Papular mycosis fungoides: a variant of mycosis fungoides or lymphomatoid papulosis?
Explore the source record for details and available documents.
Diagnosis, Differential↗
SEARCH · Search PubMed
Results for “MYCOSIS FUNGOIDES”
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Aspects of the treatment of mycosis fungoides. A report from the Scandinavian Mycosis Fungoides Study Group.
The Scandinavian Mycosis Fungoides Study Group includes dermatologic clinics in Denmark, Norway, and Sweden. The results of the first three years of activity are presented herein. In plaque stage, topical nitrogen mustard was highly effective. Preceding intravenous tolerance induction seems to be of no value. PUVA induced equally high remission rates. Both modalities were also highly effective in cutaneous tumor stage. In advanced tumor stage and in case of extracutaneous involvement systemic chemotherapy was given. Topical treatment alone was more effective on the cutaneous lesions including tumors than systemic chemotherapy alone. Therefore a combination of topical and systemic treatment is recommended in advanced stages of mycosis fungoides.
Bleomycin↗
Papular mycosis fungoides: a new clinical variant of early mycosis fungoides.
BACKGROUND: Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. In early stages of the disease many different clinicopathologic variants have been observed. OBSERVATION: We report 6 patients with early manifestations of MF characterized by the sole presence of papules which, unlike the papules of lymphomatoid papulosis, did not show a tendency for spontaneous resolution. Histologic examination confirmed the diagnosis of MF in all cases. Immunohistochemical staining for CD30 was negative in all cases. Follow-up data showed the nonaggressive behavior of the disease, confirming that the lesions were not manifestations of advanced MF. CONCLUSION: Papular MF is a new variant of early MF characterized by the presence of papules in the absence of more conventional early lesions (patches) of the disease. This variant should be added to the long list of clinicopathologic subtypes of MF.
Adult↗
Retinoids and systemic chemotherapy in cases of advanced mycosis fungoides. A report from the Scandinavian Mycosis Fungoides Group.
In cases of advance mycosis fungoides, the systemic chemotherapy combination of bleomycin, cyclophosphamide and prednisolone was given to 8 cases, and the same 3-drug combination with the addition of oral retinoids given to 12 cases. All cases were in a progressive phase of the disease. Remission was obtained in 5/8 cases treated with the combination and in 7/12 cases treated with the combination plus retinoids. The remissions were complete in half of the cases, but relapse occurred within 3 to 6 months in all but 2 cases. The two treatment patient groups were not fully comparable but the conclusion is that the addition of retinoids to systemic chemotherapy combination regimens is of some advantage. There still exists, however, need of more adequate treatment modalities in advanced mycosis fungoides.
Bleomycin↗
Follicular mycosis fungoides.
Follicular mycosis fungoides is a rare variant of cutaneous T-cell lymphoma. We report a patient with thin plaques of mycosis fungoides studied with follicular papules. Histologically, these lesions were composed of dense infiltrates of lymphocytes within and around follicular epithelium ("folliculotropism"). This case indicates that follicular mycosis fungoides is a distinct clinicopathologic entity. It is different from follicular mucinosis associated with mycosis fungoides. Conceptually, the relationship between follicular mycosis fungoides and ordinary mycosis fungoides is analogues to the relationship of lichen planopilaris to ordinary lichen planus.
Adult↗
Prednimustine in mycosis fungoides: a report from the Scandinavian mycosis fungoides study group.
Prednimustine, a chlorambucil ester of prednisolone, was administered orally to 5 patients with mycosis fungoides in advanced tumour stage. Partial remission was obtained in 2 of the patients. However, we do not consider the agent to be particularly advantageous for the treatment of mycosis fungoides.
Administration, Oral↗
Photochemotherapy (PUVA) in the tumour stage of mycosis fungoides: a report from the Scandinavian Mycosis Fungoides Study Group.
Twenty-five patients with mycosis fungoides in the tumour stage were treated with oral 8-Methoxypsoralen followed by UVA (PUVA), sometimes in combination with topical or systemic chemotherapy. In 17 patients the disease was confined to the skin, in 7 lymph nodes also were involved, and one had visceral involvement. Complete and partial remission was achieved in 14/17 patients with the disease limited to the skin (82%), and partial remission of the skin lesions in 5/8 patients with extracutaneous location.
