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PubMed2000

Rapidly progressing mycosis fungoides presenting as follicular mucinosis.

Follicular mucinosis can occur as a primary idiopathic disorder or can arise in association with benign or malignant disease, most notably mycosis fungoides. We describe a patient with an aggressive folliculotropic variant of mycosis fungoides that initially presented as follicular mucinosis with alopecia. One month after the diagnosis of follicular mucinosis, a diagnosis of mycosis fungoides was made, and 3 months later inguinal lymph node involvement with mycosis fungoides developed. A skin biopsy specimen demonstrated prominent follicular mucinosis with folliculotropism of atypical cells and intrafollicular Pautrier's microabscesses. As demonstrated in this case, follicular mucinosis can be a presenting sign of rapidly progressive mycosis fungoides. In our review of follicular mucinosis and its association with mycosis fungoides, we found that the folliculotropic variant of mycosis fungoides appears more commonly to have an aggressive course than classic mycosis fungoides.

Adult↗

PubMed1977

Immunocytological characterization of the mycosis fungoides tumour cell.

The immunocytological identification of the mycosis fungoides cell was carried out on cells extracted from the tumorous nodules of a patient suffering from typical mycosis fungoides. Various techniques, such as E and IgM-EAC rosettes and examination for surface membrane immunoglobulins, were performed on the peripheral blood cells and the tumour cell. Membrane staining with a specific anti-T lymphocyte serum conjugated with peroxidase confirmed the thymodependent origin of the mycosis fungoides cell. However, the immunolabelling (with Fab peroxidase conjugate was found constantly negative.

Humans↗

PubMed2001

Follicular mycosis fungoides. A histopathologic analysis of nine cases.

BACKGROUND: The spectrum of mycosis fungoides is exceedingly broad. Many different variants have been described, based on both clinical appearance and histological pattern. A rare form which shows preferential infiltration of hair follicles by malignant lymphocytes is follicular mycosis fungoides. METHODS: We reviewed our experience with nine cases of follicular mycosis fungoides. RESULTS: The unifying feature was infiltration of the hair follicle epithelium by atypical lymphocytes causing varying degrees of damage to the hair follicles. In some specimens the lymphocytes displayed only minor atypia leading to a misinterpretation as pseudolymphoma. Gene rearrangement studies were particularly helpful for establishing a diagnosis of malignant lymphoma. Additionally, epidermotropism of lymphocytes, eosinophils and mucin deposition were present to varying degrees. Mucin makes the distinction from mycosis fungoides-associated follicular mucinosis difficult. We found both dermal mucin and a follicular mucinosis pattern present at different stages of disease in the same patient. CONCLUSIONS: We suggest the term mycosis fungoides-associated follicular mucinosis should be replaced by follicular mycosis fungoides in future lymphoma classification schemes.

Aged↗

PubMed1975

Meningeal mycosis fungoides: clinical and cellular characteristics.

A patient with mycosis fungoides developed meningeal disease while his skin disease was in remission with systemic chemotherapy. His central nervous system involvement with mycosis fungoides was controlled with intrathecal methotrexate for 7 months. The proliferating cells recovered from the spinal fluid showed similarities to the Sézary cell by light and electron microscopy. Surface receptor sutudies suggested that these cells were lymphoid cells of thymic derivation. Although mycosis fungoides has been shown to spread to the central nervous system in autopsied cases, reports of clinical neurologic disease are rare, and in only one earlier report have malignant cells have been found in the spinal fluid. Thus, as in other lymphoproliferative disorders, prompt consideration of meningeal involvement in a patient exhibiting neurologic symptoms while in peripheral remission may allow earlier treatment of this complication.

Animals↗

PubMed1984

[Mycosis fungoides with central nervous system involvement].

Mycosis fungoides at present is classified as a malignant cutaneous lymphoma of T-cell-type. Involvement of every organ is generally possible. Referring to autopsies involvement of the central nervous system is found by chance in about 10%. Since the first description of Mycosis fungoides there are only 20 reports published dealing with a clinical picture due to involvement of the central nervous system. Based on a survey of literature the scale of individual reports concerning involvement of the nervous system is analysed and a report is given on a patient with such a malady confirmed by autopsy.

Adult↗

PubMed1976

Absence of leukocyte-migration inhibitory factor in serum from patients with mycosis fungoides.

Sera from 16 patients with mycosis fungoides at various stages of the disease were investigated for the presence of a leukocyte-migration inhibitory factor. The agarose gel techinque with human peripheral blood leukocytes as migrating indicator cells was used for the purpose. The migration inhibitory effect of patients sera, pooled AB-sera and sera from control persons was tested on peripheral blood leukocytes from patients and controls. Also upconcentrated sera were used. The presence of substances with migration inhibitory effect in serum from patients with mycosis fungoides could not be demonstrated. The significance of these findings is discussed.

