Is the severity of alopecia areata associated with arterial stiffness?

Background: This study aimed to evaluate the severity of alopecia areata (AA) associations with metabolic syndrome, body composition evaluated by bioimpedance techniques, and arterial stiffness based on pulse-wave velocity analysis. Materials and Methods: This cross-sectional study was conducted on patients referred to AA Clinic at Razi Hospital in 2021 and 2022. Patients with AA with the Severity of Alopecia Tool (SALT) score above 20% and receiving no systemic therapy were included. Patient demographic and clinical information, symptoms of metabolic syndrome, and bioimpedance factors were collected, and the relationship between disease severity, metabolic syndrome, and bioimpedance indicators was evaluated. Results: In this study, 59 patients were examined, with 26 (44.07%) being female and 33 (55.93%) being male. The mean age of the patients was 37.42 years (standard deviation [SD] =11.28). The severity of the disease was assessed using the SALT score, with the mean severity in terms of the percentage being 69.83% (SD = 28.57%). In the regression model, SALT score was independently related to the severity of vascular stiffness after adjusting for the effect of other variables (beta = 0.033, 95% CI = 0.009-0.057, P = 0.046). Moreover, SALT score was significantly related to metabolic syndrome after adjusting for the effect of other variables (OR = 1.035, 95% CI = 1.012-1.059, P = 0.002). Conclusion: This study found that AA severity is associated with a higher chance of having metabolic syndrome and arterial stiffness which may lead to cardiovascular diseases in patients with AA, and screening patients regarding cardiometabolic diseases is mandated.

Topical treatment adherence and associated factors in patients with psoriasis: A single center, cross-sectional study.

Topical medications are one of the main treatments for psoriasis. Adherence to treatment is an important factor in achieving successful control of disease. The aim of this study was to evaluate adherence to topical drugs and identify possible associated factors in in patients with psoriasis. Ninety-three patients were evaluated by a two-part questionnaire; the first part examined demographic, socioeconomic and disease-related factors while the second part, the ECOB questionnaire (Elaboration d'un outil d'evaluation de l'observance des traitements medicamenteux), examined adherence to topical drugs. The mean self-reported adherence to topical treatment in this study, was 50.5%. Severe disease, involvement of lower extremity, and positive family history of psoriasis were associated with poor adherence to treatment. Patients with a history of diabetes mellitus had better adherence to treatment. In this study adherence to topical treatment was low and had no significant relationship with most of the studied demographic and socioeconomic factors.

Illness perception in patients with Alopecia areata under topical immunotherapy.

Alopecia areata (AA) is a chronic autoimmune-mediated disorder. There is little research on how AA patients conceptualize their disease. The purpose of this study was to investigate the perception and attitude of patients with AA about their illness. The cross-sectional study was performed on 102 patients with AA referred to our alopecia clinic. Patients between 16 and 60 years were enrolled in the study. A questionnaire including demographic and clinical characteristics and Illness Perception Questionnaire-Revised (IPQ-R) was administered to each patient. More than half of patients (55%) experienced their illness as a long-lasting (17.3 ± 5.5, median reference score = 18), and timely variable (13.4 ± 2.8, median reference score = 12) disorder. Patients perceived that their illness negatively affects their lives (18.9 ± 4.8, median reference score = 18) and are considered an effective role for themselves in controlling disease (20.3 ± 4.9, median reference score = 18). Moreover, patients had a fairly good accepting of their illness (13.4 ± 2.8, median reference score = 15). We observed significant negative effects of illness on the patient's emotions (21.5 ± 5.5, median reference score = 18). We also found that men had a stronger belief in personal control compared with women (21.5 ± 4.8 vs 19.5 ± 4.8; P = .03). A positive correlation was observed between educational status and illness coherence (r = .21; P = .03). Most patients with AA considered undesirable consequences of their illness. High scores of negative affective symptoms indicate the harmful effects of this disease on patients' lives leading to problems of mental health.

Is the severity of initial sensitization to diphenylcyclopropenone in alopecia areata patients predictive of the final clinical response?

