BACKGROUND: Pemphigus is a rare and chronic life-threatening disease. The clinical picture varies in reports from different regions of the world. OBJECTIVE: To define the clinical forms of pemphigus in a large cohort of patients. METHODS: Prospective analysis of 1209 patients diagnosed and followed at the Pemphigus Research Unit, Tehran University for Medical Sciences, from 1984 to 2003. RESULTS: The mean age at onset was 42 years with a female to male ratio of 1.5/1. The most frequent form was pemphigus vulgaris. In pemphigus vulgaris, patients' mucous membrane involvement alone was observed in 18%, skin involvement alone in 12%, and both in 70%. Pemphigus foliaceus was observed in 7% of the patients. Most complications were iatrogenic. CONCLUSION: In Iran, pemphigus vulgaris is the most frequent form of pemphigus. Females are more prone to the disease. The incidence of pemphigus in Tehran is approximately 1.6 per 100,000/year, and in Iran 1.0 per 100,000/year. The age of onset was lower than classically reported. Death occurred in 6.2% of the patients. In pemphigus vulgaris, the mucosal and skin form together had a worse prognosis than the other clinical forms.
BACKGROUND: Pemphigus is a rare and chronic life-threatening disease. The clinical picture varies in reports from different regions of the world. OBJECTIVE: To define the clinical forms of pemphigus in a large cohort of patients. METHODS: Prospective analysis of 1209 patients diagnosed and followed at the Pemphigus Research Unit, Tehran University for Medical Sciences, from 1984 to 2003. RESULTS: The mean age at onset was 42 years with a female to male ratio of 1.5/1. The most frequent form was pemphigus vulgaris. In pemphigus vulgaris, patients' mucous membrane involvement alone was observed in 18%, skin involvement alone in 12%, and both in 70%. Pemphigus foliaceus was observed in 7% of the patients. Most complications were iatrogenic. CONCLUSION: In Iran, pemphigus vulgaris is the most frequent form of pemphigus. Females are more prone to the disease. The incidence of pemphigus in Tehran is approximately 1.6 per 100,000/year, and in Iran 1.0 per 100,000/year. The age of onset was lower than classically reported. Death occurred in 6.2% of the patients. In pemphigus vulgaris, the mucosal and skin form together had a worse prognosis than the other clinical forms.
Authors: Alex M Rokni; Marissa Ayasse; Adnan Ahmed; Lauren Guggina; Robert W Kantor; Jonathan I Silverberg Journal: Arch Dermatol Res Date: 2022-03-09 Impact factor: 3.017
Authors: Mina Kalantari-Dehaghi; Yumay Chen; Wu Deng; Alex Chernyavsky; Steve Marchenko; Ping H Wang; Sergei A Grando Journal: J Biol Chem Date: 2013-04-18 Impact factor: 5.157
Authors: Mh Javanbakht; M Daneshpazhooh; C Chams-Davatchi; Mr Eshraghian; M Zarei; M Chamari; Djalali M Journal: Iran J Public Health Date: 2012-05-31 Impact factor: 1.429
Authors: Mina Kalantari-Dehaghi; Grant J Anhalt; Michael J Camilleri; Alex I Chernyavsky; Sookhee Chun; Philip L Felgner; Algis Jasinskas; Kristin M Leiferman; Li Liang; Steve Marchenko; Rie Nakajima-Sasaki; Mark R Pittelkow; John J Zone; Sergei A Grando Journal: PLoS One Date: 2013-03-07 Impact factor: 3.240