The Arab world lies geographically between the Atlantic coasts of northern Africa and the Arabian Gulf. This area has wide latitudinal differences as well as variable environmental conditions ranging from deserts to forests. Approximately 370 million individuals who share the Arabic language live in this area. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the main subtypes of the pemphigus disease. Both pemphigus subtypes are present in many Arab countries; however, there is variation in the predominant subtype among countries. PV is the most common subtype in Egypt, Sudan, Morocco, Syria, Kuwait, Saudi Arabia and Yemen. On the other hand, PF is more prevalent in Libya and is endemic in Tunisia. Interestingly, there is variation in the dominant subtype in some cities within Morocco. For example, PF is more common in Marrakech which is the second largest city. The presence of anti-desmoglein 1 antibodies in the sera of normal Tunisians and the presence of anti-desmoglein 3 in normal Egyptians' sera suggested that environmental factors played a role in the disease pathogenesis in those areas. Further researches detected that traditional cosmetics were among the risk factors in Tunisia. Moreover, farming was suggested as a risk factor in Egypt, Tunisia and Sudan. Because there is no consensus for pemphigus treatment among the Arab countries, there is diversity in their pemphigus treatment regimens. Studying the demographic characteristics and the environmental conditions which caused the variations in the prevailing clinical phenotype will help us fill the gaps to understand the pathogenesis of the pemphigus disease.