Algorithm for diagnosing earlystage mycosis fungoides. Mycosis fungoides primarily localized to the palms and soles is rare and has been previously reported as cutaneous lymphoma in four patients or as woringerkolopp disease in eight patients. Mycosis fungoides mf is the most common subtype of cutaneous tcell lymphoma and may rarely infiltrate the ocular structures. Mycosis fungoides mf is a lowgrade cutaneous lymphoma. This stage can either have cancer that has affected the lymph nodes while the blood may have the presence of cancerous lymphocytes or the stage can also be described as having cancerous lymphocytes in the blood with enlarged. Mycosis fungoides mf is the most common primary cutaneous tcell lymphoma. Mycosis fungoides mf is the most common primary cutaneous tcell lym phoma variant and is closely related to a rare leukemic variant, sezary syn. Mf patients at risk of disease progression can now be identified and an international consortium has been established to address the prognostic relevance of specific biologic factors and. Patients with stages iib and above were associated with diseasespecific death p 0. Retrospective inception cohort study at a university medical center referral clinic. Mycosis fungoides mf, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma.
All cases of mf are ctcl, but not all ctcl cases are mf. To determine, in patients with mycosis fungoides and sezary syndrome, the incidence of infections, the importance of nosocomial infections, and the epidemiologic factors associated with cutaneous and visceral infections. Clinical, clinicopathologic, and histologic variants of mycosis fungoides. Stage 4a is described as mycosis fungoides with tumors that can cover any amount of the skin surface. D, pigmented purpuriclike dermatitis on the left inner thigh. Mycosis fungoides mf is the most common primary cutaneous t cell lymphoma ctcl, which comprise a heterogeneous group of nonhodgkins lymphoma. How i treat mycosis fungoides and sezary syndrome american. Mycosis fungoides, mf, is the most common form of cutaneous t cell lymphoma. Global patterns of care in advanced stage mycosis fungoidessezary. C, dyschromic patches with fine scale on the right upper arm. Mycosis fungoides an overview sciencedirect topics. In subsequent phases, patients develop plaques and.
The original staging system for ctcl was based on the tumour, lymph node, metastasis tnm system devised by bunn and lamberg in 1979. Mycosis fungoides involving the lungs detected by 18ffdg petct. Intralesional interferon in the treatment of early mycosis fungoides. Pdf mycosis fungoides mf, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous. Mycosis fungoides palmaris et plantaris jama dermatology. Furthermore, oral and zygomatic folliculitis was identified. Mycosis fungoides is a form of cutaneous lymphoma in which the skin is infiltrated with neoplastic t cells. Mycosis fungoides is rarely cured, but some people stay in remission for a long time. This condition is one of the most common types of tcell lymphoma.
Clinicopathologic variants of mycosis fungoides actas. Mycosis fungoides is a clinicopathologic term which describes a neoplasm of cerebriform t lymphocytes that form plaques and tumors. Cbc with manual differential, liver function tests, ldh, comprehensive chemistries. We further suggest that mycosis fungoides arises in a background of. A 72yearold male patient with a history of cutaneous mycosis fungoides, in clinical remission after treatment, underwent fluorine18fluorodeoxyglucose. Twelve patients with early mycosis fungoides were enrolled in a randomized, doubleblind, placebocontrolled study. Mycosis fungoides often develops slowly over many years, often presenting with a generalized erythroderma, skin patches, or skin plaques.
Mycosis fungoides follows a slow, chronic indolent course and very often does not spread beyond the skin. Mycosis fungoides often has a long natural history, and the median duration from the onset of skin symptoms to a diagnosis of mycosis fungoides may be 5 years or longer. Due to highly variable presentations and the sometimes non. Mycosis fungoides mf is the most common form of cutaneous tcell lymphoma ctcl. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl and represents nearly 50% of all primary cutaneous lymphomas. Infections complicating mycosis fungoides and sezary. Advancedstage mycosis fungoides mfsezary syndrome ss patients are weighted by an unfavorable prognosis and share an. Stage 4 mycosis fungoides is divided into stage 4a and stage 4b. Revisiting imiquimod for treatment of folliculotropic mycosis.
Mycosis fungoides mf is the most common primary tcell cutaneous lymphoma and accounts for almost 50% of all primary cutaneous lymphomas. Pdf mycosis fungoides mf is the most common group of cutaneous tcell lymphomas. Mycosis fungoides presentation in emergency bentham open. Mycosis fungoids, cutaneous lymphoma, tcell lymphoma, breast swelling. Mycosis fungoides, the most common subtype of cu taneous tcell lymphoma, displays a broad spectrum of clinical, histological and phenotypic variants with. Full text management of mycosis fungoidestype cutaneous tcell. Mycosis fungoides is characterized by a scaly, red rash that develops on the skin, particularly on areas that are not usually exposed to the sun. It presents in the lower extremities as erythematous, pruritic patches, plaques, and ulcerations or tumors. Although the terms mf and ctcl are often used interchangeably, this can be a source of confusion. Mycosis fungoides american journal of clinical pathology. Mycosis fungoides genetic and rare diseases information. Mycosis fungoides mf is the most common variant of cutaneous t cell lymphoma ctcl and is primarily characterized by the proliferation of. Cytokine profile whereas sezary syndrome expresses a th2type profile. In the latest stages of the disease, the tumor stage, mf can evolve into a systemic form.