Adult↗
Photochemotherapy (PUVA) in the pretumour stage of mycosis fungoides: a report from the Scandinavian Mycosis Fungoides Study Group.
Fifty-one patients with mycosis fungoides of pretumour stage were treated with oral 8-MOP and UVA photochemotherapy (PUVA). Complete remission was induced within 2--3 months in 58% of the cases. Twenty-seven patients are still in remission on maintenance therapy 9--53 months after starting treatment. In 9 cases PUVA treatment was stopped due to therapeutic failure and in another 15 cases due to various side effects. Maintenance therapy was given weekly, monthly, or at even longer intervals. Maintenance at long intervals seems preferable.
Adolescent↗
Retinoid dermatitis mimicking progression in mycosis fungoides: a report from the Scandinavian Mycosis Fungoides Group.
A dermatitis occurring during the treatment of mycosis fungoides with A vitamin analogues (13-cis-retinoic acid and etretinate) and mimicking a progression of the disease is described. It is considered to be a skin reaction due to the treatment. Its benign nature is revealed by histology showing a lymphocytic infiltrate without any atypical sign.
Aged↗
Thymus and activation-regulated chemokine (TARC/CCL17) in mycosis fungoides: serum TARC levels reflect the disease activity of mycosis fungoides.
BACKGROUND: Mycosis fungoides (MF) belongs to cutaneous T-cell lymphoma and is clinically divided into 3 stages: patch, plaque, and tumor stage. Thymus and activation-regulated chemokine (TARC/CCL17) is a member of the CC chemokines and is a chemoattractant for CC chemokine receptor 4 (CCR4)- and CC chemokine receptor 8 (CCR8)-expressing cells. OBJECTIVE: In this study, we examined the involvement of TARC among patients with each stage of MF. METHODS: We investigated the expression of TARC, CCR4, and CXC chemokine receptor 3 in patients with each stage of MF by immunohistochemistry. We measured serum TARC levels in 20 patients with MF in varying degrees and compared them with 10 patients with psoriasis vulgaris and 10 healthy controls. In addition, we compared serum TARC levels in patients with MF with other laboratory data. RESULTS: Immunohistochemical staining revealed that TARC was expressed in the lesional keratinocytes in the patch, plaque, and tumor stages. CCR4 was expressed on the epidermotropic cells in both patch and plaque stages and on the large cell-transformed cells in the tumor stage, whereas CXC chemokine receptor 3 was constantly expressed on the small cells in the lesional dermis. Serum TARC levels in patients with MF were significantly higher than those in patients with psoriasis vulgaris or healthy controls. Moreover, serum TARC levels in patients with the tumor stage of MF (n = 5) were remarkably higher than those with patch stage (n = 8) or plaque stage (n = 7). Serum TARC levels significantly correlated with serum lactate dehydrogenase levels (r = 0.62), serum immunoglobulin E levels (r = 0.60), serum soluble interleukin 2 receptor levels (r = 0.72), and serum macrophage-derived chemokine levels (r = 0.70). CONCLUSION: These data strongly indicate that serum TARC levels are useful for assessing the disease activity of patients with MF and that TARC and CCR4 may be involved in the pathogenesis of MF.
Adult↗
Epipodophyllotoxin (VP-16-213) in mycosis fungoides: A report from the Scandinavian mycosis fungoides study group.
Epipodophyllotoxin (VP-16-213) was administered to 9 patients with mycosis fungoides in various stages, most of them in the advanced tumour stage. In 4 of the patients VP-16 was combined with cyclophosphamide. VP-16 alone or in combination with cyclophosphamide was capable of inducing remission initially in all cases, complete in 2, partial in 3 and improvement to a lesser degree in the remaining 5 patients, but it was unable to maintain the remission. The induced remission has to be upheld by other agents, possibly added to VP-16.
Adult↗
[Macrophage activation syndrome disclosing leukemic transformation of mycosis fungoides].
