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PubMed1980

Pulmonary manifestations of mycosis fungoides.

Three patients with mycosis fungoides who developed pulmonary abnormalities are described. These abnormalities are correlated with the findings at open lung biopsy and at autopsy. The roentgenographic changes consisted of bilateral, nodular pulmonary infiltrates in two patients, and interstitial infiltrate and plate-like atelectasis with hilar adenopathy in a third patient. Examination of lung biopsies and autopsy tissue revealed interstitial and/or intra-alveolar infiltrates composed of atypical lymphoid cells similar to those seem in the skin biopsies. The short survival after detection of an abnormal chest film suggests that dissemination of mycosis fungoides to the lung heralds a poor prognosis.

Biopsy↗
PubMed1977

Photochemotherapy in mycosis fungoides.

Six patients with mycosis fungoides were treated with methoxsalen and long-wave ultraviolet light. They were assessed clinically and histologically before and after treatment. All six patients showed clinical improvement. Histological clearing of both epidermis and dermis occurred in three patients, and epidermal clearing alone occurred in two. Photochemotherapy may have a place in the treatment of the early and intermediate stages of mycosis fungoides, and it may also be useful as an adjunct to other forms of treatment in the more advanced stages.

Adult↗
PubMed2003

Management of mycosis fungoides: Part 2. Treatment.

Mycosis fungoides is a low-grade lymphoproliferative disorder of skin-homing CD4+ lymphocytes that may produce patches, plaques, tumors, erythroderma, and, ultimately, systemic dissemination. Treatment selection is generally guided by institutional experience, patient preference, and toxicity profile, as data from phase III clinical trials are limited. Effective topical treatments currently include mechlorethamine (Mustargen), carmustine (BCNU, BiCNU), corticosteroids, bexarotene (Targretin, a novel rexinoid), psoralen plus ultraviolet A, ultraviolet B, and total-skin electron-beam radiotherapy. Effective systemic treatments include interferon, retinoids, bexarotene, denileukin diftitox (Ontak), extracorporeal photopheresis, chemotherapy, and high-dose chemotherapy with allogeneic bone marrow transplant. Each of these treatments is discussed in detail, followed by specific recommendations for each stage of mycosis fungoides.

Administration, Topical↗
PubMed1981

Clinicopathologic correlations in Alibert-type mycosis fungoides.

Five cases of mycosis fungoides of the Alibert type were studied by taking multiple biopsy specimens at different stages of the disease. Large hyperchromatic, slightly irregular mononuclear cells are the most frequent cells. Ultrastructurally, the cells were only slightly convoluted, had prominent heterochromatin banding at the nuclear membrane, and unremarkable cytoplasmic organelles. Highly convoluted cerebriform nucleated cells were few. Large regular vesicular histiocytes were prominent in the early stages. Ultrastructurally, the cells showed evenly distributed euchromatin. Epidermotrophism was equally as important as Pautrier's abscess as a hallmark of the disease. Stereologic techniques comparing the infiltrate with regard to size and convolution of cells in all stages of mycosis fungoides with infiltrates seen in a variety of benign dermatoses showed no statistically significant differences.

Aged↗
PubMed1991

Application of DNA flow cytometry from paraffin-embedded tissue to the diagnosis of mycosis fungoides.

The distinction of mycosis fungoides from benign inflammatory lesions is sometimes difficult by conventional histological techniques. Aneuploidy, a feature often associated with malignant tumors, can be assessed even in tissue routinely processed in paraffin using the flow cytometric technique of Hedley and associates. In many tumor systems, there are significant diploid clones. We have evaluated the flow cytometric DNA ploidy of paraffin-embedded tissue in the diagnosis and prognosis of mycosis fungoides (MF). We studied 22 cases of MF and 10 control cases of inflammatory skin lesions with epidermal involvement. Aneuploidy was found in 27% of the MF cases, but in none of the controls (ED). Aneuploid features were seen in 23% of tissues from early stage disease. Aneuploidy did not correlate with atypia, epidermotropism, or number of mitoses. There was a trend towards showing adverse outcome in those patients with aneuploid lesions. The detection of aneuploidy might be helpful for early diagnosis of MF.

Aneuploidy↗
PubMed1984

Establishment of T and B cell lines from patients with mycosis fungoides.

