A family with leukonychia totalis

A family presented to our dermatology clinic with a complaint of white nails. Physical examination revealed clinical feature of leukonychia totalis and the presence of sensorineural hearing loss, palmo plantar keratoderma and knuckle pads (four essential criteria for the diagnosis of Bart Pumphrey syndrome).Three consecutive generations of this family were affected with variable presentations of Bart Pumphrey syndrome in male and female; and autosomal dominant pattern of inheritance

Quality of life in mild and severe alopecia areata patients

Background: Alopecia areata is a relapsing hair disorder characterized by a sudden hairloss and has a considerable impact on patient's quality of life. The goal of this study was to determine quality of life among patients with mild and severe forms of alopecia areata and compare the two groups. Methods: During one year, 176 patients (96 mild, 80 severe) were selected and asked to complete Dermatology Life Quality Index (DLQI) questionnaires. Results: Our study revealed that the severe group was predominantly female and had higher amount of unemployment, more prolonged disease duration, unstable disease course and facial involvement. The mean DLQI scores in the severe and mild groups were 10.7 ± 7.5 and 5.4 ± 6.8, respectively which was significantly different and severe group had higher DLQI scores and more quality of life impairment. As well, there was a significant association between total DLQI scores and acute stress during last 6 months. Conclusions: Our study confirms that alopecia areata considerably impacts quality of life and this is more pronounced in patients with severe disease and those who had acute stress recently.</p

A FAMILY WITH LEUKONYCHIA TOTALIS

A family presented to our dermatology clinic with a complaint of white nails. Physical examination revealed clinical feature of leukonychia totalis and the presence of sensorineural hearing loss, palmo plantar keratoderma and knuckle pads (four essential criteria for the diagnosis of Bart Pumphrey syndrome).Three consecutive generations of this family were affected with variable presentations of Bart Pumphrey syndrome in male and female; and autosomal dominant pattern of inheritance

Quality of life in mild and severe alopecia areata patients

Background: Alopecia areata is a relapsing hair disorder characterized by a sudden hairloss and has a considerable impact on patient’s quality of life. The goal of this study was to determine quality of life among patients with mild and severe forms of alopecia areata and compare the two groups. Methods: During one year, 176 patients (96 mild, 80 severe) were selected and asked to complete Dermatology Life Quality Index (DLQI) questionnaires. Results: Our study revealed that the severe group was predominantly female and had higher amount of unemployment, more prolonged disease duration, unstable disease course and facial involvement. The mean DLQI scores in the severe and mild groups were 10.7±7.5 and 5.4±6.8, respectively which was significantly different and severe group had higher DLQI scores and more quality of life impairment. As well, there was a significant association between total DLQI scores and acute stress during last 6 months. Conclusions: Our study confirms that alopecia areata considerably impacts quality of life and this is more pronounced in patients with severe disease and those who had acute stress recently

Total Serum IgE Concentration in Patients with Psoriasis: a Case-Control Study

Psoriasis is a chronic relapsing disorder that involves the skin, nails and joints. With regard to the role of the immune system in psoriasis, the current study compared serum IgE concentration in patients with psoriasis with control group. Current case-control study was conducted in Dermatology clinic of Razi hospital, Tehran University of medical sciences, Tehran, Iran in 2012. Fifty-eight patients with psoriasis e referred to the clinic were assigned as patient group and 58 healthy subjects with matched age and sex as a control group. Patient's history, family history and demographic characteristics such as age and sex, duration and severity of disease using PASI, were collected and entered into a form. Consent form was obtained from participants. Serum IgE concentrations of both study groups were measured by electrochemiluminescence assay in the laboratory A total number of 58 patients with psoriasis, mean age of 44.15 (19-76 years) and 58 controls with matched age and sex were studied. Mean average of serum IgE concentration in the control group was 115.13 versus 200/06 concentration in patients group (P=0.16). Serum IgE concentration in 22.4% of patients versus 17.2% in controls was greater than normal concentration (P=0.48). No significant correlation was between serum IgE concentration and disease severity using PASI (P=0.11, r=0.21), neither a significant correlation with disease duration, age and gender. According to the present study, serum IgE concentrations are not greater in patients with psoriasis. IgE concentration is also not associated with the severity of psoriasis based on the PASI score, therefore, the role of IgE in psoriasis can be considered insignificant as some previous studies indicate

