Mycosis fungoides

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

Clinical variants

 * Folliculotropic MF
 * Pagetoid reticulosis
 * Granulomatous sagging skin
 * 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.

Related articles

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