A young man with an ulcerated lesion on the right ankle.

We present a case of epitheloid sarcoma that was referred with a 2x3 cm 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.

Pemphigus vulgaris activity score and assessment of convergent validity.

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.

Pemphigus and pregnancy: a 23-year experience.

BACKGROUND: Studies on the interaction of pemphigus and pregnancy are limited to case reports and small case series. Pregnancy is not rare in Iranian pemphigus patients due to lower age at onset of the disease. AIM: We sought to investigate the outcome of pregnancy and the course of pemphigus in pemphigus patients in a retrospective study. METHODS: The files of 779 pemphigus women younger than 50 presenting to our center from 1984 till 2006 were examined for any mention of pregnancy. Data related to outcome of pregnancy and the course of the disease were collected. RESULTS: Sixty-six patients with a history of pregnancy were identified. Forty-eight known pemphigus patients experienced one or more pregnancies during their disease (total pregnancy number: 52). The course of pemphigus was as follows in this group: 28 cases (54%) of exacerbation, 15 cases (31%) with no alteration, and 9 cases (17%) showing improvement. The rate of abortion was 9.6% (5 cases). In 18 cases, the disease had started during pregnancy, 2 of them (11%) ended in an abortion. Overall, postpartum flare was seen in 33 cases (47.1%). CONCLUSION: Pemphigus may be exacerbated during or after pregnancy, but often to a mild degree. Although the rate of stillbirth was not as high as previously reported, the rate of abortion was considerable. Pregnancy may have an uneventful course, especially in patients in clinical remission; nevertheless, careful monitoring of the high risk mother and fetus is mandatory.

Pemphigus and pregnancy: A 23-year experience.

Background: Studies on the interaction of pemphigus and pregnancy are limited to case reports and small case series. Pregnancy is not rare in Iranian pemphigus patients due to lower age at onset of the disease. Aim: We sought to investigate the outcome of pregnancy and the course of pemphigus in pemphigus patients in a retrospective study. Methods: The files of 779 pemphigus women younger than 50 presenting to our center from 1984 till 2006 were examined for any mention of pregnancy. Data related to outcome of pregnancy and the course of the disease were collected. Results: Sixty-six patients with a history of pregnancy were identified. Forty-eight known pemphigus patients experienced one or more pregnancies during their disease (total pregnancy number: 52). The course of pemphigus was as follows in this group: 28 cases (54%) of exacerbation, 15 cases (31%) with no alteration, and 9 cases (17%) showing improvement. The rate of abortion was 9.6% (5 cases). In 18 cases, the disease had started during pregnancy, 2 of them (11%) ended in an abortion. Overall, postpartum flare was seen in 33 cases (47.1%). Conclusion: Pemphigus may be exacerbated during or after pregnancy, but often to a mild degree. Although the rate of stillbirth was not as high as previously reported, the rate of abortion was considerable. Pregnancy may have an uneventful course, especially in patients in clinical remission; nevertheless, careful monitoring of the high risk mother and fetus is mandatory.