Immunotherapy by diphenylcyclopropenone (DPCP) is generally started with 2% DPCP sensitization, however in recent years studies have questioned the necessity of sensitization that may cause patients severe reactions and troubles at the onset of therapy. The purpose of the present study was to evaluate the association between the severity of initial reaction to 2% DPCP sensitization in AA patients and clinical response. In this retrospective study, 110 AA patients who continued therapy for at least 6 months were enrolled. Hair loss and hair regrowth rates were calculated based on the Severity of Alopecia Tool (SALT) scoring system. Initial reaction to 2% DPCP sensitization after 2 weeks was graded as negative reaction (absence of any reaction), doubtful reaction (mild erythema, pruritus, and irritation for minutes after test), weak (erythema, mild edema, and scaling), and strong to extreme reaction (vesicles, bullae, ulcer, and discharge). The degrees of the initial reaction to 2% DPCP after 2 weeks were negative reaction 13 (11.81%), doubtful reaction 40 (36.36%), weak reaction 33 (30%), and strong to extreme reaction 24 (21.81%). Patients were divided into two groups: (A) patients with less than 12-month therapy (75 of 110), (B) patients with more than 12-month therapy (35 of 110). Initial reaction to 2% DPCP sensitization was not correlated with hair regrowth rate in either group (group A: Spearman's rho = 0.194, p = 0.095; group B: Spearman's rho = 0.063 p = 0.720). After 12-month treatment with DPCP, hair regrowth rate was significantly greater than 6-months therapy (group A: 17.03 ± 37.78, group B: 49.26 ± 36.34; p = 0.003). The severity of hair loss at the onset of treatment was significantly associated with the response rate in both groups (p-value <0.002). Based on our results, it is the initial severity of the disease and not the initial reaction to 2% DPCP sensitization that predicts the clinical response to DPCP immunotherapy.

Intralesional injection of biosimilar rituximab in recalcitrant mucocutaneous lesions of patients with pemphigus vulgaris: A pilot study.

The efficacy of intravenous rituximab (RTX) in patients with pemphigus have been shown in the previous studies. The study aimed to investigate the effectiveness of intralesional injection of RTX in the healing of pemphigus lesions, with lower doses and probable better safety profile than intravenous RTX. Eleven Pemphigus patients with recalcitrant lesions received two intralesional injections of biosimilar RTX, 5 mg/cm2 . During 6 months follow-up, Pemphigus Disease Area Index, the patients' satisfaction, quality of life, the disease activity, the number and size of lesions, the anti-desmoglein (Dsg) 1 and 3 antibodies and the count of CD4+ and CD19+ cells were assessed. All patients were in partial remission on therapy. The absolute count of CD19+ B cells showed a statistically significant decline (P = .006). The percentage of CD4 + T lymphocytes increased 2 weeks after injection and decreased 2 weeks later (P = .01). The average number and size of lesions decreased. The concentration of anti-Dsg 3 antibody decreased insignificantly during the study. The severe pain during the injection was considered as the main complication. At the end of the study, two patients were in complete remission on therapy, and the other nine remained in partial remission on therapy. Few side effects resulting from intralesional injection of RTX and enhanced quality of life of the patients were considered as the valuable achievements of this study. The results showed that although a low dose of RTX leads to a significant decrease of CD19+ B lymphocytes, it did not show parallel clinical effectiveness.

Estimated cut-off values for pemphigus severity classification according to pemphigus disease area index (PDAI), autoimmune bullous skin disorder intensity score (ABSIS), and anti-desmoglein 1 autoantibodies.

BACKGROUND: Pemphigus is a potentially fatal disease if left untreated. Valid scoring systems and defined cut-off values for classification of patients would help with better management through specified pharmaceutical and non-pharmaceutical treatments. METHODS: In this study, pemphigus patients who were receiving immunosuppressive treatments and had recent disease relapse were recruited for examination of pemphigus disease area index(PDAI), autoimmune bullous skin disorder intensity score (ABSIS), physician global assessment (PGA), autoimmune bullous disease quality of life (ABQoL), anti-desmoglein 1 (anti-Dsg1), and anti-Dsg3 autoantibody titers from December-2017 to February-2018. Cut-off values were estimated using model-based clustering classification and the 25th and 75th percentiles approach, performed separately for the exclusive cutaneous, exclusive mucosal, and mucocutaneous groups. RESULTS: In the 109 included patients, the 25th and 75th percentiles cut-offs were 6.2 and 27 for PDAI score, and 4 and 29.5 for ABSIS score. The model-based analysis resulted in two groups (cut-point:15) for PDAI score, and three groups (cut-points:6.4 and 31.5) for ABSIS score. The groups were significantly different for the PDAI, ABSIS, PGA, and ABQoL values. Based on anti-Dsg1 autoantibody values, the model-based analysis cut-point was 128 and the 25th and 75th percentiles cut-offs were 98 and 182. Anti-Dsg3 autoantibody values did not differentiate between pemphigus severity classes. CONCLUSIONS: Estimated cut-off values based on the anti-Dsg1 level, PDAI, and ABSIS scoring systems could be used to classify patients into different severity grades for better management and prognosis.

