects the immune system. When tumors appear in the skin, PUVA, interferon, radiation and sometimes chemotherapy are given.

Mechlorethamine/chlormethine an alkylating agent is a topical gel for the treatment of mycosis fungoides‐type cutaneous T‐cell lymphoma.

 

Prognosis

Early diagnosis is key. In most cases, at so-called mild stages, the prognosis is very good and you live a normal long life. The presence of tumors of the skin gives a much worse prognosis, as well as conditions with widespread redness and peeling, known as erythrodermia.

 

Sources

Survival in Mycosis Fungoides and Sezary Syndrome: How Can We Predict Outcome? Scarisbrick J.J. J Invest Dermatol. 2020 Feb;140(2)

Mycosis fungoides and Sézary syndrome: 2019 update on diagnosis, risk-stratification, and management. Hristov AC, Tejasvi T, Wilcox RA. Am J Hematol. 2019 Jul 16

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