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Year : 1990  |  Volume : 56  |  Issue : 1  |  Page : 40-42

Reversal of systemic sclerosis with dexamethasone pulses

Correspondence Address:
J S Pasricha

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A 22 year old female had for the last 8 years,, progressive tightness of the skin along with hyperpigmentation, Raynaud's phenomenon, calcinosis cutis over bony prominences, dysphagia and pulmonary involvement. Treatment with 100 mg dexamethasone given intravenously in 5% dextrose on 3 consecutive days every month led to a significant reversal of all the clinical manifestations over a period of 18 months.

Keywords: Systemic sclerosis, Treatment, Dexamethasone pulse therapy

How to cite this article:
Pasricha J S, Ramam M, Shah S. Reversal of systemic sclerosis with dexamethasone pulses. Indian J Dermatol Venereol Leprol 1990;56:40-2

How to cite this URL:
Pasricha J S, Ramam M, Shah S. Reversal of systemic sclerosis with dexamethasone pulses. Indian J Dermatol Venereol Leprol [serial online] 1990 [cited 2020 May 30];56:40-2. Available from: http://www.ijdvl.com/text.asp?1990/56/1/40/3478

Systemic sclerosis is commonly considered to be a progressive disease, ultimately leading to deformities, incapacitation and even fatal termination. Most textbooks of medicine refer to it as progressive systemic sclerosis.[1],[2],[3] We believe that the progress of the disease- can be arrested and even reversed with a judicious selection of therapeutic measures. The patient being reported by us had progressive disease for the last 8 years, but treatment with inter­mittent high doses of dexamethasone showed significant reversal of all the clinical mani­festations, without major side effects.

  Case Report Top

Eight years ago, a 22-year-old female noticed tightness of the skin of her hands which gradually progressed to involve her entire body. Six months after the onset, she noticed swelling on her face, hands and feet. The swelling would occur during the winter months, especially when the hands were immersed in cold water and was preceded by bluing of her hands and feet. She would also have recurrent ulceration of the finger-tips and her nails used to turn white, the colour returning to normal on warming the hands. Five years ago, she noticed gradually increasing pigmentation of the skin starting from the face and later involving the hands, forearms, feet, neck, axillae and knees. An year later, she developed calcinosis over the iliac crest, gluteal region, upper right thigh and knee, both elbows and hands. She was also having dysphagia, and fever with chills and rigors, for the last 3 years. She was diagnosed to have scleroderma which was confirmed by a skin biopsy. Barium swallow revealed hypo­motility of the oesophagus with delayed clearance of the dye. Peripheral blood picture showed microcytic, hypochromic anemia, with hemo­globin 8 gm%, PCV 27%, reticulocyte count 5%, RBC 3 million and ESR 46 mm. Chest X-ray, liver and kidney function tests were normal. Tests for LE cells, rheumatoid factor and ANA were negative. A radio-immuno­assay for antibodies to double stranded DNA was 32 units/ml (normal upto 15 units/ml). ECG was normal. She had been treated with prednisolone 5 mg daily, increased to 15 mg daily along with 500 mg of complamina retard, but her condition progressively worsened. In August 1987, when first seen by us, she had diffuse mottled pigmentation and induration of the skin all over the body, especially on her face, neck, arms and thighs. Calcinosis cutis was present over the iliac crest, gluteal region, right thigh, knee, elbows and hands. Pulmonary function tests revealed restrictive changes. ANF and ENA were negative. Her skin felt hard and unyielding and she had difficulty in moving her joints. We treated her with 100 mg dexame­thasone in 5% dextrose given intravenously on 3 consecutive days (Dexamethasone pulse) at monthly intervals along with topical 5% hydro­ quinone solution twice a day for hyperpigmented areas. After the fourth pulse, the patient started noticing improvement with softening of the skin and decrease in its stiffness. By February 1989 (18 pulses), the skin had softened to near normal consistency, there was almost no Raynaud's phenomenon throughout the winter, dysphagia and respiratory distress had dis­appeared, and calcinosis and skin pigmentation had decreased considerably. Measurement of the thickness of the fold of skin at various body sites [Table - 1] showed progressive reduction, and the pulmonary function tests showed consi­derable improvement [Table - 2].

Since March 1989, further pulses have been stopped and there has been no relapse.

