[ relation between thiopurine methyl transferase activity and efficacy and side effects of azathioprine in patients with pemphigus vulgaris]

Azathioprine is the most widely used immunosuppressive agent as an adjunct to corticosteroids in the treatment of pemphigus vulgaris [PV]. Thiopurine methyl transferase [TPMT] is a key enzyme in azathioprine metabolism and a genetic polymorphism controls its activity in human tissue. TPMT activity can provide a rational basis to determine suitable dose of azathioprine, theoretically. The aim of this study was to evaluate the clinical relevancy of this hypothesis in PV patients. In this cross sectional study in Razi Hospital, the activity of TPMT in the red blood cells of 52 PV patients who received azathioprine for at least 12 months and 29 PV patients who did not receive this drug was measured and correlated to the clinical response and side effects observed. The mean of TPMT activity was not significantly different in patients with unfavourable response, comparing to patients with favorable response to azathioprine [P=0.087]. No relationship was observed between total dose of corticosteroid and TPMT activity [r=0.089, P=0.583]. There was no difference between the mean of TPMT activity in patients receiving azathioprine and those not receiving this drug [P=0.36]. A direct relationship was not observed between TPMT activity and clinical efficacy and side effects in PV patients under treatment with azathioprine. Larger prospective studies in more homogenous patients are needed to evaluate the clinical relevance of TPMT polymorphism and to determine accurate azathioprine dosing guidelines based on TPMT activity

[Frequency of hirsutism in medical students in Tehran]

Hirsutism is a common clinical problem among women; however estimates regarding its prevalence in our population are limited. The objective of this study was to estimate the frequency and features of hirsutism in yound women in Iran. This descriptive-cross sectional study was performed on 800 female [18-25 years] medical students of Tehran medical universities in 1999. Evaluation included a history and a modified Ferriman-Gallwey hirsutism score in four different androgen-sensitive skin areas. The volunteers recorded their weight and height, the presence of menstural dysfuncion, acne, positive family history for hirsutism, as well as ingestion of oral contraceptive pills, corticosteroids or anabolic steroids using a questionnaire. Data were analyzed using chi-square test and P<0.05 was considered significant. The frequency of hirsutism in our population was 22.8% [183 of 800]. 17.3%, 5.1% and 0.3% of the population studied had mild, moderate and severe hirsutism, respectively. The prevalence of obesity [body mass index > 25], acne, positive family history for hirsutism, menstrual dysfunction, and consumption of corticosteroid and OCP was significantly higher in hirsutes. Hirsutism is a very common abnormality in this group of woman in Iran. Higher hirsutism frequency in this survey comparing to previous records in other population shows that the hirsutism definition and scoring system should be modified due to racial/ethnic variations in terminal hair quantity and distributions and different concept of this abnormality in each population

[Association of pemphigus vulgaris and tuberous sclerosis: a case report]

Pemphigus vulgaris is a blistering autoimmune disease with suprabasal cleft formation, which is the most common autoimmune blistering disease in eastern countries, such as Middle East. Predisposition to pemphigus is linked to genetic factors. Tuberous sclerosis is also a genetic disorder of hamartoma formation in many organs, particularly the skin, brain, eye, kidney and heart. We report a 17-year-old boy with pemphigus vulgaris associated with tuberous sclerosis, which to our knowledge the first report of this association