ABSTRACT
Pemphigus is a rare autoimmune blistering disease with different phenotypes. The evaluation of therapeutic interventions requires a reliable, valid and feasible to use measurement. However, there is no gold standard to measure the disease activity in clinical trials. In this study we aimed to introduce the pemphigus vulgaris activity score [PVAS] measurement and to assess the convergent validity with the experts' opinion of disease activity. In PVAS scoring, the distribution of pemphigus vulgaris antigen expression in different anatomical regions is taking in to account with special consideration of the healing process. PVAS is a 0-18 scale, based on the extent of mucocutaneous involvement, type of lesion and the presence of Nikolsky's sign. The sum of the scores of total number of lesions, number of different anatomic regions involvement and Nikolsky's sign is weighted by the type of lesion. In the present study, PVAS was assessed in 50 patients diagnosed with pemphigus vulgaris by one dermatologist. Independently, five blinded experts scored all the patients through physician's global assessment [PGA]. The convergent validity with experts' opinion was assessed. The Spearman coefficient of correlation showed the acceptable value of 0.751 [95%CI: 0.534- 0.876]. PVAS is a valid, objective and simple-to-use scoring measurement. It showed a good correlation with PGA of pemphigus disease activity in Iranian patients with pemphigus vulgaris
ABSTRACT
Adnexal tumors [ATs] are primary skin tumors with benign or rarely, malignant behavior. They have been classified based on differentiation towards hair follicle, sebaceous, apocrine or eccrine gland. Few large-scale studies have focused on ATs. To determine the prevalence of ATs and to assess clinical and histopathological trend of ATs. A retrospective descriptive study of all ATs diagnosed in Razi hospital between 2006 and 2010 was performed. A total of 30,000 pathology records were reviewed, and 1016 ATs were included. The prevalence of ATs was 3.3%. 518 patients [51%] were female, with a mean age of 34.5 years. 953 tumors [93.8%] were benign. ATs were most commonly located in the head and neck area [822, 83.5%]. The most common histopathological origin of ATs was sebaceous gland [536, 52.7%]. Sebaceous nevus of Jadassohn was the most prevalent single tumor type [40.6% of all ATs]. In 63.6% [646] of tumors, ATs were clinically suspected by the clinician prior to biopsy. The most common malignant AT was sebaceous carcinoma [23, 36.5% of all malignant ATs]. ATs are infrequent lesions, most commonly occurring in 3rd and 4th decade of life. Diagnosis of ATs is made by histopathological studies as they often express indistinctive clinical features. Malignant ATs are rare, occur at an older age, and are often hard to recognize clinically