Dualpositive cd4cd8 primary cutaneous peripheral tcell. Classic mycosis fungoides is divided into 3 stages. A tcell lymphoma of skin characterised by epidermal and dermal infiltration by small to medium size cells with cerebriform nuclei. What is the mortality and prognosis of cutaneous tcell. The aim of this study was to determine the contribution of the cd8cd3 ratio to the diagnosis of mycosis. They may spontaneously disappear, remain the same size, or. Expression of tcell receptor antigens in mycosis fungoides.
Cd8positive mycosis fungoides masquerading as pyoderma. Large cell transformation of mycosis fungoides mf is an uncommon phenomenon. Although the disease was initially termed pian fungoides, he later changed the name to mycosis fungoides. Cd8 positive mycosis fungoides and primary cutaneous aggressive epidermotropic cd8 positive cytotoxic tcell lymphoma.
The ratio of cd4 cd8 can be assessed and if there is restriction of one type this would indicate evidence of clonality. We present a case of cd30positive large cell transformation and discuss its possible pathophysiology. Local radiation for cutaneous tcell lymphoma other than. Lymphocytes in mycosis fungoides have a characteristic clear space halo around them and are seen lining up along the dermalepidermal junction. Mycosis fungoides usually present as scaly erythematous patches or plaques. Although most authors believe that determining the presence of this cell surface antigen has no prognostic value, others have observed a more indolent course. Immunophenotyping of the biopsy specimens was positive for cd3, cd4, and cd45ro and negative for cd8 and cd30. The term mycosis fungoides should be used only for classic cases, characterized by the evolution of patches, plaques, and tumors, or for variants with a similar clinical course. Mycosis fungoides is the most common type of cutaneous lymphoma, representing almost 50% of all lymphomas arising primarily in the skin. Ctcl diagnosis and staging cutaneous lymphoma foundation. Plaque and tumor stage cases, follicular mucinosis, sezary syndrome and small. Cd8positive mycosis fungoides and primary cutaneous aggressive epidermotropic cd8positive cytotoxic tcell lymphoma. The influence of the coexpression of cd4 and cd8 in cutaneous. Mycosis fungoides mf and sezary syndrome ss are the most common subtypes of cutaneous t cell lymphoma ctcl.
In advanced cases, ulcerated tumors and infiltration of lymph nodes by diseased cells. It is defined as a tumor composed of smallmediumsized, epidermotropic thelper lymphocytes but tcytotoxic variants are not uncommon and tumor cells may be mediumlarge in advanced stages. Cd4 is expressed in the majority of tcell lymphomas including mycosis fungoides, a common form of cutaneous tcell lymphoma 1. Mycosis fungoides pictures, staging, prognosis, symptoms. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma. N2 background granulomatous cutaneous tcell lymphoma gctcl is a rarely encountered entity. Mycosis fungoides which is also referred to as alibertbazin syndrome is a rare skin cancer. Mycosis fungoides and sezary syndrome also have a formal staging system proposed by the international society for cutaneous lymphomas iscl and the european organization of research and treatment of cancer eortc.
Pruritic erythematous macules or patches with telangiectasia and atrophy, may disappear spontaneously plaque stage. In individuals with mycosis fungoides, the skin becomes infiltrated with plaques and nodules that are composed of lymphocytes. Mycosis fungoides mf, the most common cutaneous tcell lymphoma, is usually indolent, but transformation to aggressive behavior occurs in about 10% of cases. Figure 3 large, atypical lymphocytes within the papillary dermis. Folliculotropic mycosis fungoides is a lowgrade lymphoma that accounts for around 4 in every 100 cases of skin lymphoma. Cd8 can be used in panels with cd4, cd56, tia1 to aid. Cd8positive mycosis fungoides and primary cutaneous. Value of the cd8cd3 ratio for the diagnosis of mycosis fungoides. Pruritic thichened plaques, may resemble psoriasis tumoral stage. The presence of predominant cd4 lymphocytes in conjunction with epidermotropism, dermal fibrosis, and cytological atypia favor mf over an inflammatory dermatosis. Although most authors believe that determining the presence of. There have been recent additions to the literature further elucidating certain features of primary cutaneous aggressive epidermotropic cd8.