The effect of l-carnitine supplementation on serum levels of omentin-1, visfatin and SFRP5 and glycemic indices in patients with pemphigus vulgaris: A randomized, double-blind, placebo-controlled clinical trial.

Pemphigus vulgaris (PV) is a chronic autoimmune disorder with potentially fatal outcomes. The aim of this study was to investigate the effect of l-carnitine (LC) on secreted frizzled-related protein-5 (SFRP5), omentin, visfatin, and glycemic indices in PV patients under corticosteroid treatment. In this randomized, double-blind, placebo-controlled clinical trial, 52 patients with PV were divided randomly into two groups to receive 2 g of LC or a placebo for 8 weeks. Serum levels of SFRP5, omentin, visfatin, and also glycemic indices were evaluated at the baseline and end of the study. LC supplementation significantly decreased the serum level of visfatin (95% CI [-14.718, -0.877], p = .05) and increased the serum levels of SFRP5 (95%CI [1.637, 11.380], p < .006) and omentin (95% CI [9.014, 65.286], p < .01). However, LC supplementation had no significant effects on the serum levels of glycemic factors such as insulin (95% CI [-1.125, 3.056], p = .426), fasting blood sugar (95% CI [-4.743, 3.642], p = .894), homeostatic model assessment of insulin resistance (95% CI [-0.305, 0.528], p = .729), and quantitative insulin-sensitivity check index (95% CI [-0.016, -0.010], p = .81). LC supplementation decreased visfatin serum level and increased omentin-1 and SFRP5 serum levels in patients with PV. However, it has no significant effect on the serum levels of insulin and glycemic indices.

Iranian guideline for rituximab therapy in pemphigus patients.

Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and/or mucosa. Rituximab (RTX) has been approved recently by US FDA as an effective and safe treatment of PV. The high incidence of PV in Iran encouraged our team to prepare a consensus guideline for RTX administration based on literature review and a decade experience of an expert panel. RTX is recommended for the treatment of new cases of PV as well as patients not responding to conventional therapy. Contraindications include history of anaphylaxis or IgE-mediated hypersensitivity to murine proteins of RTX, severe active infections, pregnancy, breastfeeding, severe heart failure, and arrhythmia. Prophylactic antiviral therapy is recommended in patients at risk of reactivation of HBV and isoniazid for those at risk of reactivation of tuberculosis. Concomitant use of systemic corticosteroids is recommended as a rule. Except for methotrexate, the combination with other immunosuppressive drugs is discouraged. Intravenous immunoglobulin is recommended for those at risk of infections or with extensive disease. The recommended dosage of RTX for the first cycle is 2 g either 500 mg weekly or 1 g biweekly. There is no general consensus whether the next doses of RTX be administered upon relapse or as maintenance therapy. We strongly recommend RTX sooner in the course of pemphigus.

The association between ST18 gene polymorphism and severe pemphigus disease among Iranian population.

Recently, ST18 polymorphism has played a role in increasing the risk of pemphigus among some populations such as Egyptian and Jewish. In addition, a variant within the ST18 promoter gene was shown to induce ST18 upregulation and cytokine secretion leading to keratinocyte susceptibility to anti-desmoglein antibodies. Thus, the present study aimed to assess the ST18 single nucleotide polymorphisms (SNP) relationship with pemphigus, disease severity and family history among Iranian population. A total of 111 pemphigus patients and 201 healthy controls were genotyped for three ST18 SNPs rs2304365, rs10504140 and rs4074067 by using TETRA-ARMS PCR method. The results indicated that risk allele A in rs2304365 was significantly higher in pemphigus patients, compared with the amount in the control group (OR = 2.43 CI = 1.49-3.975, P < 0.001). Thus, A allele represents a risk factor for pemphigus. Further, the patients carrying the risk allele had a more severe disease and a higher age of disease onset while no relationship was observed between the number of relapses and positive family history of pemphigus with the risk allele. Finally, dominant model was regarded as the strongest inheritance model for the associated risk. The present study confirmed the relationship between ST18 gene with pemphigus disease, a more severe disease, and a higher age of disease onset.

