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Mycosis Fungoides (MF) is a T-cell lymphoma initially presenting in the skin. Many clinical variants exist including follicular, vesicular, poikilodermic, hypo- and hyperpigmented, ichthyosiform and pigmented purpura-like lesions. We present an unusual variant in a case of poikilodermic MF.
Mycosis fungoides (MF) treatment must be chosen only after staging extent of lymphoma. Stage I and II diseases respond best to either topical nitrogen mustard, photochemotherapy (PUVA), or electron beam. Chemotherapy of stage III and IV MF has only limited remission rates. Combinations of therapies and experimental approaches, such as monoclonal antibodies, may give longer survivals.
Mycosis fungoides (MF) is an uncommon T-cell lymphoma which characteristically involves the skin. Two patients with MF are described who developed fatal complications secondary to involvement of the gastrointestinal tract. One developed malabsorption due to small intestinal involvement; the other had a massive haemorrhage from an ulcerated nodule of tumour in the stomach. The potential for extracutaneous spread is discussed, and it is emphasized that bowel infiltration should be considered in any patient with MF who develops gastrointestinal symptoms or complications.
Mycosis fungoides (MF) is, on extremely rare occasions, associated with vesiculobullous eruptions. This report describes clinical, histological and immunohistochemical findings of a case of MF with vesiculobullous lesions. Characteristic features of our case included: bullous lesions evolving rapidly in 12-24 h with severe itch on erythematous and normal-appearing skin; a positive Nikolsky sign; an intraepidermal blister; direct and indirect immunofluorescence stainings were all negative with IgG, IgA, IgM and C3, and atypical lymphocytes in the infiltrates were of helper/inducer T cell type.
Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. It represents a spectrum of disorders with preferential involvement of the skin and, after a period of time, may progress to involve lymph nodes, peripheral blood and/or visceral organs. MF progresses in three clinical phases: the premycotic, mycotic and tumor stages. Each stage has different characteristics and goals for treatment. Treatment such as chemotherapy and radiotherapy in the earlier stages is more effective and may be curative, while treatment in the last stage is less effective and the intent is to alleviate symptoms. The case study illustrates topical treatment using sheet hydrogel to absorb exudate, contain odor and reduce pain upon dressing removal for a patient in the tumor stage of MF.
Mycosis fungoides is the most common cutaneous lymphoma in the US, and it is increasing rapidly in both incidence and mortality. Our knowledge of its prognosis derives primarily from case series, which are subject to possible selection bias and other limitations. The current analysis examines trends in survival and prognostic factors in data from nine population-based cancer registries in the US. Among the 650 cases followed with known dates of diagnosis and no history of prior malignancy, the median survival time was 7.8 years. Advanced age, black race, prior malignancy, and Sezary syndrome presence at the time of diagnosis were each independently associated with poor prognosis. Fatality was not influenced by sex or geographic area. There was no consistent evidence of improved survival with time over the 12-year study period.
Mycosis fungoides (MF) is a cutaneous lymphoma of presumed T-helper cell origin. It usually presents with patches and infiltrated plaques, but a wide range of atypical forms have also been described. We report a 48-year-old man who had a 2-year history of a pruritic, warty plaque on the right palm with surrounding poikilodermatous changes. He subsequently developed additional macules of poikiloderma atrophicans vasculare (PAV) on the right upper limb and abdomen. Biopsies from the arm showed poikilodermatous changes, and the warty plaque had features of MF. Verrucous or hyperkeratotic lesions appear to be an uncommon presenting feature of MF with few case reports in the literature to date. This presentation may be associated with considerable diagnostic delay, and MF should be considered in the differential diagnosis of acquired verrucous lesions. This case also illustrates that PAV may herald cutaneous malignancy, and that patients with this condition require close follow up.
Mycosis fungoides (MF) is a type of cutaneous hyperproliferative T-cell disorder that may be localized. Although there is considerable controversy regarding whether MF may originate as a non-neoplastic condition, or even whether MF is a neoplastic condition until late in its course, we have seen a few cases undergo what appeared to us to be a clear progression from an inflammatory disorder to MF. We now report a 32-year-old man with MF most prominent on his right flank and buttock who developed his patches several weeks following, and in the precise locations in which he had experienced, exposure to toxic chemicals in an industrial accident. Because of this history, and because all lesions were transient except for these sites, these permanent lesions were treated with local surgical excision. There was no recurrence of disease at the treated sites, and the progression of MF markedly slowed following surgery, although he has continued to experience multi-focal transient recurrent disease, controlled by a combination of topical and systemic treatments, until the present time. Destructive methods such as excisional surgery or carbon dioxide laser may be considered a therapeutic option for localized MF.
Mycosis fungoides (MF) is a rare form of cutaneous T cell lymphoma suspected of having a viral etiology. As in adult T cell leukemia, the virus involved may be human T lymphotropic virus type 1 (HTLV-1). We cultured the peripheral blood mononuclear cells (PBMC) of 29 patients with MF HTLV-1 seronegative by enzyme-linked immunosorbent assay and Western blot. The presence of reverse transcriptase (RT) and p24 antigen was investigated in the concentrate supernatant of the culture. The DNA of all studied patients was submitted to polymerase chain reaction and Southern blot analysis using primers and probes recognizing the tax region of HTLV-1/2 and the pol region of HTLV-1. 10 of 29 patients were found positive to HTLV-1, whereas they were always negative to RT and p24. The same results were confirmed in double blind after 6 mo. Our findings suggest HTLV-1 may be involved in the etiology of MF, at least in certain cases.