BACKGROUND: Mycosis fungoides can mimic pigmented purpuric dermatitis. We report such a case which progressed to peripheral T-cell lymphoma; progression was revealed by reactive hemophagocytic syndrome (RHS). CASE REPORT: A 65-year old male patient was hospitalized for a pigmented and purpuric eruption. The skin lesions appeared 2 years earlier and at that time biopsy had shown pigmented and purpuric dermatitis. One month before hospitalization, general signs appeared. On admission, he had papular and purpuric rash, mainly on the trunk, hepatosplenomegaly, enlarged axillar and inguinal lymph nodes, and fever at 38.2 degrees. A skin biopsy showed histologic changes typical of mycosis fungoides. He also had bicytopenia, hepatitis, and increased triglyceride and ferritin levels suggesting RHS which was proved by means of bone marrow biopsy. These tests also evidenced peripheral T-cell lymphoma. The patient was treated with two courses of chemotherapy (CHOP) but the disease progressed and he deceased. DISCUSSION: Mycosis fungoides can occasionally begin with an eruption very closely resembling pigmented purpuric dermatitis. Therefore, repeated biopsies should be done in case of widespread permanent pigmented purpuric dermatitis of no apparent origin. RHS is a life-threatening disease. The diagnosis should be suspected in any cytopenic patient with fever, increased triglyceride levels and abnormal liver tests. A search for an etiology must then be undertaken a prompt treatment is needed.
Aged↗
Tumour stage of mycosis fungoides treated with bleomycin and methotrexate: report from the Scandinavian mycosis fungoides study group.
The Scandinavian Mycosis Fungoides Study Group have treated 19 patients with mycosis fungoides in tumour stage by systemic chemotherapy. Nine patients were treated with Bleomycin 15 mg i.m. twice weekly for 7 weeks and 10 patients with the same dose of Bleomycin in combination with Methotrexate 15 mg i.m. per m2 body surface each week for 7 weeks. No maintenance treatment was given. The immediate therapeutic effect of Bleomycin alone was considered good in half of the patients. Bleomycin and Methotrexate together produced a better initial effect. In both treatment series the remission was short-lived in the absence of maintenance therapy. The mortality rate was high, especially in combination treatment. Lethal complications occurred in 6 patients during the treatment, 3 of which were thromboembolic, one pancytopenia, one lung fibrosis with pulmonary insufficiency, and one bronchopneumonia. The conclusion is that these two forms of therapy cannot be recommended.
Adult↗
Constitutive activation of a slowly migrating isoform of Stat3 in mycosis fungoides: tyrphostin AG490 inhibits Stat3 activation and growth of mycosis fungoides tumor cell lines.
Mycosis fungoides (MF) is a low-grade cutaneous T cell lymphoma of unknown etiology. In this report, the Jak/Stat (Janus kinase/signal transducer and activator of transcription) signaling pathway was investigated in tumor cell lines established from skin biopsy specimens from a patient with MF. Jaks link cytokine receptors to Stats, and abnormal Jak/Stat signaling has been observed in some hemopoietic cancers. In MF tumor cells, a slowly migrating isoform of Stat3, Stat3(sm), was found to be constitutively activated, i.e., (i) Stat3(sm) was constitutively phosphorylated on tyrosine residues, and tyrosine phosphorylation was not enhanced by growth factor stimulation; (ii) band shift assays and immunoprecipitations of DNA/Stat complexes showed constitutive DNA-binding properties of Stat3(sm); and (iii) Stat3(sm) was constitutively associated with Jak3. The abnormal activation of Stat3(sm) was highly specific. Thus, neither the fast migrating isoform of Stat3 (Stat3(fm)) nor other Stats (Stat1, Stat2, and Stat4 through Stat6) were constitutively activated. The Jak kinase inhibitor, tyrphostin AG490, blocked the constitutive activation of Stat3(sm) and inhibited spontaneous as well as interleukin 2-induced growth of MF tumor cells. In conclusion, we have provided evidence for an abnormal Jak/Stat signaling and growth regulation in tumor cells obtained from affected skin of an MF patient.
