Clinical efficacy of mychophenolate mofetil in treating lichen planopilaris.

Lichen planopilaris (LPP) is a chronic inflammatory disorder of hair without a proven effective and safe treatment. To objectively assess the clinical efficacy of mycophenolate mofetil (MMF) in patients suffering from LPP, a retrospective cohort study was conducted on 52 patients who treated with MMF (2 g/day) at least for 6 months. LPP activity index (LPPAI) before and after treatment was calculated and compared. Most of the patients were female and belonged to the age group of 50-60 years. All of the disease activity indices were significantly improved after 6 months of therapy (p < 0.001). The majority of patients had LPPAI 4-6 and 0-2, before and after treatment, respectively. After 6 months of treatment, half of patients showed a disease activity decrease (LPPAI reduced >25% compared to the baseline value). Systemic MMF is an effective and relatively safe treatment modality for patients with LPP and could lead to significant reduction in disease activity regarding both subjective and objective indices.

Efficacy and safety of oral pioglitazone in the management of lichen planopilaris in comparison with clobetasol: A randomized clinical trial.

Lichen planopilaris (LPP) is a scarring alopecia for which no treatment with remarkable effect has been identified. Pioglitazone has been reported as a possible therapeutic option. To compare the efficacy and safety of pioglitazone with clobetasol in LPP. This randomized, double-blind, parallel-group was conducted at Razi hospital. Patients were treated either with pioglitazone 15 mg/daily or clobetasol lotion 0.05% once at night for 6 months. Patients were visited every 2 months to assess the lichen planopilaris activity index (LPPAI) and record probable adverse events. Forty patients (mean age: 43.6 years; 62.5% female) were randomized 1:1. The mean of LPPAI at baseline and last session were 4.68 ± 1.97 and 2.59 ± 0.97 in the clobetasol group and 5.01 ± 1.71 and 3.04 ± 1.36 in the pioglitazone group, respectively. Both treatments significantly decreased the LPPAI over the two-month interval visits (p < 0.001). No significant difference in the LPPAI reduction was detected between groups. Regarding the safety profile, three clobetasol-treated patients developed folliculitis, and two in the pioglitazone group developed mild headaches. Pioglitazone effectively controlled the signs and symptoms of the LPP with no serious side effects. It can be considered a treatment option for LPP, although it was not superior to clobetasol.

Association between human leukocyte antigens and cutaneous adverse drug reactions to antiepileptics and antibiotics in the Iranian population.

In this case-control study, class І and ІІ human leukocyte antigen (HLA) alleles in Iranian patients with benign and severe cutaneous adverse drug reactions (CADRs) due to aromatic anticonvulsants and antibiotics were evaluated. Patients diagnosed with CADRs (based on clinical and laboratory findings) with a Naranjo score of ≥ 4 underwent blood sampling and HLA-DNA typing. The control group comprised 90 healthy Iranian adults. Alleles with a frequency of more than two were reported. Deviations from Hardy-Weinberg equilibrium were not observed. Eighty patients with CADRs including 54 females and 26 males with a mean age of 41.49 ± 16.08 years were enrolled in this study. The culprit drugs included anticonvulsants (lamotrigine, carbamazepine, and phenytoin) and antibiotics (ciprofloxacin and co-trimoxazole). The comparison of allele frequencies in the Iranian healthy control group and the group with benign CADRs revealed that HLA-Cw*04, and HLA-A*24 were significantly associated with lamotrigine-induced maculopapular CADRs. Furthermore, HLA-B*51 showed a significant correlation with carbamazepine-induced maculopapular CADRs. Significant associations were also detected between ciprofloxacin-induced urticarial CADRs with HLA-B*40, and HLA-DRB1*14. In the severe group, HLA-B*38 and HLA-DRB1*13 were significantly associated with lamotrigine-induced Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). Moreover, HLA-A*31 and HLA-Cw*04 were significantly correlated with carbamazepine-induced drug reactions with eosinophilia and systemic symptoms (DRESS). HLA-B*08 also showed a significant correlation with ciprofloxacin-induced acute generalized exanthematous pustulosis (AGEP). In conclusion, Lamotrigine-induced MPE was significantly correlated with HLA-Cw*04, and HLA-A*24. Similarly, lamotrigine-induced SJS/TEN was significantly associated with HLA-B*38 and HLA-DRB1*13. Additionally, HLA-A*31 was associated with DRESS caused by carbamazepine. The most frequent CADR-inducing drugs were anticonvulsants.

