Family impact of pemphigus disease in an Iranian population using the Family Dermatology Life Quality Index.

BACKGROUND: Pemphigus vulgaris (PV) is a rare but seriously disabling disorder of the skin and mucous membranes that can gravely impact the quality of life (QoL) of patients. OBJECTIVE: The aim of the present study was to identify how family members of patients with PV are affected by the disease. METHODS: A total of 118 patients with confirmed PV and one of their family members (caregivers) were enrolled in the study. To calculate disease severity, the Autoimmune Bullous Skin Disorder Intensity Score was used. The Persian version of the Dermatology Life Quality Index (DLQI) questionnaire was used to evaluate the QoL of patients and the Family Dermatology Life Quality Index (FDLQI) to evaluate the QoL of caregivers. RESULTS: The mean age of patients was 43.14 ±â€¯12.5 years. Ninety patients (76.3%) were female. Eighty-one patients (68.6%) had the mucocutaneous phenotype and 37 cases (31.4%) the mucosal phenotype. The DLQI score was 10.1 ±â€¯7.1 for patients. The DLQI score was higher for patients with the mucocutaneous phenotype (11.8 ±â€¯7.5) than those with the mucosal phenotype (6.4 ±â€¯4.9; p < .001). QoL was significantly affected by disease severity. FDLQI score was 13 ±â€¯7 for caregivers, and was significantly higher in older caregivers and married ones. There was a positive correlation between patients' admission frequencies and FDLQI score. FDLQI score was also significantly affected by the Autoimmune Bullous Skin Disorder Intensity Score of patients' disease severity. The QoL of patients and their caregivers showed a significant positive correlation. CONCLUSION: The QoL of patients and their families are impaired significantly, and is considerably prominent in the mucocutaneous phenotype of PV and more severe forms. LIMITATION: Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Disease QoL (ABQoL) were not used in this study.

Dermatoscopic and Mucoscopic Features of Lesions in Patients with Behcet's Disease.

OBJECTIVE: Behcet's disease (BD), as a vasculitis, can affect small and large vessels. As dermatoscopy has been shown to improve the accuracy in diagnosis of various skin lesions especially vascular patterns, we set this study to find if there is any characteristic pattern in the dermatoscopy of Behcet's mucocutaneous lesions. METHODS: This prospective cross-sectional observational study designed to evaluate dermatoscopic features of Behcet's mucocutaneous lesions. Fifty six consecutive patients presenting at the outpatient clinic of the BD Research Unit were included. If present, for each patient one oral, one skin and one genital lesion were evaluated by dermatoscopy. When indicated, pathergy test was performed according to a standard protocol and the results were evaluated by dermatoscopy. RESULTS: A total of 40 oral, 8 genital, 14 skin lesions and 14 pathergy tests were evaluated by dermatoscopy. While vascular component was the most prominent feature in oral aphthae, this component was less prominent in genital lesions. Dot vessels were the most common form of vessels in both oral and skin lesions. All the oral lesions were characterized by a central white structureless area. Skin lesions were characterized by a red structureless background. In pathergy tests, negative pricks showed absence of specific features while positive pricks were characterized by a structureless background in pink, purple or red. No obvious vascular component was detected in any of the pricks. CONCLUSION: It seems that these findings have no specific clues for the diagnosis of BD, but our study is the first study in this field and the findings may give way to further investigations.

Paediatric Behçet's disease in Iran: report of 204 cases.

OBJECTIVES: This study proposed to report the characteristics of paediatric Behçet's disease (PED-BD) in a cohort of patients from Iran's registry and compare them with different reports throughout the world. METHODS: From a cohort of 7504 Iranian patients with Behçet's disease those diagnosed before the age of 16 years were included in this study. Data were collected on a standard protocol comprising 105 items, including demographic features, type of presentation, and different clinical and laboratory findings. RESULTS: PED-BD was seen in 2.7% of patients. The male/female ratio was 1.02/1, and the mean age at onset was 10.5±3.4. Positive familial history was present in 9.9%. As a first manifestation, oral aphthosis was the most frequent (75%) followed by ocular lesions in 19.1%. The prevalence rates of various manifestations were as follows: oral aphthosis: 91.7%; genital ulcer: 42.2%; skin: 51.5% (pseudofolliculitis: 43.1%, erythema nodosum: 10.3%); ocular lesions: 66.2% (anterior uveitis 52%, posterior uveitis 58.3%, retinal vasculitis 39.7%); articular manifestations: 30.9%; neurological involvement: 4.9%; vascular involvement: 6.4% (venous 4.9%, arterial 2.5%); gastrointestinal manifestations: 5.9%; epididymo-orchitis: 8.7% (boys); high ESR (≥20): 50.8%; abnormal urine: 14.1%; positive pathergy test: 57%; HLA-B5/51: 48.7%. ICBD criteria have the highest sensitivity for the classification of PED-BD patients in Iran (91.7%). CONCLUSIONS: The clinical spectrum of PED-BD in Iran in this study was similar to that of other reports; however, genital ulcers, skin lesions (notably erythema nodosum), and gastrointestinal involvement were noticed to occur less frequently, while ocular lesions were more frequent and more severe compared to other reports.