Eighteen patients with mycosis fungoides were studied in order to establish cell lines that might be associated with the human T cell leukaemia-lymphoma virus (HTLV). Three T cell lines were established, two from affected skin and one from a lymph node showing dermatopathic lymphadenopathy. The T cells expressed OKT3 and OKT4 antigens. They temporarily expressed an HTLV p19-like antigen in up to 5% of the cells during culture. None of our patients had lymphocytosis or abnormal lymphocytes, except one patient with Sézary's syndrome. We could not establish T cell lines from peripheral blood, but five B lymphoblastoid cell lines were obtained, all positive for the Epstein-Barr virus nuclear antigen. Our finding that T cell lines can be established from skin biopsies and lymph nodes of patients with mycosis fungoides, but not from the blood, supports the concept of a malignant T lymphocyte primarily localized in the skin. The temporary expression of HTLV p19 antigen may indicate the presence of retrovirus, but further studies are needed.

Adult↗
PubMed2006

Mycosis fungoides associated with B-cell malignancies.

BACKGROUND: The coexistence of mycosis fungoides, a peripheral T-cell lymphoma, and B-cell malignancies or Hodgkin's lymphoma in the same patient is unusual. Most descriptions are isolated case reports and case series are strikingly sparse. OBJECTIVES: To detect cases of mycosis fungoides associated with B-cell malignancies or Hodgkin's lymphoma and to analyse the characteristics of and the interplay between the lymphoproliferative neoplasms. METHODS: Patients with mycosis fungoides who had B-cell malignancies or Hodgkin's lymphoma were selected from among 398 patients either treated or followed up in two tertiary medical centres during a 7-year period. RESULTS: Eleven patients with mycosis fungoides and B-cell malignancy were detected (seven of non-Hodgkin's lymphoma, three of chronic lymphocytic leukaemia, one of multiple myeloma). No case of Hodgkin's lymphoma was found. In seven patients the mycosis fungoides preceded the B-cell malignancy whereas in four it was the B-cell malignancy which occurred first. The time elapsed between onset of the two malignancies ranged from 4 to 22 years (average: 12 years). Patients who had mycosis fungoides as the first neoplasm presented with earlier stages of mycosis fungoides (four of seven: IA, three of seven: IB) than those who had mycosis fungoides as their second neoplasm (of four, one: IB, one: folliculotropic, two: IIB). Among the four patients in whom the appearance of mycosis fungoides followed the B-cell malignancy, three had been treated with multiagent chemotherapy. Two patients who presented with early-stage mycosis fungoides (IA) as the first lymphoma developed mycosis fungoides tumours after becoming immunosuppressed. In two patients infiltrates composed of both malignant T- and B-cell populations were found in a single biopsy. One showed two distinct populations of the malignant cells in the skin tumour, thus constituting a classical composite lymphoma of mycosis fungoides and chronic lymphocytic leukaemia, while in the other patient the two malignant populations of marginal B-cell lymphoma and mycosis fungoides (as evidenced by both phenotypic and genotypic findings) were intermingled. CONCLUSIONS: This case series indicates that while the coexistence of Hodgkin's lymphoma and mycosis fungoides is extremely rare, the association of mycosis fungoides and B-cell malignancies is not as rare as reflected in the literature, with non-Hodgkin's lymphoma constituting the most common associated B-cell malignancy. In this series as well as in the cases reported in the literature mycosis fungoides usually preceded the development of B-cell malignancies, which may be in accordance with previous reports of an increased risk of developing a second haematological neoplasm. The importance of a competent immune system for patients with mycosis fungoides is well demonstrated in these cases. It is suggested that for greater precision the criteria for diagnosis of composite lymphoma of the skin should include both phenotypic and genotypic features.

Adult↗
PubMed1977

Adriamycin therapy in advanced mycosis fungoides.