  Comments Top

Pulse therapy (high dose intermittent therapy) with corticosteroids has been successfully used for several skin diseases.[4],[5],[6],[7] It has two distinct advantages over the conventional daily dose therapy. Firstly, the very high doses used for pulse therapy often produce therapeutic respon­ses in cases when conventional daily doses are without any effect even when the daily doses are large enough to lead to significant side effects. Secondly, the intermittent therapy does not produce any of the well known side effects of prolonged corticosteroid therapy.

After our success with pulse therapy in pemphigus,[8],[9] we felt encouraged to try this therapy in other diseases which seem difficult to treat. We have obtained gratifying results in Reiter's disease 10 (2 cases), pyoderma gangre­ nosum (3 cases), porokeratosis (1 case) am Darier's disease (I case). In pemphigus and few other bullous disorders, this mode of treat ment has been especially successful because there have been no relapses even on prolongec follow-up after the treatment has been with drawn.[9]

The results obtained in this patient witl systemic sclerosis have been certainly more thar what we anticipated. Not only was the progress of the disease arrested, but the pathological changes showed significant reversal to almost near normal levels. It will however be necessary to follow-up this patient for several years after the withdrawal of treatment to look for any recurrence of the disease, and also to try this method for other cases as well to see if all of them will have similar improvement. It is alsc important to remember, that although pulse therapy does not produce the commonly known side effects of prolonged corticosteroid therapy; such as weight gain, diabetes mellitus, hyper­tension, osteoporosis etc,[11] it is necessary to be alert about pyogenic and fungal infections and reactivation of tuberculosis which have been noticed in some cases having dexamethasone cyclophosphamide pulse therapy.[9]

  References Top

1.Gilliland BC : Progressive systemic sclerosis, in Harrison's Principles of Internal Medicine, Eleventh ed, Editors, Braunwald E, Isselbacher KT, Petersdorf RG et al : McGraw Hill Book Company, New York, 1987; p 1428-1432.  Back to cited text no. 1    
2.Nuki G : Progressive systemic sclerosis, in : Davidson's Principles and Practice of Medicine, Fourteenth ed, Editor, McLeod J : Churchill Livingstone, New York, 1984; p 575-576.  Back to cited text no. 2    
3.Wigley FM : Progressive systemic sclerosis, in The Principles and Practice of Medicine, Twenty first ed, Editors : Harvey AM, Johns RJ, McKusick VA et al Prentice-Hall International Inc, Englewood Cliffs, 1984; p 1143-1146.  Back to cited text no. 3    
4.Bell PRF, Calman KC, Wood RFM et al Reversal , of acute clinical and experimental organ rejection using large doses of intravenous prednisolone, Lancet, 1971; 1 : 876-880.  Back to cited text no. 4    
5.Levinsky RJ, Cameron JS and Soothill JF Serum immune complexes and disease activity in lupus nephritis, Lancet, 1977; 1 : 564-567.  Back to cited text no. 5    
6.Liebling MR, Leib E, Mclaughlin K et al Pulse methyl-prednisolone in rheumatoid arthritis, Ann Int Med, 1981; 94 : 21-26.  Back to cited text no. 6    
7.Johnson RB and Lazarus GS : Pulse therapy, Arch Dermatol, 1982; 118 : 76-84.  Back to cited text no. 7    
8.Pasricha JS, Thanzama J and Khan UK : Inter­mittent high-dose dexamethasone-cyclophospha­mide therapy for pemphigus, Brit J Dermatol, 1988; 119 :73-77.  Back to cited text no. 8    
9.Pasricha JS, Seetharam KA and Das U : Further studies on pemphigus patients treated with dexamethasone-cyclophosphamide pulse therapy, Ind J Dermatol Venereol Leprol, 1989; 55 : 98-104.  Back to cited text no. 9    
10.Pasricha JS and Ramii Gupta : Pulse therapy with dexamethasone in Reiter's disease, Ind J Dermatol Venereol Leprol, 1982; 48 : 358-361.  Back to cited text no. 10    
11.Pasricha JS and Ramji Gupta : Pulse therapy with dexamethasone-cyclophosphamide in pemphigus, Ind J Dermatol Venereol Leprol, 1984; 50 199-203.  Back to cited text no. 11    


[Table - 1], [Table - 2]

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