Osteoporosis in patients with Pemphigus Vulgaris before steroid therapy.

Pemphigus Vulgaris (PV) is often complicated by osteoporosis. Although corticosteroid therapy undoubtedly plays a causative role, inflammation associated with PV may also contribute to osteoporosis. This study was designed to determine the prevalence of osteoporosis in patients with PV before corticosteroid therapy and to compare these findings with those reported previously in healthy volunteers. Newly diagnosed patients with PV, who had not received systemic corticosteroids, were enrolled.  Bone mineral density (BMD) was measured both in the lumbar spine (L1-L4) and hip region. Data were compared with those of a healthy Iranian population. The association between the disease duration and severity and BMD was evaluated. A total of 50 patients (27 women) with a mean age of 42.6±14.5 years were enrolled. Osteoporosis was seen in 7 (14%) patients, 3 (11.1%) women, 4 (17.4%) men, and in both genders it was more common when compared to the population of healthy Iranians (8.2% in women and 4.9% in men). Osteopenia was found in 26 (52%) patients, 13 women and 13 men. Although both osteopenia and osteoporosis were more common in severe disease, neither the duration nor the severity of PV showed a statistically significant association with osteopenia or osteoporosis. The presence of a higher than expected rate of osteoporosis in patients with PV argues for osteoporosis screening and efforts aimed at prevention and early initiation of treatment to prevent unnecessary morbidity.

Iron deficiency state in resistant oral aphthosis of Behcet's disease.

AIM: This study was designed to evaluate iron deficiency as a predisposing factor for resistant oral aphthosis in patients with Behcet's disease (BD). METHODS: In a case control study 220 consecutive BD patients with oral aphthosis were enrolled. All patients had been treated for at least 3 months. They were divided into two groups according to their treatment response (75 patients in the Case and 145 in the Control group). Demographic and clinical characteristics of the disease, serum iron, total iron binding capacity and serum ferritin were determined in each patient. We used independent t-test and Mann-Whitney U-test to compare the quantitative variables and chi-square test for qualitative variables. Odds ratio (OR) and confidence interval at 95% (95% CI) were calculated for each item. RESULTS: There was no significant difference between the two groups in demographics or clinical characteristics of the disease. We found iron deficiency in 72 patients (32.7%, 95% CI: 6.2), higher in the Case group than Control (39.2% vs. 30.1%; P = 0.17). Despite the higher frequency of iron deficiency in men (26.8% vs. 14.5%), the difference was not statistically significant (P = 0.09). Multivariate logistic regression analysis showed that none of the iron deficiency or sex variables could predict the development of resistant oral aphthosis. The OR for iron deficiency was 1.52 (95% CI: 0.81-2.86) and for male sex was 1.04 (95% CI: 0.56-1.91). CONCLUSION: Despite the higher frequency of iron deficiency in BD patients with resistant oral aphthosis, we were not able to attribute this resistance to this deficiency.

Genital aphthosis in Behçet's disease: is it associated with less eye involvement?

Behçet's disease (BD) is a multisystem disease classified among the vasculitides with various clinical features. Genital aphthosis (GA) is one of the major manifestations of BD. The aim of this study was to evaluate the characteristics of BD patients with GA. A cross-sectional sample of BD patients registered in 37 years was selected. We determined clinical and laboratory features of BD patients with GA (GA cases) and compared them with the patients who never developed GA (non-GA cases). The comparisons were performed by the chi-square test and logistic regression analysis. Odds ratios (ORs) with 95 % confidence intervals were calculated to estimate the precision of ORs. Among 6,935 BD patients, 4,489 cases (64.7 %) were ascribed to GA cases. Male to female ratio (1.11:1.00 vs. 1.48:1.00 OR 0.753, P value <0.001) and mean age of disease onset (OR = 0.9, P value <0.001) were lower in GA subset. In GA cases, oral aphthosis (OA) was a more common onset manifestation (OR 2.250, P value <0.001), while uveitis (OR 0.140, P value <0.001) and retinal vasculitis (OR 0.077, P value <0.001) were less common at the disease onset. In the whole course of disease, eye involvement was less common in GA cases (OR 0.215, P value <0.001). On the contrary, OA (OR 19.698, P value <0.001), skin (OR 1.762, P value <0.001), joint (OR 1.257, P value = 0.001), gastrointestinal (OR 1.302, P value = 0.009), neurological (OR 1.624, P value <0.001) and vascular involvements (OR 1.362, P value <0.001), epididymitis (OR 1.596, P value <0.001), positive pathergy test (OR 1.209, P value <0.001) and positive familial history of OA (OR 1.325, P value <0.001) were more common in GA subset. This study showed that GA subset of BD is associated with less eye involvement but higher rates of other BD manifestations.

