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Pemphigus: analysis of 1209 cases
Cheyda Chams-Davatchi1, Mahin Valikhani, Maryam Daneshpazhooh
1Tehran University for Medical Sciences, Department of Dermatology, Pemphigus Research Unit, Razi Hospital, Tehran, Iran. cheyda@davatchi.net
International Journal of Dermatology
|June 9, 2005
Summary
Pemphigus vulgaris is the most common form of pemphigus in Iran, affecting females more frequently. Combined skin and mucous membrane involvement in pemphigus vulgaris indicates a worse prognosis.
Area of Science:
- Dermatology
- Autoimmune Diseases
- Epidemiology
Background:
- Pemphigus is a rare, chronic, and potentially fatal autoimmune blistering disease.
- Clinical presentations of pemphigus exhibit significant regional variations worldwide.
Purpose of the Study:
- To characterize the diverse clinical forms of pemphigus within a substantial patient cohort.
- To establish the epidemiological and clinical profile of pemphigus in Iran.
Main Methods:
- A prospective analysis was conducted on 1209 patients diagnosed with pemphigus.
- Data were collected from the Pemphigus Research Unit, Tehran University for Medical Sciences, between 1984 and 2003.
Main Results:
- Pemphigus vulgaris was the predominant subtype (70% with both skin and mucosal involvement).
- The mean age of onset was 42 years, with a female predominance (1.5:1 ratio).
- Pemphigus foliaceus accounted for 7% of cases; most complications were iatrogenic.
Conclusions:
- Pemphigus vulgaris is the most prevalent form in Iran, with a higher incidence in females.
- The incidence rates in Tehran and Iran were 1.6 and 1.0 per 100,000 person-years, respectively.
- Combined mucosal and skin involvement in pemphigus vulgaris correlated with a poorer prognosis.