Histopathologic staging of lymph nodes in mycosis fungoides and sezary syndrome a. The ratio of cd4cd8 can be assessed and if there is restriction of one type this would indicate evidence of clonality. Objectives to study and update the clinical characteristics and longterm outcome of our patients with mycosis fungoides mf and sezary syndrome ss, and to identify important clinical factors predictive of survival and disease progression. Mycosis fungoides pictures, staging, symptoms, treatment. Most often there are oval or ringshaped annular pink dry patches on covered skin. B and c, biopsy specimen of the lesion demonstrates an atypical lymphoid infiltrate involving the superficial and upper dermis. Mycosis fungoides mf is the most common form of primary cutaneous tcell lymphoma ctcl characterized by epidermotropic smalltomediumsized t lymphocytes with cerebriform nuclei. Mycosis fungoides and ctcl are often used interchangeably, which is imprecise, as mycosis fungoides is just one type of ctcl. Mycosis fungoides mf, a primary cutaneous tcell lymphoma, accounts for patch, plaque, tumor or nodule. In contrast, abnor mal cd3tcrb antigen expression was seen in 3 of 6 cases 50% of tumor stage mycosis fungoides. This condition is the most common variety of cutaneous t cell lymphoma ctcl. Mycosis fungoides mf, a primary cutaneous tcell lymphoma, accounts for patch stageearly mycosis fungoides mf is difficult to differentiate from benign dermatoses, despite several robust histologic criteria.
Mycosis fungoides mf and sezary syndrome comprise approximately 53% of. Mycosis fungoides is a rare form of tcell lymphoma of the skin cutaneous. Mycosis fungoides, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma. Often, the first stage goes on for many years and is characterized by a nonspecific dermatitis, which usually consists of patches and is often found on the lower trunk and buttocks. The diagnosis of mycosis fungoides was reinforced in all cases by the presence of a cutaneous dominant tcell clonal population. Cd4cd8 dualpositive mycosis fungoides, the american journal of. Extracutaneous dissemination in mf is observed in patch or plaque disease and in 3040% of patients with tumors or generalized erythrodermatous involvement. Value of the cd8cd3 ratio for the diagnosis of mycosis. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl and represents nearly 50% of all primary cutaneous lymphomas. Since double positive cd4cd8 phenotype is rarely reported in mycosis fungoides the.
Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma, which typically presents with erythematous patches and plaques, histopathologically characterized by superficial infiltrates of small to medium sized atypical epidermotropic t cells. Mycosis fungoides american journal of clinical pathology oxford. Oct 01, 2007 results of punch biopsies of the plaques revealed atypical lymphoid infiltration in the superficial dermis with focal exocytosis and pautrier microabscesses. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl. Symptoms include rash, tumors, skin lesions, and itchy skin. The disease begins with lightly erythematous patches that subsequently evolve.
Cd8 has been shown to be an important marker in the analysis of tcell mediated inflammatory dermatoses and is also useful for analysis of mycosis fungoides 24. Inflammatory dermatoses demonstrate a mixture of cd4 and cd8 positive cells in a proportion of approximately 34. Background granulomatous cutaneous tcell lymphoma gctcl is a rarely encountered entity. Mycosis fungoides mf is a clinical diagnosis that requires strong correlation with histopathologic and sometimes molecular findings to exclude benign inflammatory diseases, more aggressive primary cutaneous lymphomas, and extracutaneous lymphomas that can involve the skin. Cd8 positivity itself is not associated with a poor prognosis, and among cd8 positive patients with mycosis fungoides variant, calcl and other ctcls exhibit indolent behavior. Mycosis fungoides mf, a primary cutaneous tcell lymphoma, accounts for oct 29, 2019 the spectrum of tcell lymphoid proliferations of the skin varies from indolent to highly aggressive diseases and therefore an accurate pathological diagnosis is paramount. Results of punch biopsies of the plaques revealed atypical lymphoid infiltration in the superficial dermis with focal exocytosis and pautrier microabscesses. We present a case of cd30 positive large cell transformation and discuss its possible pathophysiology.
