Topisches Interferon-βEin Vorschlag zur additiven Therapie der ulzerierten Mycosis fungoides

The combination of systemic interferon-alpha and systemic photochemotherapy is one of the most effective and most frequently administered treatment regimens for mycosis fungoides. Two patients with mycosis fungoides stage IIb with ulcerated tumors were treated with this regimen. While the plaques responded favorably to the combination therapy, the ulcerated tumors were quite resistant despite treatment for several weeks. When topical interferon-beta in a gel base was added to the regimen, a rapid resolution of the tumors was noticed. This observation suggests topical interferon-beta may be an effective adjuvant strategy to be combined with systemic therapy.     

Mycosis fungoides. Results of helioclimate therapy in high mountains (Davos, 1,560)

The efficacy of topical steroids, chemotherapy, photochemotherapy, grenz-ray therapy and electron beam therapy has already been established in the treatment of mycosis fungoides. In addition, the results of dermatological climatotherapy in the high Alpine region (Davos, 1,560 m) demonstrate that natural sun irradiation in this particular climate is very effective in treating mycosis fungoides. A total of 63% out of 128 treatment cycles of 84 patients suffering from mycosis fungoides went into remission, which lasted a maximum of at least 13 months. The best results are obtained in the early stages of mycosis fungoides and if climatotherapy lasts long enough and is repeated.     

Local treatment of mycosis fungoides with dichlorodiethyl sulfide in combination with phototherapy

9 patients suffering from mycosis fungoides in various stages were topically treated with dichlorodiethyl sulfide solution associated with photochemotherapy. Topical application of dichlorodiethyl sulfide is indicated in the stages 1 to 3. In more advanced stages, the cutaneous lesions showed good response to therapy, but systemic lesions could not be influenced. Only one patient developed sensitization to topical dichlorodiethyl sulfide. The low incidence of allergic complications may be due to the combined therapy of dichlorodiethyl sulfide and photochemotherapy.     

Photochemotherapy and topical nitrogen mustard in the treatment of mycosis fungoides

Eight patients with mycosis fungoides limited to the skin have been treated with either topical nitrogen mustard alone or in combination with photochemotherapy. This regime does not prevent contact sensitization to nitrogen mustard but will clear sanctuary sites of disease which may develop when photochemotherapy is used alone.     

蕈样肉芽肿的发病机制及治疗进展

蕈样肉芽肿的发病机制目前仍不清楚,近年来对蕈样肉芽肿皮肤淋巴细胞归巢机制、淋巴细胞凋亡异常以及基因异常的研究,为揭示蕈样肉芽肿发病以及治疗提供了一些新的观点。除传统的光化学疗法、放疗、化疗、体外光化学疗法外,维A酸类、干扰素、白介素2融合蛋白、重组白介素12、人源化单克隆抗体等免疫调节剂对早期蕈样肉芽肿有较好的疗效。     

Milia in regressing plaques of mycosis fungoides: provoked by topical nitrogen mustard or not?

OBJECTIVE: To report three cases of mycosis fungoides with milia formation in the regressing lesions. PATIENTS AND SETTING: Dermatology clinic of a university hospital (referral center). Three patients with mycosis fungoides with body surface involvement of 10% in one case (stage IIb) and exceeding 30% in two cases (stages IIb and III). All patients were treated with photochemotherapy and topical nitrogen mustard ointment in a concentration of 0.01%. After approximately 3 months multiple milia erupted on regressing plaques. RESULTS: The presence of milia was evident and was confirmed by histopathology. Regression of mycosis fungoides was noted in these plaques both clinically and in comparison with the pretreatment histologic appearance. Two of the patients showed a histological picture of follicular mucinosis. CONCLUSIONS: We do not know the significance of milia in mycosis fungoides (MF). However, we suggest that follicular rupture or a degenerative process might result in milia formation.     

Fatal squamous cell carcinoma following whole body electron beam therapy and photochemotherapy for mycosis fungoides

The development of fatal metastatic squamous cell carcinoma in a patient with poikiloderma atrophicans vasculare, following treatment with whole body electron beam therapy and photochemotherapy is described. The place of electron beam therapy and photochemotherapy in the treatment of mycosis fungoides is discussed, and the conclusion drawn that the two should be used together only with caution.     

Mycosis fungoides bullosa

A case of a 52-year-old man with mycosis fungoides bullosa. plaque-stage IIA, follicular mucinosis and milia is described. The disease started about 15 years ago and evolved with periods of remission induced by photochemotherapy (PUVA) and, later, by topical nitrogen mustard and etretinate. Vesiculobullous lesions, alopecia and milia sequentially appeared in the course of its evolution. Besides characteristic features of mycosis fungoides, histopathology revealed subepidermal vesicles and follicular mucinosis associated with lymphoid cells (CD3⁺). The pathophysiology of these particular aspects of the mycosis fungoides is discussed.     

