Poikilodermatous mycosis fungoides

Mycosis fungoides, also known as Alibert-Bazin syndrome or granuloma fungoides, is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time. Symptoms include rash, tumors, skin lesions, and itchy skin.aggressive: Sézary disease Mycosis fungoides, also known as Alibert-Bazin syndrome or granuloma fungoides, is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time. Symptoms include rash, tumors, skin lesions, and itchy skin. While the cause remains unclear, most cases are not hereditary. Most cases are in people over 20 years of age, and it is more common in men than women. Treatment options include sunlight exposure, ultraviolet light, topical corticosteroids, chemotherapy, and radiotherapy. Typical visible symptoms include rash-like patches, tumors, or lesions. Itching (pruritus) is common, perhaps in 20 percent of patients, but is not universal. The symptoms displayed are progressive, with early stages consisting of lesions presented as scaly patches. These lesions prefer the buttock region. The later stages involve the patches evolving into plaques distributed over the entire body. The advanced stage of mycosis fungoides is characterized by generalized erythroderma, with severe pruritus and scaling. The cause of mycosis fungoides is unknown, but it is not believed to be hereditary or genetic in the vast majority of cases. One incident has been reported of a possible genetic link. It is not contagious, although some research suggests that the Human T-lymphotropic virus is associated with this condition. 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 is sometimes difficult because the early phases of the disease often resemble eczema or even psoriasis. Several biopsies are recommended, to be more certain of the diagnosis. The criteria for the disease are established on the skin biopsy: Pautrier microabcesses (i.e., Aggregates of four or more atypical lymphocytes arranged in the epidermis) are characteristic of the disease but are generally absent. To stage the disease, various tests may be ordered, to assess nodes, blood and internal organs, but most patients present with disease apparently confined to the skin, as patches (flat spots) and plaques (slightly raised or 'wrinkled' spots). Peripheral smear will often show buttock cells.