Pigmented purpuric dermatoses versus purpuric mycosis fungoides: Clinicopathologic similarities and new insights into dermoscopic features.

BACKGROUND: The diagnosis of purpuric mycosis fungoides (PMF) is often challenging to be clinically differentiated from inflammatory diseases such as pigmented purpuric dermatosis (PPD). Dermoscopy as a non-invasive method can be employed for the visualisation of features invisible to the naked eye. OBJECTIVES: This study aimed to survey the dermoscopic findings of PMF in comparison with PPD. METHODS: Forty-one patients with an established diagnosis of PMF (n = 28) and PPD (n = 13) were prospectively recruited. Dermoscopic images were taken by FotoFinder Medicam 1000 (FotoFinder Systems GmbH, Bad Birnbach, Germany). RESULTS: Characteristic dermoscopic patterns consisting of fine short linear vessels (35.7%, P = 0.017) and spermatozoa-like structures (50%, P = 0.014) were found to be significantly more common in PMF lesions, while PPD lesions were typified by erythematous globules (76.9%, P = 0.01), in the background colour of dull red (61.5%, P = 0.01) and reticular pigmentation (61.5%, P = 0.044). CONCLUSIONS: This study showed the usefulness of dermoscopy for the diagnosis of PMF and PPD cases. Studies with long-term follow-up are needed to affirm the value of these dermoscopic patterns in the differentiation between the two entities.

Efficacy and safety of hydrogen peroxide topical solution in treating actinic keratosis: A randomized controlled trial.

Actinic keratosis (AK) is a precancerous skin lesion associated with the development of squamous cell carcinoma. Approved topical treatments for AK are unmet needs. To evaluate the safety and efficacy of hydrogen peroxide topical solution 35% (H2 O2 ) with cryosurgery for the treatment of AKs. Eligible subjects were randomly assigned to receive H2 O2 and cryosurgery on each side of their scalp/face. Patients were assessed at baseline and after 3 months of follow-up. Efficacy was evaluated based on the Actinic Keratosis Area and Severity Index (AKASI) and dermoscopic assessments. Fifteen patients with 98 lesions completed the study. At the follow-up visit, the mean improvement of AKASI from baseline was 1.7 ± 0.9 for H2 O2 and 1.7 ± 0.8 for cryosurgery (p < 0.001, both). Both treatments significantly improved dermoscopic features of yellow scale, white scale, linear-wavy vessels, pigmented dots, and rosette at the end of the 3-month follow-up. Complete dermoscopic response occurred in 57.7% and 73.9% of lesions treated with H2 O2 and cryosurgery, respectively (p > 0.05). Regarding safety, hypopigmentation occurred more frequently in the cryosurgery group (28.3% vs. 9.6%; p = 0.017). Moreover, patients endured more pain with cryosurgery application than H2 O2 (p < 0.001). The effect of H2 O2 solution was comparable to cryosurgery. This treatment was well tolerated and had a favorable safety in patients with AKs.

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.

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.

Real life management of chronic urticaria: Multicenter and cross sectional study on patients and dermatologists in Iran.