Squamous cell carcinoma. Occurrence in mycosis fungoides treated with psoralens plus long-wave ultraviolet radiation

In two patients with mycosis fungoides, a squamous cell carcinoma developed during therapy with psoralens plus long-wave ultraviolet radiation (PUVA). In both patients the carcinoma developed on areas not usually exposed to sunlight. Before receiving PUVA therapy one patient had been treated with topically applied thiotepa and x-ray irradiation, while in the other patient PUVA was the only treatment received except for topical corticosteroids. The carcinomas developed after a total UVA dose of 2,042 and 1,296 joules/sq cm, respectively.     

Mycosis fungoides with depigmentation secondary to treatment

A 51-year-old man presented with itchy, erythematous patches and plaques on his trunk, arms, and legs. A skin biopsy specimen showed mycosis fungoides. Initially the patient did not respond to PUVA photochemotherapy but later improved on NB-UVB phototherapy combined with bexarotene and interferon-alpha. The lesions progressed from erythematous patches and plaques to hyperpigmented patches with central depigmentation and localized areas of follicular repigmentation. The development of depigmentation after PUVA photochemotherapy for mycosis fungoides has been described in the literature and does not have associated prognostic implications. It is important to be cognizant of phototoxicity associated with PUVA photochemotherapy or NB-UVB phototherapy in patients with mycosis fungoides, who may be taking photosensitizing medications or have depigmented patches which renders them more sensitive to lower doses of ultraviolet light.     

Cutaneous malignancies and metastatic squamous cell carcinoma following topical therapies for mycosis fungoides

Specific treatments for mycosis fungoides, including electron beam irradiation, topical mechlorethamine, and psoralen plus ultraviolet A (PUVA) may be associated with the development of skin cancers after a variable latency period. Because these treatments are often not curative, topical therapies for mycosis fungoides, administered sequentially or concomitantly, are being used increasingly in order to control recurrent disease. This report documents the development of multiple cutaneous tumors, including squamous cell carcinoma, basal cell carcinoma, actinic keratoses, keratoacanthomas, and one case of lentigo maligna, in seven patients who received topical therapies for mycosis fungoides. In contrast to the usual latency period between ionizing radiation therapy and the development of skin cancer, two of our patients who had received prior PUVA therapy developed multiple skin tumors upon completion of electron beam irradiation. The development of metastatic squamous cell carcinoma in two of the other seven patients with multiple cutaneous neoplasms suggests that this potential hazard must be considered in the evaluation and treatment of patients with mycosis fungoides.     

Bullous Pemphigoid

A 57-year-old woman with mycosis fungoides developed blisters within cutaneous plaques while receiving PUVA therapy and topical nitrogen mustard. Direct and indirect immunofluorescence studies showed the findings of bullous pemphigoid. Her bullous disease was controlled after cessation of these therapies and institution of prednisone and methotrexate. During the 5 months following completion of a course of electron-beam therapy, she has been free of the cutaneous manifestations of both diseases. Previous instances of PUVA-related pemphigoid have occurred in psoriatics. The role of ultraviolet light in the induction of pemphigoid is discussed, particularly with regard to its possible interaction with the altered skin of psoriasis or mycosis fungoides. Some of the rare cases of bullous mycosis fungoides might actually have represented ultraviolet-unmasked bullous pemphigoid.     

Bullous Pemphigoid

Abstract: A 57-year-old woman with mycosis fungoides developed blisters within cutaneous plaques while receiving PUVA therapy and topical nitrogen mustard. Direct and indirect immunofluorescence studies showed the findings of bullous pemphigoid. Her bullous disease was controlled after cessation of these therapies and institution of prednisone and methotrexate. During the 5 months following completion of a course of electron-beam therapy, she has been free of the cutaneous manifestations of both diseases. Previous instances of PUVA-related pemphigoid have occurred in psoriatics. The role of ultraviolet light in the induction of pemphigoid is discussed, particularly with regard to its possible interaction with the altered skin of psoriasis or mycosis fungoides. Some of the rare cases of bullous mycosis fungoides might actually have represented ultraviolet-unmasked bullous pemphigoid.     

Histological changes observed in the skin of patients with mycosis fungoides receiving photochemotherapy

Thirteen patients with histologically proven mycosis fungoides have had sequential biopsies performed in the course of photochemotherapy. In every case, pruritus was relieved after doses of UV-A ranging from 4–8 Jc/m². Complete clinical of clearing of lesions was observed in eleven of the thirteen patients. Despite objective and subjective clearance of lesions, persistence of a cutaneous infiltrate was observed in all biopsies carried out after commencing PUVA therapy. The quantity of infiltrate was, however, less and the epidermal component considerably reduced. Because of these observations we believe that maintenance therapy with PUVA is required in the mycosis fungoides patients and that careful follow-up is essential.     

Treatment of mycosis fungoides using a 308-nm excimer laser: two case studies

Early-stage mycosis fungoides (MF) is most commonly treated with skin-directed therapies such as topical steroids, phototherapy (broadband or narrowband UVB), photochemotherapy (psoralen plus UVA), topical nitrogen mustard, and total skin electron-beam irradiation. Recently, several small studies have demonstrated the efficacy of the 308-nm excimer laser in the treatment of early-stage MF. This xenon-chloride laser, which emits monochromatic excimer light at the 308-nm wavelength, has been approved by the Food and Drug Administration to treat psoriasis since 1997 and to treat vitiligo since 2001. We report two patients in which patch-stage MF was treated with a 308-nm excimer laser. Our findings confirm previous observations that the 308-nm excimer laser is a safe, effective, and well-tolerated therapy for early stage MF.     

