A Man with
Recurrent Widespread Blistering Eruption
presented by
Henry
Foong FRCP, Ipoh, Malaysia on November
11, 2004
Consultant Dermatologist, Foong
Skin Specialist Clinic, Ipoh, Malaysia
Abstracts
1: Enk AH, Knop J. Mycophenolate is effective
in the treatment of pemphigus vulgaris. Arch Dermatol 1999;135:54-56.
BACKGROUND: Pemphigus vulgaris is a potentially life-threatening
autoimmune disease. Although combination therapies with prednisone
and azathioprine are usually effective in controlling the disease,
some patients either do not respond to this treatment or show early
relapses. OBJECTIVE: To find out whether mycophenolate mofetil would
be an effective drug in controlling pemphigus vulgaris in patients
who failed initial treatment with azathioprine and prednisone. RESULTS:
Twelve patients who were initially diagnosed as having pemphigus
vulgaris and had relapsed while undergoing treatment with azathioprine
(1.5-2 mg/kg of body weight) and prednisolone (2 mg/kg of body weight)
subsequently received combination therapy with mycophenolate mofetil
(2 x 1 g/d) and prednisolone (2 mg/kg of body weight per day). Eleven
of the 12 patients responded to therapy and showed no relapse of
their disease even after tapering of the steroid dose. One patient
did not respond. Toxic effects were low with only mild gastrointestinal
symptoms in 5 patients and mild lymphopenia (World Health Organization
grade I) in 9 patients. During the 9- to 12-month follow-up, none
of the 11 patients showed reappearance of pemphigus lesions. CONCLUSION:
Treatment of pemphigus vulgaris with mycophenolate is a safe and
effective
treatment.
2. Cummins Dl et al Oral cyclophosphamide for treatment
of pemphigus vulgaris and foliaceus. J Am Acad Dermatol 2003;49:276-80.)
Cyclophosphamide is an alkylating adjuvant used in refractory cases
of pemphigus.
Objective We sought to evaluate tlhe effectiveness and safety of
oral cyclophosphamide in the treatment of patients with pemphigus
vulgaris (PV) and pemphigus foliaceus (PF) with refractory disease.
Patients We studied 23 patients with pemphigus (20 with PV; 3 with
PF) who failed to achieve clinical remissions with the use of prednisone
and antimetabolites.
Results Complete remission was achieved in 17 patients with PV
and 2 with PF. A total of 3 patients with PV failed therapy. A partial
remission was achieved in 1 patient with PF. The treatment was administered
for a median duration of 17 months with a follow-up period of 27
months. The median time to complete remission was 8.5 months. A
total of 9 patients who were severely affected received concomitant
plasma exchange. Adverse reactions included 5 cases of hematuria,
6 nonlife-threatening infections, and the development of transitional
cell carcinoma of the bladder 15 years after discontinuation of
cyclophosphamide in 1 patient. No death was associated with cyclophosphamide
treatment.
Conclusion Oral cyclophosphamide is an effective adjuvant in the
treatment of severe and refractory PV and PF, but requires close
monitoring