Recently, advances in understanding the etiology of urticaria and updates of diagnostic and therapeutic management guidelines have drawn attention to chronic urticaria (CU) morbidity. The present study aimed to evaluate Iranian dermatologists' practice and real life management of CU patients. A total of 35 dermatologists and 443 patients were included in the study. Number of female patients was 321 (72.5%). Mean (standard deviation) age of the study patients was 38 (13) years and the median (inter quartile range) of disease duration was 12 (6-48) months. Severity of patients' symptoms was mild for 32.1%, moderate for 38.7%, severe for 18.8%, and 10.4% of them had no evident signs or symptoms. The most common diagnostic methods were physical examination (96.6%), differential blood count (83.5%), erythrocyte sedimentation rate (77.4%), and C-reactive protein (62.8%). The number of dermatologists prescribed nonsedating antihistamines (nsAH) in regular dose and high dose mono therapy were 26 (74%) and 6 (17%), respectively. About 66% of dermatologists were familiar with British Association of Dermatologists (BAD) guideline. The most common first-line treatment for CU by Iranian dermatologists was nonsedating antihistamines in regular or high doses. The real-life management of patients with CU in Iran was in accordance with the available practice guidelines.

Cheilitis in acne vulgaris patients with no previous use of systemic retinoid products.

BACKGROUND/OBJECTIVES: Isotretinoin is commonly used in the treatment of acne vulgaris. While one of the more common side-effects is cheilitis, we have observed an increased incidence of cheilitis prior to the commencement of systemic isotretinoin. The aim of this study was to assess the prevalence of cheilitis among acne vulgaris patients. METHODS: A non-interventional cross-sectional study of patients with acne vulgaris. Patients with previous use of systemic retinoids were excluded. The patients were examined for signs and symptoms of cheilitis. RESULTS: Of a total of 400 patients, 134 (34%) had evidence of cheilitis at initial presentation. Two-thirds (63%) were female (P < 0.001). The distribution of the cheilitis was as follows; 55% on the lower lip, 30% on both lips, and 16% on the upper lip. Over a quarter (27%) of patients with cheilitis had acne excorie, compared with only 8% of patients with no signs of cheilitis. CONCLUSION: Our findings suggest that cheilitis is quite common among acne vulgaris patients even before treatment with isotretinoin.

Treatment of erosive oral lichen planus with methotrexate.

BACKGROUND AND OBJECTIVES: Erosive oral lichen planus (EOLP) is a chronic inflammatory disease of the oral mucosa causing significant pain and impairment of the quality of life. No immediate and definitive cure is available, especially in its chronic and most recalcitrant forms. The objective of this study was to evaluate the efficacy of methotrexate treatment in EOLP. PATIENTS AND METHODS: A prospective open trial of oral methotrexate 15 mg QWK in 18 patients with EOLP (clinically and histopathologically confirmed) unresponsive to at least one previous topical or systemic medication. DURATION: Twelve weeks followed by twelve weeks of follow-up. RESULTS: Partial response or better was achieved in 15 (83.3%) patients. A statistically significant reduction in Thongprasom scale score was observed (mean value 5 at baseline, 3.08 ± 1.11 at week 12, 2.94 ± 0.97 at week 24, P value < 0.001). A statistically significant reduction was noted in the pain visual analogue scale (mean value 6.55 ± 2.33 at baseline, 2.46 ± 2.18 at week 12, 2.29 ± 1.93 at week 24, P value < 0.001). Adverse events occurred in four (22.2%) patients, requiring discontinuation of treatment in one patient. CONCLUSION: Methotrexate is a valuable therapeutic option in longstanding recalcitrant EOLP, particularly with respect to the improvement of subjective symptoms. Randomized controlled trials are required.

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.

Prevalence of Multiple Sclerosis in Vitiligo Patients and Their First-Degree Relatives: Two Diseases with Similarities in Pathogenesis and Treatment.

INTRODUCTION: Vitiligo is a common pigmentation disorder manifested by white macules and patches. It is accompanied by some autoimmune and neurological diseases. Recently, it has been suggested that multiple sclerosis (MS) is more common in vitiligo patients and that they have a higher risk of developing MS during their lifetime. OBJECTIVES: In this study, we aimed to determine the prevalence of MS in patients with vitiligo and their first-degree relatives and compare it with the prevalence in the population. METHODS: In this cross-sectional study, data were consecutively collected from patients referred to Razi Hospital from March 2020 to December 2021. RESULTS: Seven hundred and nine patients with vitiligo participated in this study, and 15 reported a history of MS (2.12%, 95% confidence interval [CI] 1.06%-3.17%). This rate was significantly higher than the prevalence of MS in the average population of Tehran (P < 0.001). Of the 2886 first-degree relatives of the patients, 10 had MS (0.35%, 95%CI 0.13&-0.56%), which was higher than the prevalence of MS, yet not statistically significant. CONCLUSIONS: A significant association between vitiligo and MS was observed, which should be of clinical and therapeutic importance. However, the prevalence of MS in first-degree relatives of vitiligo patients was higher than the average rate, yet not statistically significant.

