Treatment of pemphigus vulgaris 
Therapy 
DEFLAZACORT - CYCLOSPORINE A COMBINA TION 
THERAPY IN THE TREATMENT OF PEMPHIGUS 
G. Trevisan, F. Kokelj and G. Lavaroni 
ABSTRACT 
Two cases of pemphigus vulgaris and one of foliaceous pemphigus, treated with deflazacort combined with low doses of 
cyclosporine A, are reported. 
Response and tolerance to treatment were markedly improved over the previous therapy. 
KEYWORDS 
pemphigus, cyclosporine A, immunosuppressants 
INTRODUCTION 
Presently treatment of pemphigus is mainly based on 
steroids alone or in combination with other immuno-
suppressants such as methotrexate, cyclophosphamide and 
azathioprine (1, 2, 3). 
Over the last several years, various authors proposed the 
use of cyclosporine A in the treatment of this immunological 
skin disease (4, 5, 6). 
This report describes our experience with cyclosporine A 
in combination with lowered steroid-dosage in three patients 
affected by different forms of pemphigus. 
acta dermatovenerologica A.P A. Vol 2, 93, No 3 
CASE REPORTS 
The clinical diagnosis of pemphigus in these three patients 
was confirmed histo- and immunohistochemically. 
Case l. 
M.B., a 72-year-old man, was affected by foliaceous 
pemphigus for 14 years . He also suffered from Basedow's 
syndrome, diabetes mellitus, and essential hypertension. He 
has been treated with doses of prednisone (40-60 mg/daily) 
over a long period of tirne. To reduce the side effects of this 
treatment, he has been receiving Deflazacon (mean dosage 
of 18 mg/daily) combined with cyclosporine A (from 2,5 to 
3,2 mg/kg/daily) for the past nine months. Routine 
93 
Treatment of pemphigus vulgaris 
hematochemical tests have been normal in the periodical 
checks: glycemia (range from 151-177 mg/dl) and blood 
pressure have not shown any remarkable increase. 
Case 2. 
D.L., a 35-year-old woman, has been affected with 
pemphigus vulgaris for the last 2 years. We treated her 
initially with Deflazacort (30 mg/daily). After 15 days no 
improvement was observed and we added cyclosporine A 
(3 mg/kg/day); this combination resulted in clinical 
improvement, allowing us to reduce the dosage to a main-
tenance level of 12 mg/dail y Deflazacort and 2,2 mg/kg/dail y 
cyclosporine A. 
Case 3. 
B.G., a 60-year-old woman, is affected with pemphigus 
vulgaris of the oral mucosa. The disease has been present for 
3 years. At the outset she was treated with a combination of 
metilprednisolone ( 40mg/dail y )- azathioprine (50 mg/dail y ); 
the clinical picture showed no major changes. 
Subsequently, she received Deflazacort (24 mg/daily) in 
combination with cyclosporine A (3 mg/kg/daily). This 
therapy resulted in a complete remission, and the treatment 
was reduced to a maintenance dosage of 18 mg/daily 
Deflazacort and 2,25 mg/kg/daily of cyclosporine A . 
DISCUSSION 
Steroids, initially given at high dosage (100-200 mg/ 
dail y ), and then decreased to a maintenance dose, are presen ti y 
considered the standard therapeutic approach for pemphigus 
(1, 2, 3, 7). 
Side effects related to steroid treatment are frequent and 
serious: infections, diabetes, osteoporosis, myopathy , 
·cataracts, nervous and gastrointestinal disorders (1 , 8) . 
Alternative therapies have been tried for the severe forms 
of pemphigus: methotrexate, cyclophosphamide, aza-
thioprine, andin the past years cyclosporine A in monotherapy 
or in combination with steroids (1 , 2, 3, 4, 5, 6, 8, 9). 
The latter is considered useful in order for reducing steroid 
dosage with consequent reduction of side effects (4, 5, 6,). 
However, other authors stress that cyclosporine A in 
monotherapy displays little activity in the acute stage of 
pemphigus vulgaris (4). 
Our data confirm that low dosages of cyclosporine A (less 
than 3 mg/kg/daily) permit a substantial decrease in the 
corticosteroid dosage with a concomitant reduction of the 
side effects. 
REFERENCES 
l. Korman N. Pemphigus. J Am Acad Dermatol 1988; 18: 
1219-1238. 
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3. Piamghongsant T, Ophaswongse S. Treatment of 
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7. Ratnam K V, Phay KL, Tan CK. Pemphigus therapy with 
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8. Kaur S, Kanwar A. Dexamethasone-cyclophosphamide 
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9. Campolmi P, Bonan P, Lotte T, Palleschi GM, Fabbri P, 
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AUTHOR'S ADDRESS 
94 
Giusto Trevisan, M.D., professor of dermatology 
Department ofDermatology Ospedale di Cattinara, 1-34129 Trieste, ltaly 
Franco Kokelj, M.D., professor of de1matology, same address_ 
G. Lavaroni, M.D., professor of dermatology, same address 
acta dermatovenerologica AP.A. Vol 2, 93 , No 3