Mahnaz Banihashemi, Yalda Nahidi, Mohammmadjavad Yazdanpanah, Habib Allah - O - Esmaeeli, Somayyeh Khatibzadeh,
Volume 4, Issue 1 (3-2013)
Abstract
Background and Aim: Pemphigus is one of the most common types of autoimmune blistering disease that requires systemic immunosuppressive therapy. Immunosuppressive therapy has improved the disease outcome in recent decades, but long-term use of them has side effects. Recently, it has been tried to evaluate immunosuppressive drugs with less side effects. One of them is mycophenolate mofetil. The aim of this study was to evaluate of therapeutic efficacy of this drug in the patients with refractory pemphigus vulgaris.
Methods: Three hundred files of patients with pemphigus vulgaris were reviewed and among them 28 patients who had received mycophenolate mofetil due to resistance to treatment were entered to this study. The data were obtained from patients’ files and were analyzed using Kendall's tau-c correlation, Mann-Whitney and ANOVA tests.
Results: Male to female ratio was 1.8:1 with an average age of 43.3±13.6 year. Twenty-eight cases of pemphigus vulgaris were treated receiving 2gr/day mycophenolate mofetil + 1mg/kg/day prednisolone. Remission occurred after 3 months. Seventeen patients (10 men and 7 women) had complete remission. There was no significant association between gender and treatment response (P=0.58). There was no significant association between mean age of treated patients and untreated patients with treatment response (P=0.77). Also, there was no significant association between severity of mucosal (P=0.80) and severity of skin involvement with treatment response (P=0.80). Ten patients who received mycophenolate mofetil more than 12 months had treatment response to mycophenolate mofetil and they did not have any relapse in the follow up period.
Conclusion: Mycophenolate mofetil is effective and safe as an adjuvant therapy in patients with pemphigus vulgaris especially in refractory pemphigus. Initiation of the therapeutic effect is slow.
Mahnaz Banihashemi, Fakhrozzaman Pezeshkpoor, Sahar Aziziahari, Mohammad Tohidi,
Volume 5, Issue 4 (Volume 5, Number 4 2015)
Abstract
|
Anderson-Fabry
which is also known as Fabry disease is an X-linked recessive enzyme
deficiency disorder. Its clinical manifestations are caused by storage of
sphingolipids in the lysosomes of the endothelial, perithelial, and smooth
muscle cells, which is due to alpha galactosidase A enzyme deficiency. Its
hallmark dermatological manifestation is diffuse angiokeratomas known as
angiokeratoma corporis diffusum. Peripheral painful neuropathy, eye
involvement, cardiovascular problems, cerebrovascular complications, and
renal failure are other manifestations of Fabry disease.
|
Herein,
we report a 22-year-old man who had been referred to a dermatology clinic for
evaluation of his skin lesions. The patient had diffuse angiokeratomas, which
were characteristic for Fabry disease as well as painful acral neuropathy.
Histpathologic examination of one of his skin lesions was reported as
angiokeratoma. Since cornea verticillata was observed in his eyes and he also
suffered from sensory-neural hearing loss, he was diagnosed as a case of Fabry
disease.
