Pemphigus: Analysis of 1209 cases

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...

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Published in:International journal of dermatology 2005-06, Vol.44 (6), p.470-476
Main Authors: Chams‐Davatchi, Cheyda, Valikhani, Mahin, Daneshpazhooh, Maryam, Esmaili, Nafiseh, Balighi, Kamran, Hallaji, Zahra, Barzegari, Massoume, Akhiani, Maryam, Ghodsi, Zahra, Mortazavi, Hosein, Naraghi, Zahra
Format: Article
Language:English
Subjects:
Adolescent
Adrenal Cortex Hormones - therapeutic use
Adult
Age of Onset
Aged
Aged, 80 and over
Azathioprine - therapeutic use
Biological and medical sciences
Bullous diseases of the skin
Child, Preschool
Dermatology
Female
Fluorescent Antibody Technique
Humans
Immunosuppressive Agents - therapeutic use
Iran - epidemiology
Male
Medical sciences
Middle Aged
Pemphigus - complications
Pemphigus - diagnosis
Pemphigus - drug therapy
Pemphigus - epidemiology
Prospective Studies
Sex Distribution
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Summary: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.
ISSN:0011-9059
1365-4632
DOI:10.1111/j.1365-4632.2004.02501.x