Quality of life and psychological status of patients with pemphigus vulgaris using Dermatology Life Quality Index and General Health Questionnaires.

Pemphigus vulgaris with painful chronic blisters and/or erosions on skin and mucosa can impair quality of life (QOL). Therapeutic modalities in the long run can have additional negative impact. There are few studies that have focused on QOL of such patients except in treated cases. The aim of this study was to describe the effect of the disease per se on QOL before receiving treatment and evaluation of psychological status of the patients and its effect on their QOL. A total of 61 patients with newly diagnosed non-treated pemphigus vulgaris participated in the study. The Persian version of the Dermatology Life Quality Index (DLQI) questionnaire was used to evaluate their QOL and the 28-item General Health Questionnaire (GHQ-28) for their psychological status. In this study, the mean DLQI score was 10.9 ± 6.9. QOL was worse in patients with nasal and pharynx involvement, with positive Nikolsky sign, patients with severe skin involvement and those who showed the symptom of itching. There was a negative correlation between DLQI score and duration of the disease. More than 77% of patients experienced anxiety and depression with more impaired QOL. In conclusion, pemphigus vulgaris is responsible for great alteration in QOL, especially in its severe form. The disease in its initial stage may have greater impact on the QOL. The high probability of anxiety and depression in these patients and its negative effect on QOL should be taken into account in the management of these patients right from the start of the treatment.

Pemphigus and pregnancy: a 23-year experience.

BACKGROUND: Studies on the interaction of pemphigus and pregnancy are limited to case reports and small case series. Pregnancy is not rare in Iranian pemphigus patients due to lower age at onset of the disease. AIM: We sought to investigate the outcome of pregnancy and the course of pemphigus in pemphigus patients in a retrospective study. METHODS: The files of 779 pemphigus women younger than 50 presenting to our center from 1984 till 2006 were examined for any mention of pregnancy. Data related to outcome of pregnancy and the course of the disease were collected. RESULTS: Sixty-six patients with a history of pregnancy were identified. Forty-eight known pemphigus patients experienced one or more pregnancies during their disease (total pregnancy number: 52). The course of pemphigus was as follows in this group: 28 cases (54%) of exacerbation, 15 cases (31%) with no alteration, and 9 cases (17%) showing improvement. The rate of abortion was 9.6% (5 cases). In 18 cases, the disease had started during pregnancy, 2 of them (11%) ended in an abortion. Overall, postpartum flare was seen in 33 cases (47.1%). CONCLUSION: Pemphigus may be exacerbated during or after pregnancy, but often to a mild degree. Although the rate of stillbirth was not as high as previously reported, the rate of abortion was considerable. Pregnancy may have an uneventful course, especially in patients in clinical remission; nevertheless, careful monitoring of the high risk mother and fetus is mandatory.

Prevalence, severity, and severity risk factors of acne in high school pupils: a community-based study.

A cross-sectional, community-based study was performed to determine the prevalence and severity of acne vulgaris in adolescents and of factors influencing the acne severity risk. The presence of acne was clinically determined and the secondary outcome measures of family acne history and the relation of acne to nutrition habits, emotional stress, menstruation, and smoking were recorded in a questionnaire. A representative sample of 1,002 pupils aged 16+/-0.9 years was enrolled. The overall acne prevalence was 93.3, 94.4% for boys and 92.0% for girls. Moderate to severe acne was observed in 14%. The prevalence of moderate to severe acne was 19.9% in pupils with and 9.8% in those without a family history of acne (P<0.0005; OR: 2.3). Acne severity risk increased with the number of family members with acne history. A mother with acne history influenced the severity of acne the most. Increasing pubertal age, seborrhea, the premenstrual phase, mental stress, and sweet and oily foods were recognized as risk factors for moderate to severe acne. In contrast, gender, spicy foods, and smoking were not associated with acne severity. In conclusion, acne is a common disorder in Iranian adolescents, with a low rate of moderate to severe acne. A genetic background is suggested, with mother's acne history being the most important prognostic factor. Skin quality and certain nutrition habits may affect acne severity.

