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PubMed1999

Mycosis fungoides masquerading as an ischemic foot.

This case is of a man with bilateral lower-extremity ischemia and a solitary nonhealing ulcerated lesion of the right great toe. After revascularization with an aortobifemoral bypass, his right ABI increased from 0.5 to approximately 0.75, but the ulcerated toe lesion did not show signs of healing and instead progressed to a deeper ulceration exposing bone. Because of presumptive osteomyelitis, we performed a great toe amputation, and immunohistochemical analysis of the lesion revealed late plaque stage mycosis fungoides (MF). We present this case to alert the vascular surgeon to this diagnostic possibility when confronted with an apparent ischemic lesion and to describe what made this particular lesion suspicious for MF. To the best of our knowledge, this is the first case of MF presenting solely as an ischemic lesion.

Adult↗
PubMed2005

Chronic axonal-demyelinating polyradicular neuropathy associated with mycosis fungoides: a case report.

Case of a 58-year-old patient diagnosed as having mycosis fungoides (MF) who presented with sensory ataxia, limbs weakness and neurophysiological and laboratory signs indicative of autoimmune chronic mixed axonal-demyelinating sensory-motor polyradicular neuropathy. The possibility that MF may be associated with an immunomediated peripheral neuropathy, even in the absence of a direct invasion of the nerves and widespread visceral involvement, should be considered.

Axons↗
PubMed2005

Occupational exposures and mycosis fungoides. A European multicentre case-control study (Europe).

OBJECTIVES: Mycosis Fungoides (MF) is a rare disease with an occurrence indicating that occupational exposures may play a role. To estimate the association between MF and occupational exposures as measured by means of an job-exposure matrix (JEM). METHODS: A European multicenter case-control study was conducted from 1995 to 1997 and included seven rare cancers, one of which was MF. Patients between 35 and 69 years of age, diagnosed with MF (n=140), were recruited and the diagnoses were checked by a reference pathologist who classified 83 cases as definite, 35 cases as possible and 22 cases as not accepted. Among the 118 accepted cases, 104 cases were interviewed, of which 76 were definite cases. We selected population controls and colon cancer controls to serve all seven case groups. Altogether 833 colon cancer controls and 2071 population controls were interviewed. Based on the reported occupational experiences, a team of industrial hygiene specialists identified five potential exposures and developed an JEM. This JEM was used to estimate the odds ratios (OR) for MF as a function of these exposures. The JEM included aromatic and/or halogenated hydrocarbons (AAHs), chrome (VI) and its salts, electromagnetic radiations, silica and pesticides. RESULTS: Exposures to AHHs (OR 6.3, C.I 2.4-16.7 for male) were associated with a high MF risk. CONCLUSIONS: The study supports the hypothesis that some MFs have an occupational etiology but only a small fraction of exposed workers are apparently susceptible since the disease is so rare.

Adult↗
PubMed2004

Therapy for mycosis fungoides.

Treatment of mycosis fungoides (MF) is indicated to reduce symptoms, improve clinical appearance, prevent secondary complications, and prevent progression of disease, all of which may have an impact on survival. Treatment of MF includes topical and systemic therapies, which can be administered alone or in combination. Psoralen and ultraviolet A radiation is effective in early-stage MF, inducing complete remissions in most patients. Psoralen and ultraviolet A radiation may also be combined with low doses of interferon (IFN)-alpha to treat stage I/II disease. However, early aggressive therapy with radiation and chemotherapy does not improve the prognosis. Local radiotherapy or total skin electron beam irradiation has been used with success to control advanced skin disease. Extracorporeal photopheresis may also be used successfully, but it is not generally available. Once the disease becomes refractory to topical therapy, IFN-alpha single-agent or combination chemotherapy may be administered, but the duration of response is often less than 1 year and ultimately all patients will relapse and become refractory. Among chemotherapeutic agents, pentostatin, gemcitabine, and liposomal doxorubicin seem to be particularly effective. Response rates after combined modality therapy with total skin electron beam irradiation and chemotherapy/IFN-alpha appear similar to response rates of chemotherapy alone. Therefore, there is a great need for the further development of novel emerging treatment modalities, such as retinoids (ie, bexarotene) and immunotherapeutic agents (ie, cytokines, tumor vaccines, and monoclonal antibodies), all of which appear to have significant therapeutic potential in patients with MF. Biologically based therapies may reduce the need for genotoxic therapies, such as cytostatics and radiotherapy.