Mycosis fungoides (MF) is the most frequent variant of cutaneous T-cell lymphomas (CTCLs). MF primarily involves the skin initially with patches and plaques. In later stages, cutaneous tumors develop and tumor cells may spread to lymph nodes and finally to visceral sites. Here, we describe an animal model for MF in immune-deficient nude mice, using the CTCL cell line MyLa. Subcutaneous transplantation of MyLa cells leads to the formation of cutaneous tumors in 80% of the mice (50/60 total). Spread of tumor cells to visceral sites was detected by immunohistochemistry and polymerase chain reaction (PCR)-based detection of specific T-cell receptor-gamma rearrangement. MyLa cells were found circulating in the blood, lymph nodes, and in blood vessels of heart, kidney, lung, and liver. In lung and liver tissue, tumor cells presented perivascular invasion, but no large secondary tumors developed. The nude mouse model described here will be a valuable test system for new therapeutic approaches for the treatment of MF and opens the unique opportunity to study the disease in vivo.
Mycosis fungoides (MF) and Sézary syndrome (SS) are uncommon neoplasms of the lymphoreticular system with distinct clinical, histologic, and immunologic features. Based on the thymus-derived nature of the neoplastic cells, MF and SS are both classified as cutaneous T-cell lymphoma. While substantially greater understanding of MF and SS has been made possible, the exact mechanism for the initiation of either disease is still unknown. The possible involvement of environmental factors as well as viral etiology, i.e., retroviruses, has been suggested. In order to investigate the possible role of HLA-associated variations in genetic susceptibility, 74 patients with histologically documented MF were typed for HLA-A, -B, and -C antigens. Half of these patients were also typed for HLA-DR antigens. An increase in DR5 was the only statistically significant deviation in HLA antigen frequencies in these patients (53% in MF as compared with 20% in controls). An increased frequency of HLA-DR5 has also been associated with scleroderma and juvenile rheumatoid arthritis both of which have immunologic alterations. Also HLA-DR5 has been associated with renal cell carcinoma and Kaposi's sarcoma. The association of MF with DR5 suggests that some individuals with the DR5 antigen may be at higher risk for virally initiated and/or neoplastic diseases possibly through an HLA-linked defect in the immune system.
Mycosis fungoides (MF) is a cutaneous T-cell lymphoma characterized in its early stages by a superficial band-like infiltrate with epidermotropism of lymphocytes without particularly atypical cytologic features. Even though clinicopathologic presentation is diagnostic in typical cases, some inflammatory skin disorders can simulate the histopathologic features of early MF. In this study we present data on 9 patients affected by lichen sclerosus (LS) (M:F ratio 8:1; age range 7-75 years; mean age 31.3 years; median age 13 years), who presented with histopathologic features simulating early lesions of MF. The histopathologic picture was characterized in all cases by a dense, band-like infiltrate of lymphocytes within the superficial dermis, with exocytosis of lymphocytes within the lower part of the epidermis. The papillary dermis was expanded and showed focally coarse bundles of collagen simulating MF. The typical signs of LS were either absent or present only focally. Molecular analyses of the TCRgamma gene rearrangement performed with the polymerase chain reaction (PCR) technique revealed a polyclonal smear in eight cases, and a monoclonal band in one. Our study shows that LS can present with histopathologic features simulating early MF. Especially in cases revealing a monoclonal population of T lymphocytes by PCR, the correct diagnosis may be overlooked without proper clinical information and clinicopathologic correlation. Lichen sclerosus should be added to the list of cutaneous T-cell pseudolymphomas.
Mycosis fungoides (MF) is a rare form of cutaneous T-cell lymphoma that may be associated with human T-cell leukemia virus type I (HTLV-I) infection. Using the polymerase chain reaction, the HTLV-I pX region was constantly detected in the genomic DNA extracted from peripheral blood mononuclear cells (PBMCs) of an HTLV-I antibody-seronegative Egyptian MF patient enrolled in a study to isolate HTLV-I from North Africa. A CD4+ and interleukin-2 (IL-2) receptor-positive T-cell line was established when the phytohemagglutinin-stimulated PBMCs of that patient were maintained in IL-2-containing culture medium. The cell line (EMF) was initially IL-2 dependent and then became IL-2 independent after gradual withdrawal of the IL-2. The cells reacted positively with monoclonal antibodies specific for the HTLV-I Env or HTLV-I Gag proteins. Using the Southern blot analysis, HTLV-I provirus could be detected in the genomic DNA extracted from the EMF cells. Limited nucleotide sequence of the env region showed more than 95% homology between the EMF provirus and other known HTLV-I isolates. Western blot analysis of the cell lysates showed the expression of the HTLV-I structural proteins. These data imply that a transforming HTLV-I provirus may be present, at least in certain cases of MF, regardless of the presence or absence of the specific antibodies.