Quality of life in mild and severe alopecia areata patients

Background: Alopecia areata is a relapsing hair disorder characterized by a sudden hairloss and has a considerable impact on patient's quality of life. The goal of this study was to determine quality of life among patients with mild and severe forms of alopecia areata and compare the two groups. Methods: During one year, 176 patients (96 mild, 80 severe) were selected and asked to complete Dermatology Life Quality Index (DLQI) questionnaires. Results: Our study revealed that the severe group was predominantly female and had higher amount of unemployment, more prolonged disease duration, unstable disease course and facial involvement. The mean DLQI scores in the severe and mild groups were 10.7 ± 7.5 and 5.4 ± 6.8, respectively which was significantly different and severe group had higher DLQI scores and more quality of life impairment. As well, there was a significant association between total DLQI scores and acute stress during last 6 months. Conclusions: Our study confirms that alopecia areata considerably impacts quality of life and this is more pronounced in patients with severe disease and those who had acute stress recently.</p

Prolactin level changes in pemphigus vulgaris:A cohort study

Background: Prolactin (PRL) appears to play a role in the pathogenesis of autoimmune diseases. Limited evidence showed an association between serum PRL levels and the activity of pemphigus vulgaris (PV). This study intends to determine PRL level changes in pemphigus patients during therapy and its correlation with disease type and severity. Methods: In this cohort study, we measured serum PRL levels by enzyme-linked immunosorbent assay (ELISA) in newly diagnosed PV patients at three time points: before therapy initiation and after two and four months. Concomitantly, we estimated disease severity by the Pemphigus Disease Area Index (PDAI). Results: We examined 42 new cases of PV. Among 32 cases who completed the study, mean serum PRL levels at the three time points were 15.9±14.1 ng/mL (before treatment), 16.7±9.8 ng/ mL (2 months after initiation of treatment), and 15.2±9.2 ng/mL (4 months after initiation of treatment). Mean PDAI values were 19.3±12.8 (before treatment), 3.7±6.2 (2 months after initiation of treatment), and 0.6±1.5 (4 months after initiation of treatment). Although the disease activity decreased significantly (P&lt;0.001), there was no significant change observed in PRL level (P=0.760). We observed no significant correlations between PRL levels and PDAI for before (r= - 0.25; P=0.180), 2 months after initiation of treatment (r= 0.02; P=0.920), and 2 months after initiation of treatment (r=0.04; P=0.800). Conclusion: The results suggest that no significant association exists between serum PRL concentrations and the severity of PV.</p

Prolactin level changes in pemphigus vulgaris:A cohort study

Background: Prolactin (PRL) appears to play a role in the pathogenesis of autoimmune diseases. Limited evidence showed an association between serum PRL levels and the activity of pemphigus vulgaris (PV). This study intends to determine PRL level changes in pemphigus patients during therapy and its correlation with disease type and severity. Methods: In this cohort study, we measured serum PRL levels by enzyme-linked immunosorbent assay (ELISA) in newly diagnosed PV patients at three time points: before therapy initiation and after two and four months. Concomitantly, we estimated disease severity by the Pemphigus Disease Area Index (PDAI). Results: We examined 42 new cases of PV. Among 32 cases who completed the study, mean serum PRL levels at the three time points were 15.9±14.1 ng/mL (before treatment), 16.7±9.8 ng/ mL (2 months after initiation of treatment), and 15.2±9.2 ng/mL (4 months after initiation of treatment). Mean PDAI values were 19.3±12.8 (before treatment), 3.7±6.2 (2 months after initiation of treatment), and 0.6±1.5 (4 months after initiation of treatment). Although the disease activity decreased significantly (P&lt;0.001), there was no significant change observed in PRL level (P=0.760). We observed no significant correlations between PRL levels and PDAI for before (r= - 0.25; P=0.180), 2 months after initiation of treatment (r= 0.02; P=0.920), and 2 months after initiation of treatment (r=0.04; P=0.800). Conclusion: The results suggest that no significant association exists between serum PRL concentrations and the severity of PV.</p