Bullous Pemphigoid is a chronic immunobullous disease, characterized by subepidermal bulla on the skin and mucosa. The purpose of the present study was to investigate the clinicoepidemiological features of bullous pemphigoid in Iranian patients. In this retrospective descriptive study, we reviewed 82 bullous pemphigoid patients within 2014-2016. The mean age of the patients was 67.13 years (range between 25 and 97) including 32(39.1%) males and 50(60.9%) females. Mucosal involvement was positive in 33(40.2%) of the patients, and oral mucosa was most commonly involved. Head and neck area were involved in 43 (52.4%) of the patients. Clinical and epidemiological characteristics of bullous pemphigoid are different in different regions of the world

Alireza Delavari

Arghavan Azizpour

Hamidreza Mahmoudi

Kamran Balighi

Maryam Daneshpazhooh

Maryam Nasimi

ACTA MEDICA IRANICA

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 ORIGINAL ARTICLE   Corresponding Author: A. Azizpour Department of Dermatology, Autoimmune Bullous Diseases Research Center ,Tehran University of Medical Sciences, Tehran, Iran  Tel: +98 21 55618989., Fax: +98 2155618989, E-mail address: azizpour63@gmail.com  Clinicoepidemiological Features of 82 Cases of Bullous Pemphigoid in Tehran, Iran Kamran Balighi1, Maryam Daneshpazhooh1, Hamidreza Mahmoudi1, Maryam Nasimi1, Alireza Delavari2, Arghavan Azizpour1 1 Department of Dermatology, Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran Hospital, Tehran University of Medical Sciences, Tehran, Iran Digestive Oncology Research Center, Digestive Disease Research Institute, Shariati 2  Received: 17 Mar. 2018; Accepted: 09 Jun. 2018  Abstract- Bullous Pemphigoid is a chronic immunobullous disease, characterized by subepidermal bulla on the skin and mucosa. The purpose of the present study was to investigate the clinicoepidemiological features of bullous pemphigoid in Iranian patients. In this retrospective descriptive study, we reviewed 82 bullous pemphigoid patients within 2014-2016. The mean age of the patients was 67.13 years (range between 25 and 97) including 32(39.1%) males and 50(60.9%) females. Mucosal involvement was positive in 33(40.2%) of the patients, and oral mucosa was most commonly involved. Head and neck area were involved in 43 (52.4%) of the patients. Clinical and epidemiological characteristics of bullous pemphigoid are different in different regions of the world. © 2018 Tehran University of Medical Sciences. All rights reserved.  Acta Med Iran 2018;56(8):522-525.  Keywords: Bullous pemphigoid; Immune bullous disease; Pemphigoid    Introduction  Bullous pemphigoid is the most common form of pemphigoid diseases. These diseases are a group of well-defined autoimmune disorders that are characterized by autoantibodies against dermal-epidermal junction structural proteins leads to the separation of the epidermis and dermis.  Bullous pemphigoid mainly affects older people, with onset usually in the late 70s. Bullous pemphigoid rarely occurs in individuals younger than 50 years (1,2). Bullous pemphigoid is clinically characterized with tense, clear, blisters and erythema, which frequently arise on the flexural aspects of the limbs and on the abdomen and can persist for several days. In almost all patients, lesions are severely pruritic. Mild oral lesions develop in 10-20% of patients, but other mucosal areas are rarely affected (3). This survey was conducted to determine the epidemiological characteristics, clinical features and frequency of mucosal lesions, in patients with bullous pemphigoid who were referred to dermatology clinics at the Razi Hospital in Tehran from 2014 to 2016.    Materials and Methods  This was a retrospective descriptive cross-sectional survey carried out in the Autoimmune Bullous Diseases Research Center of Razi Hospital, Tehran, Iran, from May 2014 to October 2016. The patient data presented in this study have not been previously published.  Diagnosis of the patients was confirmed with histology findings and linear deposition of IgG and C3 in Direct Immuno-fluorescence (DIF). The following clinical and histological data were chronicled from records of the patients; age and gender at the time of diagnosis, histological findings in the pathology report, DIF and ELISA report, anatomical location of the lesions, and involvement of mucosal surfaces.  