Pemphigus vulgaris is an autoimmune bullous dermatosis that affects between 0,1 and 0,5%000 of the population. The prognosis of the disease is determined by the severity of the side effects of corticosteroid therapy and their compensation. The present study covered 52 patients with pemphigus vulgaris treated in the Clinic of Dermatology and Venereology, Prof. P. Stoyanov Medical University of Varna, during a 10-year period (1990-2000). There were 20 males aged between 28 and 86 years and 32 females aged between 17 and 77 years. The adverse effects of the corticosteroids in the patients with pemphigus vulgaris and their influence on the course of the disease were  described. The combination with a cytostatic drug (cyclophosphamide or imuran) proved to be  effective in reducing the total course and maintenance dosage of the corticostteroids in these  patients

Pavlov, S.

Slavova, M.

English

Varna Medical University Press: Journals

Scripta Scientifica Medica, vol. 34 (2002), pp 85-86 Copyright © Medical University, Varna SIDE E F F E C T S AND PROGNOSIS W I T H T H E T R E A T M E N T O F PEMPHIGUS V U L G A R I S S. Pavlov, M. Slavova Clinic of Dermatology and Venereology, Department of Infectious Diseases and Epidemiology, Prof. Paraskev Stoyanov Medical University of Varna A B S T R A C T Pemphigus vulgaris is an autoimmune bullous dermatosis that affects between 0,1 and 0,5%000 of the popula­tion. The prognosis of the disease is determined by the severity of the side effects of corticosteroid therapy and their compensation. The present study covered 52 patients with pemphigus vulgaris treated in the Clinic of Dermatology and Venereology, Prof. P. Stoyanov Medical University of Varna, during a 10-year period (1990-2000). There were 20 males aged between 28 and 86 years and 32 females aged between 17 and 77 years. The adverse effects of the corticosteroids in the patients with pemphigus vulgaris and their influence on the course of the disease were described. The combination with a cytostatic drug (cyclophosphamide or imuran) proved to be effective in reducing the total course and maintenance dosage of the corticostteroids in these pa­tients. Key words: pemphigus vulgaris, corticosteoids, cytostatics, side effects, prognosis Pemphigus vulgaris has been described by Wichmann in 1791. It is an autoimmune blistering disease presenting with acantholysis and bullous-erosive cutaneous and mucosal skin lesions (1,7). The natural course of the disease is progressive and leads to fatal outcome until the end of the first year in 75% of the patients. There is major progress in the understanding of its pathophysiology and in the devel­opment of new diagnostic techniques and therapeutic ap­proaches (6). Incorporation of corticosteroids (CS) into the therapy since 1950 onwards sharply reduces the death rate down to approximately 30% (10). Recently, there is a con­tinuous trend towards a lower mortality rate and nowadays it is below 10%. Although CS use remains the cornerstone of effective therapeutic regimens for pemphigus vulgaris, such a prolonged administration leads to unavoidable side effects determining the prognosis (2,8). Systemic immunosuppression makes use of CS such as azathioprine, dapsone, methotrexate, cyclophosphamide and gold as ad­juvants or alternatives (9). Immunomodulation is widely applied, too (2,5,6). High-dose intravenous immunoglobu­lin is effective in 81% of the patients with pemphigus vulgaris which are more likely to respond to adjunctive therapy than to monotherapy (91 % and 56% response rate, respectively) (4). Pulse intravenous cyclophosphamide is particularly efficacious and low toxic (3). Both cyclophosphamide and methylprednisolone "pulse ther-Address for correspondence: S. Pavlov, Clin, of Dermatology and Venereology, Dept. of Infectious Diseases and Epidemiology, Prof. P. Stoyanov Medical University of Varna, 55 Marin Drinov St, BG-9002 Varna, BULGARIA ару" are reserved for cases unresponsive to high doses of oral CS (8). The purposes of the present study are to follow-up the ther­apeutic results from the treatment of the patients with pemphigus vulgaris, the adverse effects of the therapy with CS alone and in combination with cytostatic drugs and to suggest a model of therapeutic behaviour for minimization these unwanted effects. M A T E R I A L AND M E T H O D S The study covered a total of 52 patients with pemphigus vulgaris treated in the Clinic of Dermatology and Venereology, Prof. P. Stoyanov Medical University of Varna, during a 10-year period (January 1, 1990 - January 1, 2000). They represented 0,95% of 5447 patients hospi­talized in the Clinic of Dermatology and Venereology dur­ing this period. Their total hospital sojourn amounted to 4050 days (4,12% of the days of all the patients). There were 20 males (38,5%) aged between 28 and 86 years and 32 females (61,5%) aged between 17 and 77 years. Pa­tients' records were analyzed by means of a special file card taking into consideration the following items: i) from the anamnesis and status: gender, age, primary lo­calization of lesions, accompanying diseases, duration of remissions, number of hospital days; ii) from the paraclinical parameters: ESR, leukocyte count, blood sugar, urea, histologic examinations of skin and mucosal membranes, iii) from the treatment: kind and initial dosage of CS, total weekly and average daily dosage for achieving the remis­sion, and 85 S. Pavlov. M . Slavova I complications from CS therapy and cause for death. R E S U L T S AND D I S C U S S I O N I the patients were treated with CS such as hydrocorti-те, prednisolone, methylprednisolone (MP), and dexa-rthasone. The mean initial dose was equivalent to 57,5 I of MP/24 hours. The mean daily dose of CS varied m 54,3 lmg MP/24 hours in the first week down to ,03mg MP/24 hours in the fourth month. The average lintenance dose was of ll,13mg MP/24 hours. A ostatic drug such as cyclophosphamide or imuran was led to the therapy in one half of the patients admitted to ipital. The mean daily dosage for achieving the remis-n was of 186,5mg/24 hours for cyclophosphamide and 3,5mg/24 hours for imuran. The hospital stay of the pa-its treated with CS alone was on the average 30,2 hospi-days long while that of the patients with accompanying ostatic therapy was 48,6 hospital days long. The remis-n occurred between the third and the eight week with st patients. It is, therefore, accepted that the duration of hospital treatment coincides with the time required for lieving the remission. mplications due to CS therapy were observed in 44 pa-its (85% of the acses) treated longer than one year. Arte-hypertension was most common - in 17 patients ,6%). Next came obesity and fades limata - in 13 pa-its (25%), steroid diabetes and disturbed glucose toler-e as well as bacterial infections such as abscessus. pneu-nia and sepsis - in 7 patients (13,4%) each, proximal opathy - in 6 patients (11,5%), severe osteoporosis with lological fractures - in 3 patients (5,7%), thromboses embolisms - in 2 patients (3,8%). The sum was higher l 52 as there were more complications in one and the le patient. Six patients (11,5% of the cases) died during a year period from the onset of the disease. The death re­ed from a progressive cardiovascular and respiratory ire in 4 patients, from pulmonary thromboembolism from cerebral stroke - in one patient each, side effect of CS required cessation of the treatment as it /ithout any alternative at all. The arterial hypertension compensated by antihypertensive dings in 32,6% of patients. The course of the bacterial infections was pro-ted and with relapses in 13,4% of the cases. As a rule, • were managed with antibiotic combinations rather I by monotherapy alone. Increasing overweight and re-ibution of fatty deposits in 25% of the patients tended ormalization during the periods of longer remissions, vever, the intake of Ca 2* preparations and the periodic relic therapy failed to prevent the progressive osteopo-; having provoked pathological bone fractures and a :imal neuropathy. The adverse effects of CS in the pa-s with pemphigus vulgaris are in a direct dependence le sum dosage and duration of treatment. One way to ce this dosage is to combine the immunosuppressive :t of CS with that of the cytostatics like in one half of Datients. The immunosuppressive effect of imuran in a dose of 3-4mg/kg b. w. occurred after 3-4 weeks and coin­cided with the reduction of CS dosage after achieving the morbistasis. Its relatively better tolerability has made it ap­propriate for a long-lasting maintenance therapy for 12-24 months. Cyclophosphamide was effective in a dosage of 2-3 mg/kg b. w. It was used in patients unresponsive to CS only because of its higher toxicity. C O N C L U S I O N ^ With a view to reduction of the total course and mainte­nance dosage of CS it seems reasonable to combine these drugs with cytostatic ones even in the slight forms of pemphigus vulgaris. The different therapeutic protocols should obligatorily be accompanied with a strict control of the arterial blood pressure and blood sugar. Additionally, use of prophylactic gastroscopy and bone densitometry is strongly recommended, particularly in menopausal women. R E F E R E N C E S 1. C a r s o n , P . J . , A . Hameed , A . R . A h m e d . In­fluence of treatment on the clinical course of pemphigus vulgaris.- J. Am. Acad. Dermatol., 34, 1996, No 4, 645-652. 2. F e l l n e r , M . J . , A . N . S a p a d i n . Current therapy of pemphigus vulgaris. - Ml Sinai J. Med., 68, 2001, No 4-5, 268-278. 3. F o x , L . P . , A . G . P a n d y a . Pulse intravenous cyclophosphamide therapy for dermatologic disor­ders.- Dermatol. Clin., 18, 2000, No 3, 459-473. 4. J o l l e s , S . A review of high-dose intravenous immu­noglobulin (hdivIG) in the treatment of the autoim­mune blistering disorders.- Clin. Exp. Dermatol., 26, 2001, No 2, 127-131. 5. K o r m a n , N . J . New and emerging therapies in the treatment of blistering diseases.- Dermatol. Clin., 18, 2000, No 1, 127-137. 6. K o r m a n , N . J . New immunomodulating drugs in autoimmune blistering diseases.- Dermatol. Clin., 19, 2001, No 4, 637-648. 7. L e v e r , W. F . , G . S c h a u m b u r g - L e v e r . Treat­ment of pemphigus vulgaris. Results obtained in 84 patients between 1961 and 1982.- Arch. Dermatol. Res., 120, 1977, No 1,44-47. 8. M i g n o g n a , M . D . , L . L o M u z i o , R . E . Mignogna , R . Ca rbone , E . Ruoppo , E . B u c c i . Oral pemphigus: long term behaviour and clinical response to treatment with deflazacort in six­teen cases.- J. Oral Pathol. Med., 29, 2000, No 4, 145-152. 9. S c u l l y , C , S. J . C h a l l a c o m b e . Pemphigus vulgaris: update on etiopathogenesis, oral manifesta­tions, and management.- Crit. Rev. Oral Biol. Med., 13, 2002, No 5, 397-408. 10. S e i d e n b a u m , M . , M . D a v i d , M . Sandbank . The course and prognosis of pemphigus. A review of 115 patients.- Int. J. Dermatol., 27, 1988, No 8, 580-584. 

SIDE EFFECTS AND PROGNOSIS WITH THE TREATMENT OF PEMPHIGUS VULGARIS

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SIDE EFFECTS AND PROGNOSIS WITH THE TREATMENT OF PEMPHIGUS VULGARIS

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