Cell Migration Inhibition↗

PubMed1983

Results of lymphography in early mycosis fungoides.

Lymphography was performed in 28 patients with mycosis fungoides. In 22 of the patients, the investigation took place prior to 2 months after the diagnosis was established, and in 7 of these lymphography was made before the histological verification of mycosis fungoides was possible. Five patients with widespread, persistent and severe atopic dermatitis served as controls. Eighteen patients with mycosis fungoides (64%) had abnormal lymphograms, while all 5 controls had normal lymphograms. Abnormal findings were diagnosed in 12 of 22 patients at the earliest time possible during the course of their disease and even found in 5 of 7 patients who only had premycotic lesions at the time of investigation. These results may have some bearing on therapy, suggesting that systemic treatment could possibly be introduced at a far earlier disease stage than is the custom at present.

Dermatitis, Atopic↗

PubMed1976

Further evidence for the T-cell nature of the atypical mononuclear cells in mycosis fungoides.

It is well known that in some cases of mycosis fungoides the lymph nodes contain atypical mononuclear cells with a characteristic electron-microscopic morphology, first described in skin lesions of mycosis fungoides. Because it has been shown, that these cells have T-cell membrane characteristics the question can be raised, if these cells have other properties of T cells. One of these is a preferential localization in the T-cell dependent regions (paracortical areas) of the lymph node. In this paper we present a study of dermatopathic lymph nodes from four patients with mycosis fungoides (plaque stage). The lymph nodes of these patients contained atypical mononuclear cells in the paracortical areas only, and not in the follicles or medulla. In one of the patients we could demonstrate the migration of these cells through the epitheloid venules into the paracortical area. Our observations give further evidence of the T-cell nature of the atypical mononuclear cells in mycosis fungoides.

Adult↗

PubMed1983

Langerhans cells and their precursors with S100 protein in mycosis fungoides.

Twenty-four cases of mycosis fungoides and two cases of Sézary's syndrome were studied with special reference to the infiltration and proliferation of Langerhans cells stained with anti-S100 protein antibody. The number of S100+ histiocytes (Langerhans cells and their precursors) ranged from 104 to 345 (average 188) per 1,000 lymphoid cells in the erythematous and plaque stages, while it decreased to 4-71 (average 34) per 1,000 lymphoid cells in the tumor stage. Conversely, the number of lymphoid cells averaged 6 and 10 per S100+ histiocyte in the erythema and plaque stages, respectively, while it increased to 21.0 per S100+ histiocyte in the tumor stage. The mitotic index of lymphoid cells correlated with the lymphocyte/S100+ histiocyte ratio. A small number of S100+ histiocytes in mycosis fungoides indicated poor prognosis.

Adult↗

PubMed1976

Tumor cell characterization in mycosis fungoides.

Five patients with tumor stage mycosis fungoides had tumor lesions excised and examined by light and electron microscopy. The tumor cells were also isolated and the percentages of E rosettes (T cells) and EAC rosettes (B cells) were determined and the rosettes examined by electron microscopy. The predominant cell type isolated from four of the five tumors was a T cell. Peripheral lymphocyte function measured by LTT to phytohemagglutinin and pokeweed mitogen was normal. Serum immunoglobulins gave variable results. The results support the concept that mycosis fungoides is primarily a T cell tumor involving the skin and morphologically similar to the Sézary cell.

Aged↗

PubMed1988

Topical mechlorethamine therapy for early stage mycosis fungoides.

One hundred seventeen patients with mycosis fungoides were treated with topical mechlorethamine hydrochloride. The probability of achieving a clinically apparent remission within 2 years of therapy was 75.8% in patients with stage I disease, 44.6% in patients with stage II disease, and 48.6% in patients with stage III disease. Patients with stage I disease achieved complete remission sooner (median, 6.5 months) than patients with stage II (median, 41.1 months) or stage III (median, 39.1 months) disease. The median time to relapse was 44.5 months. Sixty-eight patients (58.1%) developed a delayed hypersensitivity reaction, but only one patient had to discontinue therapy as a consequence. No appreciable differences were seen in the probability to achieve complete remission or time to complete remission as stratified by gender, substage, or the development of a delayed hypersensitivity reaction. Survival analysis revealed that the probability of surviving at 5 years was 89% for all patients. These findings compare favorably with results with other treatments for early stage mycosis fungoides.

Administration, Topical↗

PubMed1975

Management of mycosis fungoides--current status and future prospects.