Thirteen patients with advanced mycosis fungoides received induction therapy with Adriamycin, 60/m2 I.V. repeated at 21-day intervals. Ten patients had extensive skin tumors; all patients had lymph node enlargement with mycosis fungoides involvement in eight; four patients had biopsy-proven visceral involvement. Only two patients had received no prior therapy. The overall response rate with Adriamycin therapy was 85% with three patients (23%) achieving a biopsy-proven complete remission and five patients (39%) partial remissions. The median number of courses to maximum response was two (range two to four). The principle toxicity was myelosuppression, but this was not severe and the entire group received more than 90% of the intended doses of Adriamycin. One patient developed probable Adriamycin cariotoxicity. Maintenance therapy for patients achieving a remission was methotrexate 15 mg/m2 I.M. twice weekly and cyclophosphamide 750 mg/m2 I.V. every 21 days. The median duration of complete remission was 32+ weeks (range 16+-40+ weeks) while the median duration of partial remission was 18 weeks (range 8-111+ weeks). Adriamycin has proven to be an effective induction agent in the treatment of advanced mycosis fungoides and its incorporation into combination chemotherapy regimens is warranted.

Bone Marrow↗
PubMed1982

Controversies in mycosis fungoides.

Most clinicians agree that mycosis fungoides is the prototypic cutaneous T cell lymphoma. However, certain clinical characteristics indicate that this disorder may begin as a reactive rather than a neoplastic process. The concept of a nonneoplastic etiopathogenesis of mycosis fungoides is further supported by recent data on the function of Langerhans cells, a population of epidermal cells known to play a critical role in immune surveillance and the development of contact sensitivity. It has been suggested that chronic occupational exposure to environmental allergens results in persistent antigenic stimulation, leading to a breakdown in immune surveillance and eventually, malignancy. Modern laboratory technics have enhanced the clinician's ability to diagnose and stage mycosis fungoides. Data obtained from such studies have indicated that systemic spread may occur much earlier in the course of disease than has previously been appreciated. The therapeutic implications of such knowledge are as yet uncertain.

Allergens↗
PubMed1978

Subcutaneous mycosis fungoides.

In five cases of mycosis fungoides, previously treated with electron-beam therapy, subcutaneous nodules developed. Clinically, these lesions were thought to be epidermoid cysts or lipomas, but on biopsy were discovered to be subcutaneous infiltrates, three of which were diagnosed as mycosis fungoides. The other two specimens showed only a nonspecific subcutaneous infiltrate. There is no ready explanation for the appearance of these lesions, but it is speculated that they may be the result of inadequate penetration of the electron beam to the depth at which some atypical cells may originally have been located. Patients with mycosis fungoides who develop unusual subcutaneous nodules should be fully investigated so that appropriate and adequate therapy may be initiated.

Adult↗
PubMed2003

Carbamazepine-induced pseudo mycosis fungoides.

OBJECTIVE: To report a case of pseudo mycosis fungoides due to carbamazepine. CASE SUMMARY: A 54-year-old man experienced a skin lesion resembling mycosis fungoides without any systemic symptoms or signs 2 months after he had begun carbamazepine treatment for his seizures. Skin-punch biopsy specimens revealed mycosis fungoides-like histopathologic appearance. After drug discontinuation, the patient experienced complete remission of the clinical and pathologic findings. This suggests a diagnosis of pseudo mycosis fungoides due to carbamazepine. DISCUSSION: Mycosis fungoides is the cutaneous T-cell lymphoma of the skin that needs aggressive chemotherapy and radiation treatment. Pseudo mycosis fungoides is a condition caused by certain drugs that has a similar clinical and histopathologic appearance to mycosis fungoides. When the causative drug is discontinued, the lesions resolve completely. CONCLUSIONS: An objective causality assessment revealed that carbamazepine was highly probable as the cause of the adverse reaction. Patients who are diagnosed with mycosis fungoides should be asked about any drug use, and clinicians should recognize signs of pseudo mycosis fungoides.

Carbamazepine↗
PubMed1979

Gingival involvement in mycosis fungoides: report of case.

A case of mycosis fungoides of the gingiva is reported. Mycosis fungoides is a lymphoproliferative disease that primarily affects the skin. Systemic dissemination is a distinct aspect of the natural course of mycosis fungoides and usually involves the lymph nodes, spleen, and liver. The diagnosis of mycosis fungoides depends on the presence of a variety of atypical cells, including small and large variants of the mycosis cell with an infiltrate of polymorphous inflammatory cells in both the dermis and epidermis. Various modes of treatment have been used. In the current case, remission was achieved with radiation therapy.

Aged↗
PubMed1976

Leptomeningeal mycosis fungoides.

