Diagnosis of Behçet's disease: clinical characteristics, diagnostic criteria, and differential diagnoses.

BACKGROUND: The diagnosis of Behçet disease (BD) is challenging in many cases. The purpose of this study was to describe the clinical characteristics of patients at a referral BD clinic. METHODS: In a retrospective study, we collected data from patients at a national referral Behçet clinic from November 2018-August 2019. A BD diagnosis was confirmed (BD group) or ruled out (Non-BD group), and the two groups were compared for differences. RESULTS: A total of 238 patients satisfied the inclusion criteria. Forty patients (16.8%) were finally diagnosed with BD. Ocular and genital lesions were significantly more prevalent in the BD group. A positive pathergy test and HLA-B51 were also significantly more common in BD. However, oral lesions, articular involvement, and gastrointestinal manifestations were similar between groups. Also, patients with BD were significantly more likely to have multi-organ (≥2 organ systems) involvement. CONCLUSIONS: Being the first study to evaluate the clinical characteristics of patients who are visited at a referral BD clinic and are believed to have a high probability of Behçet, the results of this study are important from an epidemiological standpoint. Also, the findings of this study could be used by referral Behçet clinics, which evaluate and diagnose patients with a high pretest probability and atypical presentations of BD on a daily basis. The alternative diagnoses established in this study could be used as the list of the most common differential diagnoses for Behçet's disease.

Quality of life and mental health status in patients with lichen planopilaris based on Dermatology Life Quality Index and General Health Questionnaire-28 questionnaires.

BACKGROUND: Lichen planopilaris (LPP) is a relatively uncommon inflammatory skin condition that causes permanent hair loss. Irreversible hair loss can have a significant psychosocial and psychological impact on patients' lives. Limited studies have assessed the psychological status of patients suffering from LPP, and to our knowledge, none have evaluated patients with LPP as a separate group in this regard. OBJECTIVE: This study aimed to assess the quality of life (QoL) and general health of patients with LPP using the Dermatology Life Quality Index (DLQI) questionnaire and General Health Questionnaire-28 (GHQ-28), respectively. METHODS: Our study employed a cross-sectional design. In total, 41 patients with LPP attending the follow-up skin clinic at the Razi Hospital in Tehran, Iran were asked to complete the DLQI and GHQ-28. Furthermore, selected demographic information was obtained from patients to evaluate their association with general health and QoL. RESULTS: Forty-one patients (14 men and 27 women) with a mean age of 44.02 ±â€¯10.8 years completed both questionnaires. QoL was affected moderately to extremely in 70.7% of patients. Also, 26 patients (63.4%) were at risk for psychological disorders. Lower QoL was reported by patients age <45 years (p < .05). Both QoL and general health had a negative relation with the disease activity index (p < .05), but were not affected by sex, marital status, education level, treatment type, presence of mucous lesions, and disease duration. CONCLUSION: LPP significantly affects patients' QoL and general health. Dermatologists should address these issues in patients with LPP alongside treating physical symptoms.

Eosinophilic infiltration: an under-reported histological finding in urticarial vasculitis.

BACKGROUND: Urticarial vasculitis (UV) is a clinicopathologic entity in which skin lesions resemble urticaria clinically but last more than 24 hours and resolve with residual purpura. In this study, we evaluated distinguishing histological features of UV from common urticaria to differentiate between these two entities. METHODS: During 1 year, 55 urticaria and 245 UV samples were assessed. The selection criteria for UV included: (1) samples that the histological diagnosis was confirmed by two independent dermatopathologists, (2) the histological diagnosis of UV was inconsistent with the first clinical impression and lesion characteristics. RESULTS: Of our 245 UV patients, 96% had endothelial swelling, 18% had red blood cell (RBC) extravasation, neutrophilic infiltration was seen in 46.7%, and perivascular, interstitial, and perineural eosinophilic infiltration in 198 (81.8%), 181 (74.2%), and 118 (48.1%), respectively. Among different histological findings, endothelial cell swelling as well as neutrophil around vessels and eosinophilic infiltrations were statistically different between two groups (P-value Ë‚ 0.001). Both urticaria and UV samples showed a predominantly lymphocytic infiltration more than 10 cells per high power field (HPF) (98.2% of urticaria, 98.8% of UV), and only two patients (0.8%) with UV had predominant neutrophilic infiltration (more than 10 cells/HPF). CONCLUSION: We have found that both urticaria and UV showed a predominantly lymphocytic infiltration along with RBC extravasation, endothelial cell swelling, and fibrin deposition in UV. Moreover, significant perineural, perivascular, and interstitial infiltration of eosinophil in UV lesions compared to urticaria was another point that indicates special contribution of these cells in UV pathogenesis.

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.

Efficacy of Oral Itraconazole in the Treatment and Relapse Prevention of Moderate to Severe Seborrheic Dermatitis: A Randomized, Placebo-Controlled Trial.