Cell Division↗
Retinoids plus PUVA (RePUVA) and PUVA in mycosis fungoides, plaque stage. A report from the Scandinavian Mycosis Fungoides Group.
Sixty-nine patients with mycosis fungoides, plaque stage, were treated in an open study with photochemotherapy (PUVA) or the combination of oral retinoids and PUVA (RePUVA). The response rate of Re-PUVA was equal to that of PUVA, with complete remission in 73% and 72%, respectively. Remissions were obtained with fewer PUVA sessions, and with a lower UVA dosage, if PUVA was combined with retinoids. A lower UVA dosage was needed if treatment was given four times weekly in stead of twice weekly. The duration of the remissions tended to be prolonged if retinoids were given as maintenance therapy.
Adult↗
Intramuscular low dose alpha-2B interferon and etretinate for treatment of mycosis fungoides.
BACKGROUND: Mycosis fungoides is a lymphoma of cutaneous origin characterized by a proliferation of cells with a T phenotype. METHODS: In this pilot study, 13 men with mycosis fungoides in various stages were treated with alpha-2b interferon and etretinate. RESULTS: In ten of them, such a therapy proved to be effective (7 complete responses, 3 partial responses), sometimes with prolonged remissions (up to 20 months, and still persistent) after suspension of the drugs. CONCLUSIONS: We chose low-dose interferon administration in order to prevent side effects, which are said to be dose-dependent. In our experience this is not true, but this drawback seems to be overcome by the very good, sometimes spectacular, response to this combination therapy, particularly in low stage forms of the disease. This fact, compared with results provided by other groups, prompts us to plan new research protocols based on associations of retinoids with different interferon types (or even associations of different interferons), because we believe they will have an important place in treatment of cutaneous T-cell lymphoma in future.
Adult↗
The mycosis fungoides cell: the skin window in mycosis fungoides.
Explore the source record for details and available documents.
Hodgkin Disease↗
Dermatopathic lymphadenopathy. An immunophenotypic comparison of cases associated and unassociated with mycosis fungoides.
Documenting focal mycosis fungoides in lymph node biopsies that exhibit dermatopathic lymphadenopathy is morphologically difficult. Since mycosis fungoides is a lymphoma with the phenotype of mature T cells, usually of the T-helper class, the authors investigated whether there are alterations in the ratio of Leu 3a (T-helper):Leu 2a (T-suppressor) cells in dermatopathic lymphadenopathy in order to determine the significance of immunologic markers as a possible solution to the problem. Ten lymph node biopsy specimens with diagnostic evidence of dermatopathic lymphadenopathy, but not of mycosis fungoides, were studied with the use of fresh-frozen section immunohistochemistry (FS), cell suspensions (CS), or both; five of the specimens came from patients with known cutaneous mycosis fungoides, and the other five came from patients without mycosis fungoides. The mean Leu 3a/Leu 2a ratio was 7.0 +/- 1.06 (SE) in all 10 cases of dermatopathic lymphadenopathy studied by FS and 6.9 +/- 1.14 in the 6 cases studied by CS. These ratios were significantly higher (P less than 0.001) than the mean Leu 3a/Leu 2a ratios of 2.9 +/- 0.29 (FS) and 2.4 +/- 0.22 (CS) in control lymph nodes exhibiting nonspecific reactive follicular hyperplasia, but were comparable to the mean Leu 3a/Leu 2a ratio of 5.9 obtained in two lymph node biopsies with unequivocal involvement by mycosis fungoides. Despite the increase in Leu 3a staining cells in dermatopathic lymphadenopathy, however, there were no essential differences in the Leu 3a/Leu 2a ratios between patients with and those without known mycosis fungoides. The use of other antibodies reactive with T cells, such as anti-Leu 8, anti-Leu 9, and anti-Tac also did not aid in this discrimination. The results indicate that determination of the Leu 3a/Leu 2a ratio and use of other conventional T-cell monoclonal antibodies do not provide conclusive evidence in support of a presumptive or early diagnosis of mycosis fungoides in a lymph node which fails to show histologic evidence of the disease.
Animals↗