The efficacy and safety of oral low dose naltrexone versus placebo in the patients with lichen planopilaris: a randomized controlled clinical trial.

BACKGROUND AND OBJECTIVES: Lichen planopilaris (LPP) is one of the important causes of cicatricial alopecia. We aimed to evaluate the efficacy and safety of low-dose naltrexone (LDN) in the setting of a clinical trial in patients with LPP. METHODS: We included patients with LPP between 2018 and 2020. Patients were allocated to two groups. The first group received topical clobetasol plus oral low dose naltrexone (3 mg) while the second received topical clobetasol plus placebo. The assessment was made for the disease severity by lichen planopilaris activity index (LPPAI) instrument and the safety of the drug in 2-month intervals up to 6 months. To compare both groups, we used the ANOVA test for repeated measures. Clinical trials registry code: IRCT20180809040747N1. RESULTS: Thirty-four patients were analyzed in an intention-to-treat fashion. There was a decrescendo pattern on LPPAI scores that was statistically significant within the LDN (p = .001) but almost significant within the placebo group (p = .060) and non-significant between the groups (p = .813). The side effects attributable to the low dose naltrexone was not statistically different between studied groups. CONCLUSION: Low-dose naltrexone (3 mg) failed to improve the severity of the LPP more than what is achievable with topical clobetasol.

A split-face comparison of fractional erbium: YAG laser plus Kligman's formula vs. Kligman's formula monotherapy for facial melasma.

Melasma is a chronic hyperpigmentation disorder. Although several treatment methods are used for patients, it remains a challenging problem for physicians. Erbium-YAG laser is one of the laser treatment methods that proved its efficacy in melasma treatment. We aimed to compare the efficacy of combining the fractional Er: YAG laser and Kligman's formula with Kligman's formula alone in the treatment of melasma. Twenty female patients with bilateral melasma were randomly treated in a split-face controlled manner with a fractional Er: YAG laser followed by Kligman's formula on one side and Kligman's formula on the other side. All patients received three laser sessions with four-week intervals. The efficacy of treatments was determined through photographs, Visioface, and Melasma Area Severity Index (MASI) score, all performed at baseline and three months after the end of laser sessions. Fractional Er: YAG laser and Kligman's formula showed a significant decrease in MASI score than Kligman's formula alone (P = 0.005). There was a significant decrease in cheek Visioface score on Er: YAG laser and Kligman's formula vs Kligman's formula (P = 0.02). However, the Patient Global Assessment Scale of both sides was not statistically significant (P = 0.23). The combination of Fractional Er: YAG laser with Kligman's formula is an effective treatment method for melasma.

Comorbidities in Patients with Lichen Planopilaris: A Case-Control Study.

Introduction: Lichen planopilaris (LPP) is a lymphocyte-mediated type of scarring alopecia and considered to have autoimmune etiology. Studies about systemic comorbid conditions are limited. Our goal is to identify the prevalence of medical comorbidities in patients with LPP. Methods: In a retrospective case-control study, the medical records of 208 LPP patients and 208 controls were reviewed for existing comorbidities such as thyroid diseases, cardiovascular disorders, hypertension, hyperlipidemia, and lupus erythematous. Results: Hyperlipidemia was found in 41.8% of all patients with LPP and in 17.3% of controls (p value <0.001, OR = 4.167). Chances of hypertension and cardiovascular disorders were lower in the LPP group in comparison to controls (p value = 0.009). Thyroid disorders were more prevalent in LPP patients, but the difference was not statistically significant (p value = 0.277). Conclusion: Our study further emphasizes that LPP patients should be screened for medical comorbidities, especially lipid profile abnormalities.

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.