seyed mohammad ali mortazavizadeh

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.

We present a case of epitheloid sarcoma that was referred with a 2×3cm ulcerated lesion on the right ankle and edema of the lower leg. Foot drop of the right side was present that had caused walking difficulty. After a few months, he developed several sporthricoidal nodular lesions on the medial aspect of right thigh, inguinal lymphadenopathy, weight loss, anorexia and respiratory symptoms. Chest x-ray and HRCT showed pulmonary metastasis. Histopathological evaluation and immunohistochemical profile of both skin lesion and involved inguinal lymph node were consistent with epitheloid sarcoma. The case is interesting because as far as we know there has been no report of epitheloid sarcoma in literature presenting with foot drop and edema prior to obvious skin involvement.