|Year : 2000 | Volume
| Issue : 5 | Page : 251-253
Mycosis fungoides treated with Puva and Topical Corticosteroids
M Raman, Paschal Souza D, JS Prasad Ravindra, Iyer
Source of Support: None, Conflict of Interest: None
An 89-year old patient had mycosis fungoides with extensive skin involvement and palpable but pathologically uninvolved lymph nodes. He was successfully treated with PUVA combined with topical 0.1% fluocinolone acetonide ointment. PUVA therapy is highly effective in the treatment of mycosis fungoides confined to the skin, especially in the elderly where more aggressive therapy may not be tolerated.
Keywords: Mycosis fungoides, PUVA
|How to cite this article:|
Raman M, PaschalS, Ravindra JP, Iyer. Mycosis fungoides treated with Puva and Topical Corticosteroids. Indian J Dermatol Venereol Leprol 2000;66:251-3
|How to cite this URL:|
Raman M, PaschalS, Ravindra JP, Iyer. Mycosis fungoides treated with Puva and Topical Corticosteroids. Indian J Dermatol Venereol Leprol [serial online] 2000 [cited 2020 Jul 4];66:251-3. Available from: http://www.ijdvl.com/text.asp?2000/66/5/251/4937
| Introduction|| |
Mycosis fungoides is a cutaneous T-cell lymphoma that is slowly progressive and is usually confined to the skin. Clinically apparent extracutaneous involvement is uncommon and indicates acceleration of the course of the disease. The treatment modalities for mycosis fungoides are numerous with the choice depending on the extent and aggressiveness of the disease, age, general health, compliance of the patient and availability of treatment techniques. PUVA therapy has been used successfully for the treatment of early mycosis fungoides for the past several years. We report our experience in treating a case of mycosis fungoides having no extracutaneous involvement with PUVA combined with topical fluocinolone acetonide.
| Case Report|| |
Five years ago, an 89-year-old man noticed two ill-defined, mildly itchy, erythematous, gradually progressive macular lesions on the trunk which cleared up completely on application of a moderately potent topical steroid but recurred after a few months on stopping the application. Two years later the lesions became more extensive with prominent scaling and some evolved to form edematous scaly plaques on the chest, abdomen and back while others formed large erythematous as well as dark brown macules on the trunk. Plaques also appeared on the upper and lower limbs but there was relative sparing of the face, neck and scalp. The plaques were extremely itchy and some showed areas of erosions, exudation and hemorrhagic crusting [Figure - 1]. He had intermittently received varying doses of oral steroids along with topical steroid applications for the past 3 years with improvement in itching and flattening of the lesions but there had never been a complete remission. Six months prior to presentation he noticed progressive, reticulate, poikilodermatous pigmentation on neck, chest, axilla, abdomen and back. He also had diffuse atrophy of the skin which was more than that expected in a person of his age. One lymph node in each axilla was palpable. There was no history of weight loss, constitutional symptoms, dyspnoea, dysphagia, abnormal bowel movement or erythroderma. He had extensive vitiligo for the past 30 years for which no treatment was taken.
Laboratory investigations showed a mild increase in ESR. Examination of the peripheral smear did not reveal any abnormal cells. Skin biopsy done on 2 occasions showed a dense dermal infiltrate of lymphocytes with a few atypical cells invading the epidermis. Biopsy of an axillary lymph node showed dermatopathic lymphadenitis. Bone marrow aspiration revealed a myeloid reaction with a shift to the left. No mycosis cells were seen. X-ray of the chest and ultrasound examination of the abdomen detected no abnormalities. On the basis of the clinical features and skin and lymph node biopsies, a diagnosis of mycosis fungoides stage II A was made (i.e. skin showing generalised involvement with papules and plaques covering more than 10% of the skin surface, clinically enlarged but histopathologically normal lymph nodes, and no metastases).
The patient was treated with 30 mg of 8methoxy psoralen followed by UVA exposure 2 hours later. He was also advised to apply fluocinolone acetonide 0.1% on itchy and eroded lesions. The initial dose of UVA was 2J/cm. The skin lesions began improving after the first 6 sessions of 2J/cm2 each. The dose of UVA was then increased to 2.5 J/cm. There was steady improvement over the next 2 weeks. After 21 sessions, the infiltration and scaling had almost completely disappeared and there was no itching. The irregular hyperpigmentation and depigmentation however persisted [Figure - 2]. A repeat biopsy was taken which showed significant reduction in the dermal infiltrate. However a few atypical cells were still seen in the epidermis. The patient was then put on a once fortnightly maintenance PUVA and was continuing to do well when he was last seen a month later. He was subsequently lost to follow up.
Modalities available for the treatment of mycosis fungoides without tumours, affecting the skin alone include topical corticosteroids, topical chemotherapy such as mechlorethamine (HN2) and carmustine (BCNU), PUVA, single agent systemic chemotherapy and electron beam irradiation. Beneficial results have been claimed for each of these therapies though there appear to be no formal comparisons of the relative effectiveness of all the different modalities. The choice of therapy in a particular case is determined by the age of the patient, ability to tolerate chemotherapy, and availability of the treatment modality. We chose to use PUVA in our patient because of his age and the dermatitic component of many of the plaques as a measure to relieve troublesome itching. It is also effective in the treatment of early mycosis fungoides and may have contributed to the clinical remission in our patient.
PUVA was first used in the treatment of mycosis fungoides in 1976 and has since been shown to be effective in the treatment of early stage disease in a number of studies.,,, It has been shown to induce a remission in over 80% of patients with skin involvement alone, with no tumours or ulcers. Follow up studies have shown that after initial clearance, remission is maintained for 6.3 - 20 months., Studies of the histopathological changes following PUVA therapy show that in spite of clinical clearance of the lesions the infiltrate persists, though it is reduced in amount. It thus appears that PUVA induces clinical remissions and relieves symptoms but does not cure the disease.
The exact mechanism by which PUVA acts in the treatment of mycosis fungoides is unclear. Improvement may occur by a direct effect on the infiltrating cells in the epidermis and upper dermis.
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[Figure - 1], [Figure - 2]