Comparison of clinical efficacy and safety of thermotherapy versus cryotherapy in treatment of skin warts: A randomized controlled trial.

The effect of thermotherapy in the treatment of skin warts in comparison to cryotherapy, as the standard conventional method, has remained uncertain. This study aimed to assess the clinical efficacy and safety of thermotherapy and cryotherapy in removing skin warts. This randomized controlled trial was conducted on 52 patients aged 18 years and over with ≤ 10 skin warts. The participants were randomly assigned into two groups to receive cryotherapy (every 2 to 3 weeks up to six sessions if required) or thermotherapy (one session). The patients in both groups were followed every 2 to 3 weeks for the first three months, and then three months after the last treatment session. The clearance rate was 79.2% in the thermotherapy group and 58.3% in the cryotherapy group with no significant difference (p = 0.212). The rate of scarring in the thermotherapy group was 20% (p = .018). A higher clearance rate was achieved in the thermotherapy group. However, this result was not statistically significant. There were some minimal post-treatment complications. Patients needed only one session of thermotherapy. Due to the risk of scarring, we suggest thermotherapy only as a suitable treatment method for palmoplantar warts.

Characteristic features of cutaneous melanoma in a dermatology referral centre in Tehran, Iran.

BACKGROUND/OBJECTIVES: The characteristics of cutaneous melanoma in the Middle-Eastern countries is poorly described. Therefore we conducted this study to determine the characteristics of melanoma in Iran. METHODS: A retrospective, cross sectional study of melanoma patients seen at a tertiary referral centre, Iran, from May 2004 to October 2014. Clinical data included age and gender of the patients at the time of diagnosis, tumour location and tumour size. Histological characteristics included Breslow thickness, Clark level and subtype of tumour. RESULTS: A total of 450 cases of melanoma with a male/female ratio of 1.1:1 were reviewed. The mean age of patients was 57.5 years. The most frequent histological subtypes were acral lentiginous melanoma (30%) and lentigo maligna melanoma (29%). In 215 cases (49%) the tumour was located on the extremities. The second most common site was the face. Tumour invasion was mainly at Clark level III and IV. The mean Breslow thickness was 2.8 mm; 143 (38%) melanomas had a Breslow thickness less than 1 mm (T1) and 86 (23%) were more than 4 mm (T4). CONCLUSION: This study indicates that clinical and histological features of melanoma in Iranians (who are mainly of skin phototypes 3-4) are different from those observed in Western countries. Further cohort studies are required to evaluate the role of ethnic and environmental risk factors for melanoma in different populations.

Immunologic prediction of relapse in patients with pemphigus vulgaris (PV) in clinical remission.

BACKGROUND: Pemphigus vulgaris (PV) is characterized by multiple relapses, occurring especially in patients on minimal therapy or off therapy. OBJECTIVE: To identify immunologic predictors (anti-desmoglein [Dsg] 1 and 3 antibodies; direct immunofluorescence [DIF]) for relapse in PV patients. METHODS: Eighty-nine patients in complete clinical remission for at least 6 months and receiving less than or equal to 10 mg prednisolone daily and no immunosuppressive drugs were evaluated using DIF (n=89) and Dsg ELISA (n=46). They were followed until relapse or for at least 18 months. RESULTS: DIF was positive in 44 of 89 patients (49.5%); anti-Dsg 3 antibodies were detected in 18 of 46 patients (39.1%) and anti-Dsg 1 antibodies were detected in 4 of 46 patients (8.7%). Relapse occurred in 38 patients (42.7%). Mean relapse-free time was significantly shorter in anti-Dsg 3-positive patients compared to anti-Dsg 3- negative patients (P = .015) and in DIF-positive patients compared to DIF-negative patients (P = .047), but not in anti-Dsg 1- positive patients compared to anti-Dsg 1-negative patients (P = .501). Sensitivity and predictive values of neither of these tests were high. LIMITATIONS: Small number of anti-Dsg 1-positive patients and use of conventional ELISA. CONCLUSION: Positive anti-Dsg 3 ELISA and, to a lesser degree, positive DIF are predictors of relapse in PV patients in clinical remission. Decision on discontinuing treatment should be based on the results of these tests as well as on clinical findings.