Current knowledge concerning the course of mycosis fungoides and recognized prognostic factors have been reviewed. Those factors with prognostic significance at the time of biopsy diagnosis include age and the clinical findings of skin tumors, ulceration or palpable lymphadenopathy. During the course of disease, the development of skin tumors, ulceration or palpable lymphadenopathy were each associated with a poor prognosis and median survival was only 12 months if all those clinical parameters were present. Patients who developed overt visceral mycosis fungoides rarely survived more than a few months. Lymphocytopenia and the presence of malignant lymphoma in biopsied lymph nodes were also poor prognostic findings. The various modalities of therapy for proven mycosis fungoides were reviewed. Topical therapy and external irradiation were generally of symptomatic benefit only, but two recent studies have shown that aggressive use of topical nitrogen mustard and electron beam therapy are associated with long-term responses in patients with disease confined to the skin. Single agent chemotherapy often resulted in transient responses in advanced and refractory mycosis fungoides. Future approaches to the management of mycosis fungoides have been suggested. These include a thorough review of the histological features, a thorough and systematic pretreatment evaluation and randomized studies of the various treatment modalities including combination therapy in appropriately staged patients.

Administration, Topical↗

PubMed1978

Banding studies of chromosomes in a patient with mycosis fungoides.

Chromosomes from a patient with mycosis fungoides were examined in detail with banding techniques. Hyperdiploid cells from a lymph node had common anomalies of certain chromosomes which formed three similar clones. The abnormalities involved chromosomes No. 1, 2, 5, 8, 9, 10, 14, and 18, in addition to an unkwown small metacentric marker (M3). Although there were a number of mitotic cells in peripheral blood cultured both with and without PHA, none of the few cells with abnormal karyotypes was similar to the clonal cells of the lymph node. One of the abnormalities in the lymph node was a 14q rearrangement, which could be the result of a translocation of Nos. 8 and 14 involving a third chromosome, No. 2. An abnormality in the blood resulted from a translocation between the long arms of Nos. 1 and 14. These findings could be useful for studies in which mycosis fungoides is compared with the Sézary syndrome and other lymphoid malignancies.

Bone Marrow Examination↗

PubMed1991

[Mycosis fungoides. Clinical characteristics and treatment of 6 patients].

Mycosis fungoides is a low grade malignant cutaneous T-cell lymphoma. It is a rare disorder where diagnosis may be difficult to establish in early stages. Usually several years may pass between onset of disease and confirmation of the diagnosis, which should be confirmed histopathologically. Prognosis is difficult to predict and depends on whether mycosis fungoides is limited to the skin or has spread to lymph nodes or viscera. Based on the confirmation of six new cases of mycosis fungoides during the last 18 months by the Department of Dermatology, University Hospital of Tromsø, we discuss clinicopathological findings and alternative methods of treatment.

Aged↗

PubMed1985

Sclerodactyly in a patient with mycosis fungoides.

A 44-year-old man had mycosis fungoides and generalized plaque disease involving 80% of his skin surface with diffuse lymphadenopathy and alopecia of the scalp and groin. In addition, distal to the wrist, there were sclerodermatous changes involving the skin of the hands with associated sclerodactyly of all digits with loss of normal palmar creases. There were no subungual telangiectasis or digital ulcers. The changes in the hand that occurred in this case, no doubt arose as a result of the patient's neoplasm. Abnormalities of collagen biosynthesis and degradation probably occur with mycosis fungoides as a result of the extensive infiltration of the epidermis and dermis with malignant cells. To our knowledge, the association of sclerodactyly with mycosis fungoides has not been previously reported.

Adult↗

PubMedDate not supplied

Laryngeal involvement by mycosis fungoides.

Symptomatic involvement of the larynx by mycosis fungoides is rare. We report a case of mycosis fungoides occurring in the larynx of a 74-year-old man. The laryngeal localization was the first visceral manifestation of the disease to be detected clinically.

Aged↗

PubMed2002

Mycosis fungoides of the larynx: case report and review of the literature.

Mycosis fungoides is a rare cutaneous lymphoma. Dissemination to extra-cutaneous sites occurs at advanced stages of disease. Laryngeal manifestations of mycosis fungoides have been reported in only 13 cases in the available literature. We present a further calla of mycosis fungoides of the larynx at the terminal stage of disease with an additional manifestation in the left maxillary sinus and review the cases published to date. To our knowledge this is the first reported case of mycosis fungoides with laryngeal and paranasal sinus manifestation. Concluding from the present case and from literature therapy should be palliative to improve quality of life.

Aged↗

PubMed1993

Mycosis fungoides involving the cervical esophagus.

Review of the otolaryngologic literature reveals no case reports of mycosis fungoides involving the esophagus. Post-mortem studies report 12 cases of esophageal involvement in 131 autopsies of patients with mycosis fungoides. We describe a 54-year-old man with mycosis fungoides involving the larynx, hypopharynx, and esophagus. Treatment consisted of radiation to this area, with resolution of the patient's hoarseness and dysphagia. The charts of 96 patients with mycosis fungoides treated at our institution were retrospectively reviewed. Three additional cases involving the aerodigestive tract, but not the esophagus, were found. Esophageal mycosis fungoides was an incidental finding in two of seven autopsies at our institution.

Esophageal Neoplasms↗