Long-term control of mycosis fungoides of the hands with topical bexarotene

BACKGROUND: Limited Stage IA mycosis fungoides (MF) is often treated with topical steroids, which can cause atrophy, or with nitrogen mustard, which imposes several limitations on the patient's lifestyle. Topical bexarotene is a novel synthetic rexinoid with few side-effects that has shown efficacy for treatment of mycosis fungoides skin lesions in recent Phase II-III clinical trials. The Phase I-II trial involving 67 stage IA-IIA MF patients demonstrated complete response (CR) in 21% and partial response (PR) in 42% of the patients. The median time to response was approximately 20 weeks. In the phase III trial of refractory stage IA, IB and IIA MF, the patients demonstrated a 44% response rate (8% CR). Patients with no prior therapy for mycosis fungoides responded at a higher rate (75%) than those with prior topical therapies. METHODS: Case report of a patient with MF limited to the hands treated with topical bexarotene 0.1% gel in a open label phase II clinical trial. RESULTS: Partial response occurred after 2 weeks of topical bexarotene therapy and the lesions were well controlled for 5 years using bexarotene monotherapy, with only occasional mild local irritation. CONCLUSIONS: Topical bexarotene is effective as long-term treatment monotherapy for limited MF lesions. To our knowledge this is the longest use of the drug by any individual.     

The Effects of Non-Interval PUVA Therapy on the Plaque Stage of Mycosis Fungoides

The effectiveness of non-interval topical PUVA treatment was studied in four patients with mycosis fungoides at the plaque stage. Five regions of each patient were exposed to UVA immediately, 30 minutes, 60 minutes, 90 minutes, and 120 minutes, after topical application of 8-methoxypsoralen, respectively. The effects of these treatments were evaluated by clinical appearance and histological findings after the 20th treatment. All five regions were more improved clinically and histologically than the control region, which was not given PUVA therapy. There were no clear differences clinically among these five regions. Biopsy specimens from each region revealed the disappearance of epidermotropism and a marked decrease in atypical mononuclear cell infiltrations in the dermis. From these data, we concluded that there were no clear differences between these five treatments clinically or histologically and that non-interval PUVA therapy is useful for the early stages of mycosis fungoides. To our knowledge, this is the first report of non-interval PUVA therapy for mycosis fungoides.     

THE TREATMENT OF MYCOSIS FUNGOIDES WITH PUVA*

Twenty patients with mycosis fungoides were treated with photochemotherapy using oral psoralens and long wave ultraviolet light (PUVA) over a two-year period. PUVA was effective in producing a diminution of cutaneous deposits of mycosis fungoides with each clinical pattern of presentation. In most patients complete clearing could not be achieved, and in those considered free of disease, sustained total clearing off PUVA could not be maintained. Lack of response to the effect of PUVA if reinstituted for recurrence of disease did not occur. The palliative use of PUVA for the treatment of mycosis fungoides is recommended.     

Oral photochemotherapy in lichen planus (LP) and mycosis fungoides (MF): ultrastructural modifications of the infiltrating cells.

Six patients (5 with mycosis fungoides and 1 with lichen planus) treated with PUVA, were subjected to biopsy of lesional skin before and during oral photochemotherapy. Ultrastructurally, a reduction in the density of cellular infiltrate was observed in the superficial dermis. In the same areas, necrotic cellular changes were observed. PUVA therapy exercises its beneficial effect by direct destruction of these cells.     

Cutaneous T-cell lymphoma and human immunodeficiency virus infection: 2 cases and a review of the literature

Cutaneous non-Hodgkin's lymphomas are rare in patients with HIV-1 infection and almost all of the cases reported are of T-cell lineage with histopathological features of mycosis fungoides or Sezary syndrome. We studied 2 cases of mycosis fungoides in HIV-1-positive patients who were intravenous drug abusers and were in stage II and IV C2 (CDC'86), respectively. The first patient (stage II) had multiple, erythematous and infiltrated large plaques on the abdomen, back, arms and legs, whereas the second patient (stage IV) had smaller erythematous, slightly scaly and infiltrated pruritic plaques on the trunk and limbs. Their CD4 lymphocyte counts were 634 and 250 cells/mm3, respectively. Biopsies showed features consistent with mycosis fungoides, with an epidermotropic pattern. The immunohistochemical study revealed a T-cell lineage of this atypical infiltrate. Both patients partially responded to topical steroid ointment, showing moderate improvement. Further biopsies performed 6 months later confirmed the prior diagnosis of mycosis fungoides. No tumour stage was observed during a 2-year follow-up. We conclude that mycosis fungoides is rare in HIV-positive patients, but must be included in the differential diagnosis of erythematous plaques in these patients. In suspected, but non-diagnostic cases of mycosis fungoides in HIV-positive patients, only a close clinical and histopathological follow-up can confirm the diagnosis.