Cutaneous lymphomas
Among the cutaneous lymphomas affecting T cells, mycosis fungoides and Sèzary syndrome stand out, two primary neoplasms that manifest themselves in the skin, developing an asymptomatic but incurable disease. In this regard, in most cases, the therapies fail to cure patients suffering from mycosis fungoides or Sèzary syndrome, but the treatments are useful to alleviate the symptoms.
Therapies
In the initial stage, the therapies are almost identical for both cutaneous lymphomas: the skin of patients is usually treated with phototherapy, locally acting corticosteroids, radiotherapy, chemotherapy, immunomodulators or Bexarotene (topical application or systemic administration).
Usually, systemic therapies to reduce the toxicity of mycosis fungoides and Sèzary syndrome are used in the stages in which the lymphoma is transformed and in the most advanced ones; however, in some patients, systemic therapy is more advantageous than topical therapy, already at the onset of the neoplasm. It should also be remembered that patients with mycosis fungoides and Sèzary syndrome respond very often in a different way to the treatments administered. .
Mycosis fungoides and Sèzary syndrome are two closely related skin neoplasms: according to some authors, it seems that Sèzary syndrome is the leukemic erythrodermal variant of mycosis fungoides.
Let us now try to clarify the two malign conditions in more detail.
Mycosis fungoides
Mycosis fungoides is a cutaneous lymphoma that is included in the category of "non-Hodkgins" lymphomas involving T lymphocytes. If in the first stage the mycosis fungoides begins in the skin, in the advanced stages it can spread to the lymph nodes and metastasize in the internal organs . The affected person may not even notice the presence of mycosis fungoides, a subtle disease that can remain hidden for many years and be confused with other pathologies; even a biopsy could give uncertain and confused results. Suddenly, mycosis fungoides makes its debut with eczema and pruritic erythema, the lesions of which are very similar to those caused by fungal infections. The sores, in the first period, are not constant: in fact, there are acutations alternating with stationary phases or, in some cases, with regressions of the disorder, which then reappears.
From the histological point of view, the cells appear as agglomerates, known as Paurtier micro-abscesses, both on the skin surface and in the intraepidermal layers. If the disease evolves, the skin lesions can worsen causing swelling associated with a widespread condition of erythroderma (atopic dermatitis, redness, scaling, erythema, psoriasis).
Mycosis fungoides mainly affects people over the age of sixty, with a double incidence rate in men compared to women; mycosis fungoides was recorded in 0.5% of skin cancers in the United States.
Mycosis fungoides is the most frequent form of cutaneous lymphoma, although it remains a rare type of non-Hodgkin's lymphoma: this means that cutaneous lymphomas are rare, but decidedly aggressive.
Sèzary syndrome
We had mentioned that the two lymphomas are closely linked: in fact, if traces of neoplastic cells (called cerebriformes) are identified in eczematous, squamous and erythematous lesions, we speak of Sèzary syndrome: precisely for this reason, this syndrome is known as the leukemic variant of mycosis fungoides.
Around 1950, Prof. Sèzary noticed the presence of large abnormal cells, located in the skin and blood of subjects who complained of erythroderma, splenomegaly (abnormal increase in the volume of the spleen) and lymphadenopathy (enlarged lymph nodes), the three factors pivotal to detect the disease. Sèzary syndrome, which bears the name of the discoverer, could manifest itself by presenting, in the first stage, only one of the three symptoms (e.g. splenomegaly): in most cases, in a relatively short period, the other two conditions are also revealed .
Generally, subjects suffering from Sèzary syndrome do not respond positively to therapies: it has been statistically recorded that patients with mycosis fungoides have a better prognosis than those affected by Sèzary syndrome.
The connections between the two cutaneous neoplasms have been demonstrated: the advanced stages of mycosis fungoides are in fact characterized by erythroderma, a skin condition that defines the Sèzary syndrome. Another analogy emerges from the histological study: the cancer cells that spread to both cutaneous lymphomas derive from the thymus.
Furthermore, the cutaneous lesions and the malignant infiltrations in the lymph nodes represent another analogy between the two diseases, as well as the histological characteristics of the malignant cells, almost the same, both in Sèzary syndrome and in mycosis fungoides.
The probability that the neoplasm remains limited to the skin is not high, on the contrary; it is very frequent that, following the onset of one - or both - of the aforementioned diseases, extra skin diseases develop which involve, above all, the spleen, lungs, liver, stomach and intestine.
As with most lymphomas, the etiological picture is uncertain: environmental factors, immunological alterations, infections and other tumors are possible factors that could somehow favor the onset of mycosis fungoides and Sèzary syndrome. the subjects affected by these pathologies have a familial predisposition to tumors, it seems that genetic factors are more involved in the onset of mycosis fungoides and in Sèzary's syndrome than other lymphomas.