Combined Modality Therapy↗
PubMed2006

Mycosis fungoides and sezary syndrome: an update.

Mycosis fungoides (MF), and the associated leukemic variant Sezary Syndrome (SS), are the most common group of cutaneous T-cell lymphomas. MF/SS is a non-Hodgkin's lymphoma of mature, skin-homing, clonal, malignant T lymphocytes that initially presents in the skin as patches, plaques, tumors, or generalized erythema (erythroderma) and can involve the lymph nodes and peripheral blood. Much progress has been made in recent years in understanding the origin of the malignant T cell in MF/SS and the pathophysiology and immunology of the disease. This recent work has made a great impact on diagnosis, prognostication, and treatment. In this review, we survey the MF/SS published literature over the past year and highlight some of the important advances.

Combined Modality Therapy↗
PubMed1988

Minimal change glomerulonephropathy and interstitial infiltration with mycosis fungoides.

The nephrotic syndrome developed in a patient with mycosis fungoides shortly after systemic involvement by his tumor occurred. Renal biopsy examination revealed atypical lymphocytic interstitial infiltration and changes consistent with minimal change glomerulonephropathy. The patient's proteinuria decreased following steroid therapy. This is the first report of an association between minimal change glomerulonephropathy and a proven T-cell malignant lymphoma. The implications are discussed with reference to the literature.

Humans↗
PubMed1986

Involvement of chromosomes 12 and 14 in the cutaneous stage of mycosis fungoides: cytogenetic evidence for a multistep pathogenesis of the disease.

Cytogenetic studies were performed on the cells of bone marrow, peripheral blood, and skin tumor biopsies from a patient with mycosis fungoides at an early stage. Chromosome abnormalities were detected in 100% of the cells harvested from the cutaneous specimen, whereas the cells of the bone marrow and blood were karyotypically normal. Three related clones, showing increasing cytogenetic complexity, were found. Chromosome #12 was abnormal in all metaphases, and an abnormal 14q chromosome was present in a minority of cells belonging to the most complex emerging subclone. These data, along with the findings of important signs of chromosome imbalance, suggest a polyphasic evolution of this chronic T lymphoproliferative disease.

Chromosomes, Human, 13-15↗
PubMed1991

Mycosis fungoides-like lesions associated with phenytoin and carbamazepine therapy.

We report the cases of four patients who were taking the anticonvulsant drugs phenytoin or carbamazepine and in whom skin lesions developed that showed histologic features suggestive of mycosis fungoides. Two patients had a solitary lesion on the trunk, whereas the other two patients had multiple plaques. In all four patients systemic signs were absent.

Adult↗
PubMed1991

Topical mechlorethamine in the treatment of mycosis fungoides. Uniformity of application and potential for environmental contamination.

Topical mechlorethamine hydrochloride is commonly used in the treatment of mycosis fungoides and has been formulated in both aqueous and ointment vehicles. Two concerns regarding the topical application of mechlorethamine hydrochloride relate to the adequacy of skin coverage that can be attained by the patient and the extent to which others in the patient's household might be exposed to the drug. In this study six patients applied either aqueous or ointment vehicles containing a fluorescent dye. Subsequent examination of the skin under a Wood's lamp revealed a significant percentage of body surface area to be missed during application; several areas were noted to be missed most commonly. These observations have led to specific alterations in instructions given to patients regarding drug application. Examination of the surrounding environment showed minimal evidence of contamination.

Administration, Cutaneous↗
PubMed1992

Mycosis fungoides--type cutaneous T-cell lymphoma arising before 30 years of age. Immunophenotypic, immunogenotypic and clinicopathologic analysis of nine cases.