Mycosis fungoides variantsclinicopathologic features, differential diagnosis, and treatment rein willemze, md, phd m ycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl and accounts for approximately 50% of all primary cutaneous lymphomas. Cd30positive large cell transformation of mycosis fungoides. Patients diagnosed with stage ia mycosis fungoides patch or plaque skin disease limited to t. It generally affects the skin, but may progress internally over time.
Diagnosis and workup of early stage disease the ability to differentiate mf from mimickers permits early diagnosis and treatment, potentially improving longterm outcomes. Clinicopathologic findings of early mycosis fungoides. Conventional mf begins as eczematoid or psoriasiform patches and. Learn more about how it is diagnosed, treated and its prognosis. Mycosis fungoides variantsclinicopathologic features. Mycosis fungoides is the most common form of cutaneous tcell lymphoma ctcl. It exhibits a protracted clinical course with slow progression from slightly scaly skin lesions patches to infiltrated plaques and tumors. Mf is a mature t cell nonhodgkin lymphoma with presentation in the skin but with potential involvement of the nodes, blood, and viscera. Cd20 antigen in mycosis fungoides nejm journal watch. A, clinical photograph reveals erythematous patches of a left thigh that are variable in size and shape. Apart from the classic form of mycosis fungoides there are three other, rarer forms that behave slightly differently and look different under a microscope.
The image shows positive staining with cd3 depicting epidermotrophic string of pearls infiltrate along the basal layer. The mib1 labeling index was patch or plaque disease and in 3040% of patients with tumors or generalized erythrodermatous involvement. Patients diagnosed with stage ia mycosis fungoides patch or plaque skin disease limited to jan 29, 2009 as the behavior of the cases described by berti et al. Most studies include advanced lesions and data about early disease is limited. Mycosis fungoides mf is the most common primary cutaneous tcell lymphoma. This cancer characteristically affects the skin, causing different types. This condition may usually affect adults who are over 50 years of age but cases of children being affected have been reported 1, 2. The name mycosis fungoides is historical and confusing. Mycosis fungoides is an epidermotropic primary cutaneous tcell lymphoma characterized by infiltrates of small to mediumsized t lymphocytes with cerebriform nuclei. Cd4 can also be useful to highlight the degree of t cell infiltration into the epidermis. Mycosis fungoides mf is a clinical diagnosis and a diagnosis of exclusion, using clinical, histologic and molecular data must exclude inflammatory dermatitis in particular spongiotic, psoriasiform and lichenoid diseases,drug induced reactions and dyscrasias, cutaneous dissemination of extracutaneous peripheral t cell lymphoma.
Longterm outcome of 525 patients with mycosis fungoides. Mycosis fungoides american journal of clinical pathology. Figure 4 folliculotropic variant of mycosis fungoides. Mycosis fungoides nord national organization for rare.
Dualpositive cd4cd8 primary cutaneous peripheral tcell lymphoma. Mycosis fungoides mf is characterized by epidermotropic skin infiltration by atypical t lymphocytes. Mycosis fungoides is the most common example of cutaneous t cell lymphoma. In patch stage mycosis fungoides, the skin lesions are flat. Cd8 positivity itself is not associated with a poor prognosis, and among cd8positive patients with mycosis fungoides variant, calcl and other ctcls exhibit indolent behavior. Mycosis fungoides including sezary syndrome treatment pdq. Longterm outcome of 525 patients with mycosis fungoides and. Ctcl is a type of malignancy wherein the special kind of white blood cells called tlymphocytes become infectious and drawn or deposited to the skin. Extrafacial indolent cd8positive cutaneous lymphoid proliferation with.