Central nervous system involvement with mycosis fungoides complicated the clinical course of a patient at a time when his skin was clinically free of disease following systemic chemotherapy. A leptomeningeal syndrome of blurred vision and papilledema, and confusion progressing to coma, was associated with elevated spinal fluid pressure and abnormal spinal fluid cells morphologically similar to those seen in the Sezary syndrome. The symptoms were dramatically reversed by intrathecal methotrexate, brain irradiation, and steroids. Mycosis fungoides recurred in the skin, in the spinal fluid, and in both eyes. Despite continued systemic and intrathecal chemotherapy, the patient died from mycosis fungoides. This is the second patient reported with meningeal mycosis fungoides.

Antineoplastic Agents↗
PubMed1992

Granulomatous mycosis fungoides. Clinicopathologic study of two cases.

Granulomatous mycosis fungoides is a rare form of mycosis fungoides with controversial histogenesis. Early reports seemed to indicate a favorable prognosis for these patients. We report two cases of granulomatous mycosis fungoides, both of which had other unusual clinical features. The cases were studied with routine light microscopy, immunohistochemistry, electron microscopy, and gene probe studies. Despite some clinical and histopathologic similarities, the results of the immunohistochemical and molecular biologic studies were diverse. These results suggest that granulomatous mycosis fungoides does not define a single subset of cases, immunophenotypically or biologically.

Adult↗
PubMed2002

Mycosis fungoides causing severe lower eyelid ulceration.

Mycosis fungoides is a form of cutaneous malignant T-cell lymphoma initially confined to the skin. A range of ophthalmic manifestations has been described; these usually appear in advanced disease. Lid involvement is most common and ranges from ectropion, usually cicatricial, to diffuse thickening and placoid tumours. A case of a 72-year-old man presenting with progressive, full thickness, lower eyelid ulceration is presented. The patient had a long history of placoid skin lesions, previously diagnosed as discoid lupus. Repeat biopsies of these lesions including the eyelid eventually revealed mycosis fungoides. The ulceration progressed to destroy most of the lower lid before the correct diagnosis was made. This type of eyelid involvement has not previously been reported in mycosis fungoides. This case also highlights the diagnostic difficulties encountered in the early phases of mycosis fungoides. If suspected, multiple biopsies are essential to establish the diagnosis.

Aged↗
PubMed1996

Mycosis fungoides with marked hyperpigmentation.

Pigmentary changes in mycosis fungoides are not rare. Although poikiloderma and hypopigmented skin lesions have often been reported in the literature, there are few cases of mycosis fungoides presenting as a hyperpigmented skin lesion. We present a 57-year-old Japanese male with mycosis fungoides whose skin lesions showed marked hyperpigmentation. The skin lesion initially appeared as an irregularly shaped itchy annular erythema with central pigmentation predominantly on his extremities. During our 5-year follow-up, these skin lesions gradually increased in size and number. The erythema extended peripherally and became elevated with marked hyperpigmentation. Histology revealed extreme elongation of the rete ridges with infiltration of atypical large lymphoid cells characteristic of mycosis fungoides and numerous melanin granules in both the epidermal melanocytes and dermal melanophages. Although the exact mechanism of the marked hyperpigmentation is one of the unique characteristics in mycosis fungoides, especially in non-white individuals.

Humans↗
PubMed1999

Mycosis fungoides with mucinosis follicularis in childhood.

Mycosis fungoides is a cutaneous T-cell lymphoma (CTCL) usually observed in mid to late adulthood. It occurs only rarely during childhood. Follicular mucinosis is a chronic dermatosis involving the sebaceous glands and outer root sheaths. It is normally differentiated into a juvenile benign form and an adult form possibly associated with mycosis fungoides. We report a 12-year-old boy who presented with an 8-month history of erythematous mucinous plaques on the scalp. Three months later, he developed erythematous patches and plaques on his whole body, accompanied by cervical lymphadenopathy. A biopsy showed follicular mucinosis and epidermotropism of the lymphocytic infiltrate. Immunophenotyping and a PCR clonality test were consistent with CTCL. The patient received PUVA treatment and local steroids, resulting in partial remission. Mycosis fungoides should be considered in the differential diagnosis of chronic, scaling dermatoses in childhood. Moreover, follicular mucinosis in childhood can be associated with mycosis fungoides.

Child↗