BACKGROUND: Cutaneous T-cell lymphomas (CTCLs) rarely arise before 30 years of age; therefore the characteristics of these lymphomas are largely unknown. OBJECTIVE: Our purpose was to assess the clinical and pathologic aspects of CTCL in young persons. METHODS: We identified nine patients who had epidermotropic CTCL by 30 years of age and analyzed their lymphoma phenotypes and genotypes. RESULTS: The diagnosis of CTCL was made an average of 6 years after the reported onset of the lesion. Histologic examination revealed the mycosis fungoides (MF) form of CTCL, and none of the patients underwent conversion to nonepidermotropic or large-cell variants of CTCL. The immunophenotypes were typical of MF-type CTCL; seven of eight lymphomas tested were predominantly CD4+ although in only three were abnormal CD4/CD8 ratios present. All four cases tested were CD7- (Leu-9-), and seven of eight specimens tested exhibited deficient Leu-8 expression. The loss of one or more pan-T-cell markers was found in four of eight patients tested. Clonal beta-chain T-cell receptor gene rearrangements occurred in skin samples from four of eight tested cases. CONCLUSION: A persistent eruption, even in youths and young adults, should be thoroughly evaluated for possible CTCL.

Age Factors↗
PubMed1993

Home UV phototherapy of early mycosis fungoides: long-term follow-up observations in thirty-one patients.

BACKGROUND: In 1982 we reported our preliminary observations on the use of home UV phototherapy for patch and early plaque phase mycosis fungoides (MF). OBJECTIVE: Our purpose was to present follow-up data of the original 31 patients, covering an interval of up to 15 years. METHODS: All patients used a commercially available UV phototherapy unit that contained four Westinghouse FS40 fluorescent lamps for daily exposures of their non-sun-exposed skin regions. RESULTS: A complete clinical and histologic response to home phototherapy occurred in 23 patients (74%) with a maximum duration of the response from 5 months to more than 15 years (median 51 months). After maintenance phototherapy was discontinued, seven patients (23%) had a sustained disease-free interval lasting more than 58 months (median > 90 months). This indicates that cure may have been achieved in a minority of patients. Phototherapy was well tolerated without evidence of significant photodamage or photocarcinogenicity. CONCLUSION: These observations indicate that home phototherapy may be a therapeutic option for treatment of selected patients with early MF.

Adult↗
PubMed1995

Treatment of mycosis fungoides with photochemotherapy (PUVA): long-term follow-up.

BACKGROUND: Mycosis fungoides (MF) is a non-Hodgkin's T-cell lymphoma of the skin that often begins as limited patches and plaques with slow progression to systemic involvement. No studies have been published comparing photochemotherapy (PUVA) with other topical therapies in the treatment of early-stage disease. OBJECTIVE: The purpose of the study was to examine our long-term experience using PUVA to treat early-stage MF and to compare its effectiveness and side-effect profile with other previously reported topical therapies. METHODS: Eighty-two patients with MF (83% stage IA or IB) were treated with PUVA. Clinical and histologic features were observed for a period from 2 months to 15 years (median, 43 months). RESULTS: A response was noted in 78 patients (95%) with complete clinical and histologic clearing in 53 patients (65%) for all stages. The mean duration of total complete response to PUVA for all stages was 43 months (3.6 years). The mean survival of our study group for all stages was 8.5 years. Signs of chronic actinic skin damage were found in 10% of patients, including three patients with basal cell carcinomas and three patients with squamous cell carcinomas. In a nonrandomized comparison with previously reported data for other topical therapies, the efficacy and side-effect profile of PUVA compared favorably. CONCLUSION: PUVA is an effective and safe therapy for MF with prolonged disease-free remissions being achieved. Patients with stage I and II MF respond best to PUVA. Palliative therapy with PUVA is useful in more advanced cases of MF.

Biopsy↗
PubMed1995

Hypopigmented variant of mycosis fungoides: demography, histopathology, and treatment of seven cases.