BACKGROUND: Seborrheic dermatitis (SD) is a chronic and relapsing disease and topical therapy may be associated with failure, particularly in severe disease. Itraconazole has been suggested as an effective treatment for severe SD. Previous studies have been open clinical trials with variable results. OBJECTIVE: The aim of this study was to determine the efficacy of oral itraconazole in the treatment of patients with moderate to severe SD. METHODS: Sixty-eight patients with moderate to severe SD were randomly assigned to the itraconazole (n = 35) or placebo (n = 33) groups. The trial was undertaken in Razi Hospital, Tehran. An internet-generated table was used to allocate treatments. Patients and investigator were blinded to treatments. Itraconazole 200 mg/daily or placebo was prescribed for 1 week and then for the first 2 days of every month for the following 3 months. Patients were followed for 4 months and the Seborrheic Dermatitis Area Severity Index (SDASI) was measured on nine anatomical sites. Fifty-seven patients (29 in the itraconazole group and 28 in the placebo group) completed the study. RESULTS: Statistically significant improvement was observed in SDASI of both itraconazole and placebo groups (p = 0.000) but the itraconazole group showed significantly higher efficacy compared with placebo (p = 0.023). We observed clinical improvements of 93.8, 87.5, and 93.1% at the end of 2 weeks, 1 month, and 4 months, respectively, in the itraconazole group, and 82.1, 64.3, and 53.6% in the placebo group. Furthermore, recurrence rate in the itraconazole group was significantly lower than in the placebo group (p = 0.003). No blood test abnormality was seen in any patient. CONCLUSION: Itraconazole is not only an effective and safe therapy for controlling exacerbations of SD but may also be used as maintenance therapy to prevent disease recurrence.

Comparison of systemic mycophenolate mofetil with topical clobetasol in lichen planopilaris: a parallel-group, assessor- and analyst-blinded, randomized controlled trial.

INTRODUCTION: Lichen planopilaris (LPP) is the most common cause of inflammatory immune-mediated cicatricial alopecia. If not diagnosed and treated properly, it may lead to irreversible hair loss with a devastating impact on quality of life. However, treatment can be a challenge. In an area lacking these sorts of studies, we conducted a randomized controlled trial (RCT) to study the tolerability and therapeutic effects of topical clobetasol versus systemic mycophenolate mofetil (MMF). METHODS: A randomized, assessor- and analyst-blinded controlled trial was conducted in 60 patients with LPP in Razi Dermatology Hospital, Tehran, Iran, between February and December 2013. Patients were treated with clobetasol lotion 0.05 % applied at night or oral MMF 2 g/day and were followed for 6 months. The Lichen Planopilaris Activity Index (LPPAI) was the primary measure of response to treatment. RESULTS: Systemic MMF and topical clobetasol were equally effective in reducing the LPPAI over 6 months of treatment. Treatment tolerability was excellent in both groups and no serious irreversible adverse effects were detected. Satisfaction with treatment rose in the MMF group over time; however, it declined in the clobetasol group. CONCLUSION: Given the similar efficacy profiles, topical clobetasol seems to be a more suitable and reasonable choice for treatment of LPP than MMF.

Assessment of androgens in women with adult-onset acne.

BACKGROUND: Acne is generally recognized as a disorder of young adults; however, the referral of patients aged over 25 years with acne is increasing. Disturbed androgen production in the ovaries or adrenal gland and impaired plasma transport of androgens in women with adult-onset acne or acne associated with hirsutism have been described. METHODS: Thirty-five white women with adult-onset acne (onset after the age of 25 years) and hirsutism (A + H), 35 white women with adult acne without hirsutism (A - H), and 35 age-matched white female controls were recruited in this case-control study. Serum levels of luteinizing hormone (LH), follicle-stimulating hormone (FSH), total testosterone, dihydroepiandrosterone sulfate (DHEA-S), and sex hormone binding globulin (SHBG) were determined in all patients and compared. RESULTS: The mean SHBG, free androgen index (FAI), and DHEA-S were significantly different between A + H and control subjects. The only significant difference between A - H and control subjects was observed for DHEA-S. CONCLUSION: DHEA-S plays a key role in the pathogenesis of adult-onset acne. Measurement of circulating androgens, including DHEA-S, especially in patients presenting with adult-onset acne and hirsutism, is helpful, and patients with elevated levels can benefit from hormonal therapy.

Two unusual cases of dental (periapical) infection.

Cutaneous sinus tracts of dental origin are often a diagnostic challenge. A delay in correct diagnosis can result in inappropriate treatments. We describe two unusual cases of periapical infection. The first patient presented with a fistulized tumoral mass under the chin present for 1 year. The patient had only one tooth, and that was anatomically unrelated to the tumor. Radiological examination showed a dormant tooth that was infected periapically. The second case was a man who complained of asymmetry and change in his left nasolabial fold of 4-5 week's duration. On examination, the nasolabial folds were asymmetric without any sign of facial palsy. On intraoral examination, there was a severely decayed tooth on the same side. Radiological survey confirmed periapical infection. A few weeks after proper treatment, the nasolabial folds became symmetric.