Year : 2007 | Volume
: 52 | Issue : 4 | Page : 176--178
ABO blood groups, rhesus factor and pemphigus
Mahin Valikhani, Sima Kavand, Siavash Toosi, Golnaz Kavand, Maryam Ghiasi
Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
Department of Dermatology, Razi Hospital, Pemphigus Research Unit, Tehran University of Medical Sciences, Vahdat Eslami Square, Tehran - 119960
Background: Pemphigus is an autoimmune blistering disease of the skin and mucous membranes with significant mortality and morbidity. Genetic factors are known to be involved in pemphigus. Several studies have reproducibly shown significant associations of ABO blood groups with various autoimmune human diseases. Aim: To study the relationship between ABO and Rhesus (D) blood groups and pemphigus in Iranian patients. Materials and Methods: Data on age, sex, ABO and Rhesus blood type and clinicopathological diagnosis of the patients with pemphigus were collected. A total of 573 patients with pemphigus were assessed for their association with ABO or Rhesus (D) blood groups and compared with the normal population in the area. Results: The distribution of ABO and Rhesus blood groups in patients with pemphigus was similar to the normal local population in Iran. No relationship was found between ABO or Rhesus blood groups and the phenotype of pemphigus. Conclusion: It appears that there is no association between ABO or Rhesus (D) blood groups and the frequency of pemphigus variants in comparison with normal population in Iran.
|How to cite this article:|
Valikhani M, Kavand S, Toosi S, Kavand G, Ghiasi M. ABO blood groups, rhesus factor and pemphigus.Indian J Dermatol 2007;52:176-178
|How to cite this URL:|
Valikhani M, Kavand S, Toosi S, Kavand G, Ghiasi M. ABO blood groups, rhesus factor and pemphigus. Indian J Dermatol [serial online] 2007 [cited 2020 Jul 13 ];52:176-178
Available from: http://www.e-ijd.org/text.asp?2007/52/4/176/37720
Antigens of the ABO blood group family have been known for a long time. The ABO system is the most investigated erythrocyte antigen system for all populations due to the ease of identifying its phenotypes. The genes that determine the A and B phenotypes are found on chromosome 9p and are expressed in a Mendelian codominant manner.  ABO blood groups have been used as a genetic marker in the studies of associations with infectious and noninfectious diseases. , Among the first epidemiological studies to establish associations between the blood groups and the diseases, there were some manifestations of high frequencies of the O blood group and non-secretor phenotype of ABO antigens in patients suffering from peptic ulcers.  Since the association between blood group A and gastric cancer was reported in 1953, the relationship with blood groups had been studied in many cancers and other human diseases.  On the other hand, since the inheritance of blood groups are not confounded by environmental factors, they seem to be a useful source of research for human studies. Case control studies have reproducibly showed significant associations of ABO blood group and particular HLA antigens with various human diseases. These were mainly autoimmune disorders such as juvenile diabetes, multiple sclerosis, rheumatoid arthritis, psoriasis and celiac disease. 
Pemphigus is an autoimmune blistering disease of the skin and mucous membranes with significant mortality and morbidity. It is caused by autoantibodies directed against desmosomes, the principal adhesion structures between epidermal keratinocytes. Binding of autoantibodies leads to the destruction of desmosomes, thereby resulting in the loss of cell-cell adhesion (acantholysis) and the formation of epidermal blisters.  There are five clinical and pathologic variants of pemphigus: (1) pemphigus vulgaris, (2) pemphigus foliaceus, (3) drug-induced pemphigus, (4) paraneoplastic pemphigus, and (5) intercellular IgA dermatosis. Pemphigus vegetans and pemphigus erythematosus are considered as variants of pemphigus vulgaris and pemphigus foliaceus correspondingly.  To date, no etiologic cause is established for pemphigus. Predisposition to pemphigus is linked to genetic factors. First-degree relatives of patients with pemphigus vulgaris are more susceptible to the development of autoimmune diseases than controls , and have a higher incidence of circulating anti-desmoglein antibodies.  Certain major histocompatibility complex class II genotypes, in particular alleles of HLA-DRB1*04 and DRB1*14 sub-types, are common in patients with pemphigus vulgaris across the racial barriers. Pemphigus occurs in association with other diseases characterized by immunological disturbances. 
We found no study published with regard to the frequency of blood groups in pemphigus in the world. Therefore, we decided to study the blood groups in patients presented to our hospital. Because of the possible importance of epidemiological factors such as sex, age and different types of pemphigus, we also studied them in the target population. The frequencies of ABO and Rhesus (D) blood groups vary from one population to another and the genes in charge are distributed differently among socioeconomic groups.  Therefore, an assessment of the relation between blood groups and pemphigus was performed with an outlook to start the study of the genetics of this immunobullous disorder in our environment.