BACKGROUND: Hypopigmented macules have been described infrequently as a presenting form of mycosis fungoides (MF). OBJECTIVE: This study was designed to clarify general characteristics of a hypopigmented MF variant. METHODS: Seven new cases were investigated with the use of descriptive epidemiology techniques. Demographic parameters, histopathology, and treatment outcomes were analyzed. These data were combined with those from prior reports to develop a broad composite view of this disease process. RESULTS: The median ages in our series were 36 years for disease onset and 39 years at biopsy diagnosis. All patients had brown or black skin. Histologic findings consistently showed a lack of epidermal atrophy and moderate to profound exocytosis. Treatment with PUVA induced rapid and complete repigmentation in six of seven patients. CONCLUSION: On the basis of our experience and a literature review, the hypopigmented variant of MF occurs in a younger population than typical forms of the disease and affects persons with dark skin almost exclusively. Microscopic features include lack of epidermal atrophy and moderate to extreme epidermotropism of infiltrating mononuclear cells. The treatment of choice appears to be PUVA.

Adult↗
PubMed1986

OKT4/OKT8 ratio and serum beta 2-microglobulin in mycosis fungoides and chronic benign dermatitis.

Pre-treatment Serum Beta 2-microglobulin (S B2-m) and OKT4/OKT8 Ratio (T4/T8 R) were studied in 15 patients with Mycosis Fungoides (MF) and in 10 subjects with Chronic Superficial Benign Dermatitis (CSBD) in order to verify whether these parameters may lend support to an earlier differential diagnosis. S B2-m levels and T4/T8 R showed no significant difference in CSBD as compared to normal controls. MF patients displayed elevated S B2-m and T4/T8 R values in comparison to healthy controls and subjects suffering from CSBD (P less than 0.001). After photochemotherapy (PUVA), markedly decreased S B2-m and T4/T8 R values were observed in all patients but two who proved to be unresponsive to PUVA treatment. On the basis of reported data, S B2-m and T4/T8 R can be regarded as an additional tool to discriminate CSBD and MF when clinical and histological features are not significantly diagnostic. Finally, these parameters seem to provide reliable information in monitoring response to treatment.

Adult↗
PubMed1992

Linoleate produces remission in canine mycosis fungoides.

Linoleic acid (LA) administered orally as safflower oil (SFO), which is 76% LA, produced remission in 6 out of 8 dogs with mycosis fungoides (MF). Following each feeding of SFO on 5 successive days to a normal dog peak plasma levels of non-esterified (free) LA in excess of 200 microM were observed. No clinical toxicity was observed from the SFO feedings in the normal or MF animals at the levels of SFO (3 ml/kg) used in these studies. However, a marked rise in white blood cells (WBC) and lymphocytes and a marked transient drop in the serum glutamine transaminases SGOT and SGPT was noted both in the normal and MF animals. These effects of LA may be significant for the remission of MF.

Alanine Transaminase↗
PubMed1983

Total skin electron irradiation for mycosis fungoides: failure analysis and prognostic factors.

From 1970 to 1980, 106 patients with mycosis fungoides received total skin electron irradiation to full tolerance. The majority received 30 Gy of 3 MeV electrons in 12 treatments over three weeks. Eighty-eight patients had received prior therapy. Fifty patients had cutaneous plaques only (T1-2N0), and 56 had more advanced disease. At five years, actuarial survival is 66.7% and disease-free survival 21.4%. The median time to relapse is 12 months; prolonged survival is seen only with complete response. Compared with more advanced stages, T1-2N0 patients have more frequent complete response (96% vs 71%) and better relapse-free survival at five years (32 vs 7%). Of 14 patients with T2 disease in continuous complete remission for from 45-113 months, only one has relapsed. This suggests that cure is possible in up to 26% of patients with T2 disease who achieve complete response. In advanced stages, complete response is more likely with doses over 25 Gy (80 vs 50%). First recurrences were predominently in sites of previous involvement. Death resulted mainly from extracutaneous dissemination or failure to induce remission.

Aged↗