Statistical analysis The SPSS version 20.0 software package was used in the statistical analysis. The descriptive values obtained in the study are presented as means±SD for quantitative variables and as frequencies for qualitative ones. For the comparison of frequencies, the chi-square test or Fisher’s exact test was used and for the comparison of continuous variables, Mann-Whitney and Kruskal-Wallis test was applied in both groups. P<0.05 was considered K. Balighi, et al.     Acta Medica Iranica, Vol. 56, No. 8 (2018)    523 statistically significant.  Results  We reviewed the files of 98 patients with BP in Tehran, Iran. Only 82 patients’ files entered the study due to incomplete files. The mean age of the patients was 67.13 years at the time of diagnosis (range between 25 and 97). The study group was composed of 32 (39.1%) males and 50 (60.9%) females. Thirty-nine (47.6%) were outpatient, and 43 (52.4%) were hospitalized. Mucosal involvement was positive in 33 (40.2%) of the patients, and oral mucosa was the most common location (Table 1).  Table 1. Frequency, percentage, and location of mucosal involvement in bullous pemphigoid patients The site of mucosal involvement Frequency of involvement Percentage of involvement (%) Oral mucosa 29 35.4 Genital mucosa 8 9.8 Nasal mucosa 2 2.4 Perianal mucosa 1 1.2 Total 33 40.2   The most common locations for bullous pemphigoid lesions were anterior of the thighs and ventral surface of the arms. Head and neck area were involved in 43 (52.4%) of the patients. Bullous lesions lead to scar formation and milia in 19 (23.2%) and 4 (4.9%) of patients, respectively. The comparison between bullous pemphigoid patients hospitalized or managed outpatient is demonstrated in table 2.  Table 2. The comparison between BP patients with inpatient or outpatient management  Outpatient  Hospitalized  Total P Age 63.9 70.7 67.1 0.04 Male 16 (37.2%) 16 (41%) 32 (39%) 0.82 Female 27 (62.8%) 23 (59.0%) 50 (61.0) No millia 42 (97.7%) 36 (92.3%) 78 (95.1%) 0.27 With milia formation 1 (2.3%) 3 (7.7%) 4 (4.9%) No scar 29 (67.4%) 34 (87.2%) 63 (76.8%) 0.03 Scar formation 14 (32.6%) 5 (12.8%) 19 (23.2%) No mucosal involvement  26 (60.5%) 23 (59.0%) 49 (59.8%) 0.31 With mucosal involvement 17 (39.5%) 16 (41.0%) 33 (40.2%) No involvement in head and neck 23 (53.5%) 16 (41.0%) 39 (47.6%) 0.18 With involvement in head and neck 20 (46.5%) 23 (59.0%) 43 (52.4%)   ELISA was performed in 49 of the patients. Anti BP-180 antibody was positive in 39 (79.6%), and anti-BP-230 was positive in 25 (51.0%) of the patients. The presence of these antibodies was not related to the anatomical location of the lesions (P=0.16) and mucosal involvement (P=0.44). Direct Immuno-Fluorescence (DIF) for IgG and C3 was positive in 78 (95.1%) of the patients. Positive DIF had no relation with the anatomical location of the lesions or mucosal involvement (P for IgG=0.27 and for C3 P=0.32)  Discussion  Bullous Pemphigoid is a common immunobullous disease with a high rate of mortality and morbidity. In order to understand different aspects of this disease, it is essential to investigate clinical presentation in different populations. This study represents clinicoepidemiological characteristics of bullous pemphigoid among Iranian population from Tehran, Iran. Although in most regions bullous pemphigoid is usually presented in seventh to ninth decade of life (4), the mean age at the time of diagnosis (67.13) in our patients was observed to be relatively lower than that reported in the literature (73.7–82.4±7.4 years) (5,6). Bullous pemphigoid 524    Acta Medica Iranica, Vol. 56, No. 8 (2018)    Similarly, in previous reports from Iran, Daneshpazhooh et al., (7) and Esmaeli et al., (8) reported a mean age of 59.4 and 65.0 years respectively. In a more recent report from Iran investigating immunobullous disease among elderly patients (over 60-year-old), the mean age of 77.6 years was reported (9). A study from south-western Iran also reported a mean age of 64.1 years (10). Younger age at the time of diagnosis in developing countries such as Iran may be due to the lower age of the elderly population in comparison to developed countries. As in our study, bullous pemphigoid has been reported to be more common in females worldwide, except for China (11), Germany (2), and Tunisia (12), where a predominance of the male has been observed. Previous reports from Iran similarly reported female predominance (7-9). In our patients, we observed a higher incidence of mucosal involvement (40.2%) than reports from other ethnicities (10-25%) (13-16). Nanda et al., reported 37% of mucosal involvement in moderate to severe Bullous Pemphigoid patients from Kuwait. One reason which has been proposed in this report was higher incidence of mucosal involvement in more severe cutaneous lesions (17). However, as demonstrated in table 2 the only significant difference between bullous pemphigoid patients with mild to moderate disease and patients with moderate to severe disease mandating hospitalization were higher age in hospitalized patients. Mucosal involvement was not related to disease severity in our patients. Previous reports from Iran demonstrated lower incidence of mucosal involvement in bullous pemphigoid (between 11.1 and 31.1%) (9,18).  