Materials and Methods
ABO and Rhesus (D) blood groups were determined using standardized hemagglutination methods. Data on age, sex, ABO and Rhesus blood groupings and clinical, pathological and immunological diagnosis of the patients with pemphigus and its subtypes were collected in the Razi Hospital in Iran from October 2001 to February 2005. A total of 573 patients with pemphigus were assessed for the association with ABO or Rhesus blood groups. In these series, the histopathological diagnosis of pemphigus was available in all the cases.
The ABO and Rhesus blood group distribution of the blood donors from the Central Blood Bank in Iran was used as the control groups. Entry criteria were those to qualify for routine blood donation, which included the following: (1) adequate general health, (2) adequate hemoglobin level and (3) age of 16-70 years (or greater in donating for autologous usage). There were no exclusion criteria. Demographic data collected for each person were limited to that required for standard blood donation including (1) age, (2) gender and (3) home address.
Data were stored in a computer database and analyzed using a professional statistical computer software (SPSS, version 11). The differences of ABO and Rhesus blood group distribution in patient groups and normal population in the area were assessed using Chi-square test and Fisher's exact test. P values less than 0.05 were considered to be statistically significant.
Of the 573 patients enrolled in this study, 203 (35.4 %) were male and 370 (64.5 %) were female. The mean age at the onset of the disease was 42 years (SD = 14.9). The majority of cases (82%) were between 21 and 60 years of age. The major clinical form of the disease was pemphigus vulgaris, as observed in 537 patients (93.7%). Pemphigus foliaceus was observed in 29 patients (5%), pemphigus vegetans in four patients (0.6%) and pemphigus erythematosus in three patients (0.5%).
ABO blood groupings disclosed 37% (212/573) of blood group O, 33.5% (192/573) of blood group A, 22.9% (131/573) of blood group B and 6.6% (38/573) of blood group AB.
Rhesus blood groupings revealed 88.3% (506/573) of Rh-positive and 11.7% (67/573) of Rh-negative phenotypes. Blood group distribution in different variants of pemphigus is presented in [Table 1].
The results of the ABO and Rhesus (D) screening from Central Blood Bank in Iran shows that blood group O was the highest with 36.35% followed by blood group A, B and lastly AB with 32.14%, 23.72% and 7.79% respectively. The frequency of Rh-positive blood was 89.62%, while 10.38% of the donors were Rh-negative.
The distribution of ABO and Rhesus blood groups in the patients group was not significantly different from that of the normal population with P values of 0.78 and 0.45, respectively. The frequency of ABO and Rhesus blood groups was not different among the four variants of pemphigus enrolled in this study, with P values of 0.3 and 0.39, respectively.
In this study, we found a female-to-male ratio of 1.82:1. The female predominance has been reported previously with ratios from 1.2:1 in France  to 4.1:1 in Tunisia  and Mali. 
Chams et al .  reported the female-to-male ratio of 1.5:1 in the same geographic region as our study. The mean age at the onset of the disease was 42 years, which was the same as that reported by Chams et al.  and nearly similar to some other studies in other countries. ,,,,, The most common clinical form of the disease was pemphigus vulgaris (93.7%) followed by pemphigus foliaceus (5%). The ratio of pemphigus vulgaris to pemphigus foliaceus varies widely in different parts of the world. Pemphigus vulgaris is more common in Jews and probably Mediterranean populations; however, this predilection does not apply to pemphigus foliaceus.  Our data is nearly similar to the high proportion of pemphigus vulgaris to pemphigus foliaceus with a ratio of 12:1, as reported by Chams et al . 
The explanation for the association between ABO blood groups and some special diseases is still unclear. However, the relationship between the blood groups and some disorders has clarified the etiology and pathophysiologic aspects of those disorders.  Pemphigus is a clinically important autoimmune skin disease characterized by intercellular substance antibodies, detected by indirect immunofluorescence on monkey esophagus tissue. It has been observed that anti-A and anti-B blood group antibodies can produce staining patterns similar to that seen in pemphigus.  Although this potential problem is not well recognized, it may propose an association between ABO blood group antigens and pemphigus.
We have observed that the frequency of different ABO or Rhesus blood groups does not differ significantly between the patients with pemphigus and the normal population. This may exclude a probable relationship between blood grouping and pemphigus in our patients. In our opinion, further controlled trials in other socioeconomic groups are required to rule out any relationship between blood group systems and pemphigus. Immunologic response to A and B erythrocytic antigen stimulation was studied in patients with pemphigus, patients with systemic lupus erythematosus and normal subjects. Patients with pemphigus and normal subjects demonstrated a similar specific response (isohemagglutinins).  The distribution of ABO and Rhesus blood groups varies in different geographical and ethnic groups. As a result, proper controls are very important for such studies. The age range of blood donors was limited, excluding children and adults aged more than 70 years, unless the donation was for autologous blood usage. We think this factor may limit the applicability of results in this study to the wider community.
We found no association between ABO or Rhesus (D) blood groups and the frequency of pemphigus variants in comparison with normal population in Iran.
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