Nail changes in pemphigus vulgaris.

BACKGROUND: The frequency and types of nail changes in pemphigus vulgaris (PV) are unclear. Aim To determine the frequency and types of nail changes, and their correlation with the number of skin and periungual bullae. METHODS: Seventy-nine patients with PV, including 59 new patients and 20 patients in relapse, were entered into the study. Microscopic examination in potassium hydroxide and culture for fungus were performed on all clinically abnormal nails. RESULTS: Twenty-five (31.6%) of 79 patients showed nail changes, with paronychia (n = 8) and onychomadesis (n = 6) being the most common. One patient in relapse had onychomycosis. The frequency of nail changes in fingers affected by periungual bullae was significantly higher than in other fingers (P < 0.05). The number of nail changes was higher in patients with a larger number of skin bullae and in those with a longer duration of disease (P < 0.05). CONCLUSIONS: Nail changes in PV are common and related to the number of skin bullae and the presence of periungual bullae.

Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris.

BACKGROUND: Pemphigus is a severe autoimmune blistering disease affecting the skin and mucosa. Mortality is high in the absence of treatment. Nowadays, treatment is based mainly on corticosteroids and cytotoxic drugs; however, because of the rarity of the disease worldwide, there is not yet a standard treatment based on randomized controlled trials, and the treatment used is based mainly on the experience of experts. OBJECTIVE: The aim of this study was to compare the efficacy and safety of 4 treatment regimens for pemphigus vulgaris: prednisolone alone, prednisolone plus azathioprine, prednisolone plus mycophenolate mofetil, and prednisolone plus intravenous cyclophosphamide pulse therapy. METHODS: One hundred twenty new cases of pemphigus vulgaris were enrolled. These patients were randomly allocated into 1 of 4 treatment groups (each comprising 30 patients) and received prednisolone (P), prednisolone and azathioprine (P/A), prednisolone and mycophenolate mofetil (P/MM), and prednisolone and intravenous cyclophosphamide pulse therapy (P/PC). They were followed up for 1 year at the Pemphigus Research Unit. RESULTS: In groups P, P/A, P/MM, and P/PC, 23 (76.5%), 24 (80%), 21 (70%), and 22 (73.3%) of the patients, respectively, followed the regimen for the full 1-year period. The mean total dose of prednisolone administered in groups P, P/A, P/MM, and P/PC was 11631 mg (standard deviation [SD] = 7742), 7712 mg (SD = 955), 9798 mg (SD = 3995), and 8276 mg (SD = 810), respectively. The mean total dose of prednisolone in group P (prednisolone alone) was 11,631 mg, The mean total dose of prednisolone in the 3 cytotoxic groups was 8652 mg. By using analysis of variance, the difference was statistically significant (P = .047). In the cytotoxic groups, there was a significant difference between the P/A and P/MM groups (P = .007), but not between P/A and P/PC (P = .971), and P/MM and P/PC (P = .670). Side effects were not significantly different among the 4 groups. LIMITATIONS: Larger sample sizes and blind design are suggested for future studies. CONCLUSION: The efficacy of prednisolone is enhanced when it is combined with a cytotoxic drug. The most efficacious cytotoxic drug to reduce steroid was found to be azathioprine, followed by cyclophosphamide (pulse therapy), and mycophenolate mofetil.

Lichen planus and Hepatitis C: a case-control study.

BACKGROUND: The association of lichen planus with hepatitis C (HCV) has been widely reported in the literature. However, there are wide geographical variations in the reported prevalence of HCV infection in patients with lichen planus. This study was conducted to determine the frequency of hepatitis C in Iranian patients with lichen planus at Razi hospital, Tehran. METHODS: During the years 1997 and 1998, 146 cases of lichen planus, 78 (53.1%) women and 69 (46.9%) men were diagnosed. They were diagnosed on the basis of the usual clinical features and, if necessary, typical histological findings. The patients were screened for the presence of anti-HCV antibodies by third generation ELISA and liver function tests. We used the results from screening of blood donors for anti HCV (carried out by Iranian Blood Transfusion Organization) for comparison as the control group. RESULTS: Anti-HCV antibodies were detected in seven cases (4.8%). This was significantly higher than that of the blood donors' antibodies (p < 0.001). The odds ratio was 50.37(21.45-112.24). A statistically significant association was demonstrated between erosive lichen planus and HCV infection. Liver function tests were not significantly different between HCV infected and non-infected patients. CONCLUSION: HCV appears to have an etiologic role for lichen planus in Iranian patients. On the other hand, liver function tests are not good screening means for HCV infection.