Quality of life in patients with vitiligo: a cross-sectional study based on Vitiligo Quality of Life index (VitiQoL).

BACKGROUND: Vitiligo is a multi-factorial pigmentary skin disorder. Recently, the importance of emotional and psychological issues is proposed in incidence, progression, relapse and remission of vitiligo. There are limited studies conducted in developing countries, which assess life quality of patients with vitiligo. The aim of this study was the application and evaluation of a disease-specific quality of life index in Iranian patients, for the first time. METHODS: This cross-sectional biphasic study was conducted on 25 patients as a pilot and another 173 patients as the main study group, in Razi Hospital, Tehran, Iran, 2013-2014. Persian version of Vitiligo Quality of Life index (VitiQoL) was developed with backward-forward method. Based on the pilot study, the validity and reliability were assessed. The Vitiligo Area and Score Index (VASI), VitiQoL, and their relationship, demographic and clinical characteristic of patients were measured. RESULTS: The Mean and standard deviation of the VitiQoL score was 30.5 ± 14.5 (range 0-60 in Persian version). There was a significant relationship between VASI score and VitiQoL (p = 0.015, r = 0.187). Confirmatory factor analysis revealed three important factors within VitiQoL: participation limitation, stigma, and behavior. In subscale analysis based on behavior factor, female patients had poorer quality of life (p = 0.02). Concomitant psychiatric problems, e.g. anxiety and depression, were not associated with QOL; however, they were near to being meaningful (p = 0.06, r = 0.14). CONCLUSION: VitiQoL is a valid index in estimating life quality of vitiligo patients and has proper relation to disease severity. Focusing on patient's life quality is an important entity in the management of vitiligo patients; relevant supportive group-based consultations and therapies are also important arms when approaching vitiligo.

Trauma-induced pemphigus: a case series of 36 patients.

BACKGROUND AND OBJECTIVES: Pemphigus is a group of autoimmune diseases characterized by intraepidermal acantholytic blisters. Isomorphic responses, or Koebner phenomenon (KP), defined as the appearance of typical lesions of a disease following trauma are rarely reported in pemphigus. Our aim was to present patients who developed new pemphigus lesions as a result of skin trauma. PATIENTS AND METHODS: The medical files of pemphigus patients from the Autoimmune Bullous Diseases Research Center, who had a history of trauma before the onset or flare of their disease, between 1999 and 2013 were reviewed. RESULTS: Thirty-six pemphigus vulgaris (PV) patients had a history of trauma. Thirteen patients developed new-onset PV and the other 23 had previously been diagnosed with PV. Pemphigus lesions developed most often following major surgeries including abdominal, orthopedic, and chest surgeries as well as dental procedures, blunt physical trauma, and skin surgeries. Moreover, post-cataract laser surgery, burns, radiation therapy, and physiotherapy were also shown to induce pemphigus. Mean time between trauma and lesions was 4.7 weeks for recurrent PV and 15.0 weeks for new-onset PV. CONCLUSIONS: Unnecessary surgery and blunt trauma should be avoided in pemphigus patients. Furthermore, posttraumatic pemphigus should be suspected in poorly healing surgical wounds and confirmatory biopsies are mandatory.

Paraneoplastic pemphigus associated with inflammatory myofibroblastic tumour of the mediastinum: A favourable response to treatment and review of the literature.

Paraneoplastic pemphigus (PNP) is an autoimmune blistering disorder that occurs in association with an underlying neoplasm. Inflammatory myofibroblastic tumour (IMT) is a rare low-grade sarcoma of fibroblasts and myofibroblasts associated with inflammatory cells, most commonly occurring in the lung. In this study, a case of PNP associated with IMT of the mediastinum is reported. The patient had a favourable outcome following surgical resection and treatment with a systemic steroid, azathioprine, and i.v. immunoglobulin. The occurrence of PNP with sarcomas, specifically IMT, is noteworthy although it is not well studied in the existing literature.