It is hypothesized that the genetic mutations in these cancer cells lead to increased growth and escape from programmed cell death.Additionally, the disease is an unusual expression of CD4 T cells, a part of the immune system. These T cells are skin-associated, meaning they are biochemically and biologically most related to the skin, in a dynamic manner. Mycosis fungoides is the most common type of cutaneous T-cell lymphoma (CTCL), but there are many other types of CTCL that have nothing to do with mycosis fungoides and these disorders are treated differently. Diagnosis
Diagnosis often requires a combination of clinical and pathological studies. Diagnosis is sometimes difficult because the early phases of the disease often resemble inflammatory dermatoses (such as eczema, psoriasis, lichenoid dermatoses including lichen planus, vitiligo, and chronic cutaneous lupus erythematosus), as well as other cutaneous lymphomas. Several biopsies are recommended, as the key microscopic features are often absent in early MF, and a complete diagnosis requires a combination of clinical and histological study. Furthermore, long periods of treatment can alter the biopsy findings, making it difficult to distinguish from other inflammatory dermatoses. Childhood Mycosis fungoides (MF) makes up 0.5% to 7.0% of cases. Although data on childhood MF is limited, a 2021 systematic review observed that there is a significant delay in the diagnosis of childhood MF which may negatively affect a child’s prognosis. Notably, most pediatric persons with MF present with early-stage disease.
In the tumorous stage a dense infiltrate of medium-sized lymphocytes with cerebriform nuclei expands the dermis. Accurate staging of mycosis fungoides is essential to determine appropriate treatment and prognosis. Staging is based on the tumor, node, metastasis, blood (TNMB) classification proposed by the Mycosis Fungoides Cooperative Group and revised by the International Society for Cutaneous Lymphomas/European Organization of Research and Treatment of Cancer. This staging system examines the extent of skin involvement (T), presence of lymph node (N), visceral disease (M), and presence of Sezary cells in the peripheral blood (B).Most patients with mycosis fungoides have early-stage disease (Stage IA-IIA) at the time of their initial diagnosis. People with early stage disease that is primarily confined to the skin have a favorable prognosis. People with advanced stage (Stage IIB-IVB) are often refractory to treatment and have an unfavorable prognosis. Treatment options for people with advanced stage disease are designed to reduce tumor burden, delay disease progression, and preserve quality of life. Treatment
The most commonly recommended first-line treatment for mycosis fungoides is psoralen plus ultraviolet A (PUVA therapy). PUVA is a photochemotherapy that involves topical or oral administration of the photosensitizing drug psoralen followed by skin exposure to ultraviolet radiation. Systemic treatments of mycosis fungoides often lead to resistance; as such, additional treatment options are often necessary in advanced disease.Other treatments that have been suggested, however, larger and more extensive research is needed to identify effective treatment strategies for this disease.