Cutaneous lymphomas
Among the cutaneous lymphomas affecting T cells, there are mycosis fungoides and Sèzary's syndrome, two primary neoplasms that occur at the skin level, developing an asymptomatic but incurable disease. In this regard, in most cases, therapies fail to heal patients suffering from mycosis fungoides or Sèzary's syndrome, but the treatments are useful to lighten the symptoms.
therapies
In the initial stage, the therapies are almost identical for both cutaneous lymphomas: the patients' skin is generally treated with phototherapy, locally acting corticosteroids, radiotherapy, chemotherapy, immunomodulators or Bexarotene (topical application or systemic intake).
Usually, systemic action therapies to reduce the toxicity of mycosis fungoides and Sèzary syndrome are exploited in the stages in which the lymphoma is transformed and in the more advanced stages; however, in some patients, systemic therapy is more advantageous than the topical one, already at the onset of the neoplasm. It should also be remembered that those suffering from mycosis fungoides and Sèzary's syndrome very often respond differently to the treatments administered.
Mycosis fungoides and Sèzary syndrome are two closely related skin neoplasms: according to some authors, Sèzary syndrome appears to be the leukemic erythrodermic 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 affecting T lymphocytes. If in the first stage mycosis fungoides begins at the level of the skin, in advanced stages it can spread to the lymph nodes and metastasize in the internal organs . The affected subject 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 onset with eczema and itchy rash, whose lesions are very similar to those caused by fungal infections. The sores, in the first period, are not constant: in fact, they highlight acute alternations with stationary phases or, in some cases, to regressions of the disorder, which then reappears.
From the histological point of view, the cells appear as agglomerates, known as Paurtier's micro-abscesses, both on the skin surface and in the intraepidermal layers. If the disease evolves, the skin lesions may worsen causing swelling associated with a widespread condition of erythroderma (atopic dermatitis, redness, scaling, erythema, psoriasis).
Mycosis fungoides mainly affects the over-sixties, 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 definitely aggressive.
Sèzary syndrome
We had mentioned that the two lymphomas are closely related: in fact, if in the eczematous, scaly and erythematous lesions traces of neoplastic cells (called cerebriformes) are identified, one speaks of Sèzary syndrome: for this reason, this syndrome is known as the leukemic variant of mycosis fungoides.
Around 1950, Prof. Sèzary noted the presence of large anomalous cells, located in the skin and blood of subjects complaining of erythroderma, splenomegaly (abnormal increase in the volume of the spleen) and lymphadenopathy (enlarged lymph nodes), the three factors pivot to identify the disease. The syndrome of Sèzary, which bears the name of the discoverer, could manifest itself presenting, in the first stage, only one of the three symptoms (eg splenomegaly): in most cases, in a relatively short period, the other two conditions are also revealed .
In general, subjects suffering from Sèzary syndrome do not respond positively to therapies: it has been statistically recorded that patients with mycosis fungoides present a better prognosis than those affected by Sèzary syndrome.
The connections between the two skin neoplasms have been demonstrated: the advanced stages of mycosis fungoides are in fact characterized by erythroderma, a cutaneous condition that defines Sèzary Syndrome. Another analogy emerges from the histological study: the tumor cells that spread in both cutaneous lymphomas derive from the thymus.
Furthermore, skin lesions and malignant infiltrations in the lymph nodes represent another analogy between the two diseases, as well as the histological characteristics of malignant cells, almost equal, both in Sèzary's 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 cutaneous pathologies 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's syndrome. Considering that the subjects suffering from these pathologies have a family predisposition to tumors, it seems that genetic factors are more implicated in the onset of mycosis fungoides and in Sèzary's syndrome compared to other lymphomas.
