Mycosis fungoides

Mycosis fungoides knee

Mycosis fungoides

Mycosis fungoides – June 1902

Mycosis fungoides – October 1902

Mycosis fungoides is an epidermoid cutaneous T-lymphoma characterized by the proliferation of small or medium-sized lymphocytes. It primarily affects the skin, but may gradually develop to form squamous foci with gradual tumor formation, lymph node and internal organ involvement.

Clinical picture

Development takes years (without treatment 10-15 years). Gradual development in stages.

1. Premycotic stage: in the form of so-called parapsoriasis en plaques (formation of peeling areas on the trunk) or in the form of poikilodermic (telangiectasia and depigmentation).
2. Infiltration stage: tumors with decaying ulcers are formed.
3. Tumor stage: tumors formed, some with a sunken cap - shaped surface (hence the name). Internal organs are also affected at this stage.

Manifestations are usually asymptomatic, but when the cutaneous nerves are affected itching 'does not respond to antihistamines.

Clinical variants

• Folliculotropic MF
• Pagetoid reticulosis
• Granulomatous sagging skin
• Sézary's syndrome

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Sézary's syndrome

Sézary's syndrome

Sézary's syndrome is a leukemic form of Mycosis fungoides along with erythroderma, generalized lymphadenopathy and hepatosplenomegaly. Clinically, there is red-purple discolouration of the skin, peeling of the entire skin, loss of nails and alopecia, and persistent itching.

Histopathological picture

Epidermotropism and Pautrer's microabscesses in the epidermis.

Therapy

Photochemotherapy (PUVA) for skin involvement only. Furthermore, applications of carmustine, corticoids and retinoids. Interferon α is used in case of major exposure.

Course and prognosis

Chronic course for decades. Prognosis according to the extent of disability.

Links

Related articles

• Lymphoma
• Cutaneous lymphoma
• CNS lymphomas
• Hodgkin's lymphoma

References

• ŠTORK, Jiří, et al. Dermatovenerologie. 1. edition. Prague : Galén, Karolinum, 2008. ISBN 978-80-7262-371-6.