Another significant finding in our series was higher incidence of head and neck involvement (52.4%). In Esmaili et al., series 25.4% of patients showed head and neck involvement (8). Bullous pemphigoid typically affects flexural aspects of the limbs and the abdomen (3). Higher incidence of head and neck involvement may be due to genetic and environmental factors. The important observations in our study were higher female predominance, higher incidence of mucosal involvement and higher incidence of head and neck involvement. These may provide evidence for a specific subtype of bullous pemphigoid in Asian populations. This may be due to environmental factors and genetic background of the patients. Further multi-centric studies are required to determine the difference between populations in relation to ethnic, genetic and environmental factors.  References  1. Langan S, Smeeth L, Hubbard R, Fleming K, Smith C, West J. Bullous pemphigoid and pemphigus vulgaris—incidence and mortality in the UK: population based cohort study. BMJ 2008;337:a180. 2. Junga M, Kippesb W, Messer G, Zillikens D, Rzany B. Increased risk of bullous pemphigoid in male and very old patients: a population-based study on incidence. Am Acad Dermatol 1999;41:266-8. 3. Schmidt E, Della Torre R, Borradori L. Clinical features and practical diagnosis of bullous pemphigoid. Dermatol Clin 2011;29:427-38. 4. Marazza G, Pham HC, Scharer L, Pedrazzetti PP, Hunziker T, Trueb RM, et al. Incidence of bullous pemphigoid and pemphigus in Switzerland: a 2-year prospective study. Br J Dermatol 2009;161:861-8. 5. Colbert RL, Allen DM, Eastwood D, Fairley JA. Mortality rate of bullous pemphigoid in a US medical center. J Invest Dermatol 2004;122:1091-5. 6. Korman NJ. Bullous pemphigoid. The latest in diagnosis, prognosis, and therapy. Arch Dermatol 1998;134:1137-41. 7. Daneshpazhooh M, Chams-Davatchi C, Payandemehr P, Nassiri S, Valikhani M, Safai-Naraghi Z. Spectrum of autoimmune bullous diseases in Iran: a 10-year review. Int J Dermatol 2012;51:35-41. 8. Esmaili N, Hallaji Z, Soori T, Chams Davatchi C. Bullous pemphigoid in Iranian patients: a descriptive study on 122 cases. Acta Med Iran 2012;50:335-8. 9. Ghiasi M, Daneshpazhooh M, Ismonov M, Chams-Davatchi C. Evaluation of Autoimmune Bullous Diseases in Elderly Patients in Iran: A 10-Year Retrospective Study. Skinmed 2017;15:175-80. 10. Jowkar F, Sadat Sadati M, Tavana S, Agah MA. Epidemiology of autoimmune bullous diseases and therapeutic modalities during a 10 year period in Iran. Acta Dermatovenerologica Croatica 2014;22:246-9. 11. Jin P, Shao C, Ye G. Chronic bullous dermatoses in China. Int J Dermatol 1993;32:89-92. 12. Zaraa I, Kerkeni N, Ishak F, Zribi H, El Euch D, Mokni M, et al. Spectrum of autoimmune blistering dermatoses in Tunisia: an 11‐year study and a review of the literature. Int J Dermatol 2011;50:939-44. 13. Schmidt E, Zillikens D. Pemphigoid diseases. Lancet 2013;381:320-32. 14. Chang Y, Liu H, Wong C. Bullous pemphigoid—a report of 86 cases from Taiwan. Clin Exp Dermatol 1996;21:20-2. 15. Bernard P, Vaillant L, Labeille B, Bedane C, Arbeille B, Denoeux JP, et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group. K. Balighi, et al.     Acta Medica Iranica, Vol. 56, No. 8 (2018)    525 Arch Dermatol 1995;131:48-52. 16. Rzany B, Partscht K, Jung M, Kippes W, Mecking D, Baima B, et al. Risk factors for lethal outcome in patients with bullous pemphigoid: low serum albumin level, high dosage of glucocorticosteroids, and old age. Arch Dermatol 2002;138:903-8.  17. Nanda A, Al-Saeid K, Al-Sabah H, Dvorak R, Alsaleh QA. Clinicoepidemiological features and course of 43 cases of bullous pemphigoid in Kuwait. Clin Exp Dermatol 2006;31:339-42. 18. Banihashemi M, Zabolinejad N, Vahabi S, Razavi HS. Survey of bullous pemphigoid disease in northern Iran. Int J Dermatol 2015;54:1246-9.     

Clinicoepidemiological Features of 82 Cases of Bullous Pemphigoid in Tehran, Iran

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Clinicoepidemiological Features of 82 Cases of Bullous Pemphigoid in Tehran, Iran

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