Management

Mycosis fungoides typically follows a slow progression from patches to plaques to tumours. While initially localised to the skin, the disease may become generalised requiring systemic therapy.

All Stages

Good skin care is essential to prevent complications of the disease from developing. These include:

  • Moisturisers
  • Topical corticosteroids to minimise dermatitis and itch
  • Antibiotics to treat complicating skin infections

Patch and Plaque Stage

There are numerous treatment options available, none of which are curative unless the disease is truly localised.

Topical Ointments

Corticosteroid ointments are useful for both patches and plaques. They are thought to induce apoptosis in the malignant lymphocytes.
Mechlorethamine, or nitrogen mustard, is available as an ointment. It works by causing DNA cross links (similar mechanism to cisplatin) It can induce long standing remission if applied to patches or plaques.
Retinoids including beraxotene cause apoptosis in malignant lymphocytes. Rash and itch are common side effects.

Phototherapy

Photons in the ultraviolet energy range are highly effective at inducing apoptosis in lymphocytes. This makes UV therapy a useful option in treating mycosis fungoides. There are two types of UV energy:

  • UVA, which has photon energies just higher than visible light, and greater depth penetration in the skin (affects epidermis and dermis). Apoptosis is caused through the activation of caspases.
  • UVB, which has photon energies above UVA, has less penetration of skin and only affects the epidermis. Apoptosis is caused by activation of death receptors (Fas)

Summary

Localised patches and plaques treatable with:

  • topical steroids
  • PUVA
  • superficial radiotherapy / electron beam radiotherapy (electron beam RT recommended)

Extensive patch/plaque disease treatable with total skin electron radiotherapy.

Localised tumours treatable with:

  • Electron beam radiotherapy
  • Systemic therapy (eg. retinoids,

Widespread tumours require:

  • Total skin electron radiotherapy
  • Systemic therapy as per localised tumours

Nodal disease treatable with standard photon beam radiotherapy or systemic treatment.

Sezary Syndrome