We conducted a critical review of hypopigmented mycosis fungoides in historical perspective with emphasis on criteria clinical and histopathologic for diagnosis of that lymphoma as they are set forth in every article ever written about it. Toward that end, we undertook analysis of each article in the medical literature that mentioned hypopigmentation in mycosis fungoides (34 in toto). Each was scrutinized regarding content, photographs of lesions clinical pictured, and photomicrographs. On the basis of all the information in the 34 publications available to us, we made a determination about which patients had mycosis fungoides without doubt, which surely did not, and which about whom no judgment could be made by us because too little data requisite for such a decision was provided, especially in terms of photographs of lesions clinical and of photomicrographs. To date, 106 patients with "hypopigmented mycosis fungoides" have been reported on. Features clinical and findings histopathologic in 23 of those 106 patients were sufficient to permit us to determine, with a high degree of confidence, whether or not a particular patient truly had mycosis fungoides. In our judgment, 19 patients did have mycosis fungoides, whereas at least four patients did not. In regard to the other 83 patients, the information provided by the authors simply was not sufficient to allow us to come to a decision that we could justify.
BACKGROUND: Interstitial mycosis fungoides (IMF) is a rare variant of mycosis fungoides that resembles the interstitial form of granuloma annulare and inflammatory morphea. IMF has received little attention in the literature. METHODS: Clinical, histological, immunophenotypical, and genotypical findings of five cases of IMF were reviewed. The histological and immunophenotypical findings were compared with those of eight cases of interstitial granuloma annulare and six cases of inflammatory morphea. RESULTS: Five patients with IMF presented with non-indurated, erythematous macules; ill-defined erythematous plaques with slight scale; and nodules on the trunk and proximal limbs. Two of five patients had a prior diagnosis of mycosis fungoides. Skin biopsies revealed a striking dermal interstitial infiltrate of lymphocytes with rare histiocytes that resembled the interstitial form of granuloma annulare or inflammatory morphea. Epidermotropic lymphocytes were present at least focally in all cases. A band-like lymphocytic infiltrate was observed in two of five cases. In contrast, many plasma cells and histiocytes were observed in cases of inflammatory morphea and interstitial granuloma annulare, respectively. With Movat-pentachrome stains, increased dermal mucin deposition was observed in two of five IMF cases, in all cases of interstitial granuloma annulare, and in one of six cases of inflammatory morphea. There was focal loss of elastic fibers in all cases of inflammatory morphea. Immunohistochemical studies of IMF highlighted a dominant population of T cells (CD3+) in the dermis and epidermis. In contrast, moderate numbers of B cells (CD20+) were admixed with T cells and plasma cells in inflammatory morphea. Almost equal numbers of histiocytes (CD68+) and T cells comprised the infiltrate of interstitial granuloma annulare. In two of five IMF cases, a clonal T-cell population was detected by PCR T-cell gamma gene rearrangement analysis. CONCLUSION: Mycosis fungoides occasionally presents as an interstitial lymphocytic infiltrate that mimics granuloma annulare and inflammatory morphea. Hematoxylin & eosin (H&E) findings alone can sometimes distinguish the three disorders. Immunophenotyping and genotyping may be helpful in difficult cases.
The authors describe a case of cutaneous and lymph node granulomas first reported as sarcoidosis. As skin sarcoidlike reactions disappeared, the development of typical histologic and immunopathologic features of cutaneous mycosis fungoides suggested granulomatous mycosis fungoides. This case illustrates the difficulties in differentiating true systemic sarcoidosis associated with mycosis fungoides from sarcoidlike reactions when extensive granulomas obscure the underlying cutaneous lymphoma. This report emphasizes the utility of immunohistochemical analysis to identify the early cutaneous T-lymphomatous infiltrate, initially admixed with epithelioid and giant cell granulomas. This technique also made it possible to characterize a Ki-1-positive anaplastic large-cell lymphoma when the transformation of mycosis fungoides into highly malignant lymphoma occurred in the lymph node.
Twenty patients with mycosis fungoides and four with Sézary's syndrome were treated with 13-cis-retinoic acid as single therapy in an initial dose of 1 to 2 mg per kg body weight in most cases. Complete remission in mycosis fungoides was obtained in six cases (33%) and partial remission in another ten cases (50%). No convincing response was observed in three cases, and progression of limited nodular lesions occurred in one case. In cases responding to treatment the first sign of remission was observed within two to four weeks. Our short-term experience is that the drug is effective in early as well as advanced stages of mycosis fungoides. Patients with Sézary's syndrome, however, did not respond to the same extent.
The frequency and prognostic importance of various characteristics of patients registered by the Mycosis Fungoides Cooperative Group between November 1974 and December 1977 are reported. Variables which were considered include demographic and historical factors, symptoms, extent of disease, and other physical findings. A staging system which is based on the extent of skin involvement and the number of nodal sites clinically involved is described. Finally, a description of therapeutic results to date for patients randomized into Mycosis Fungoides Cooperative Group protocols is presented.