Brain MRI findings in patients with bullous pemphigoid: A case-control study.

Evidence suggests that bullous pemphigoid (BP) is associated with multiple neurological disorders. We aimed to compare brain magnetic resonance (MRI) findings between BP patients and a control group. This case-control study included patients with BP referred to two dermatology clinics during a two-year period. A group of individuals attending the same clinics for cosmetic procedures were selected as controls. First, participants' general information including age, gender, education, weight and underlying disease was recorded. For BP patients, the drugs and the BP Disease Area Index (BPDAI) were recorded as well. Then, all participants underwent brain MRI without contrast. The Fazekas scale, the general cerebral atrophy (GCA) score, and the Medial Temporal lobe Atrophy (MTA) score were used to assess MRI images. Overall, 24 BP patients and 24 controls were evaluated in this study. Both groups were comparable regarding age, gender and education. However, diabetes and hypertension were more frequent in the control group. The mean BPDAI total score was 51.39 ± 68.92 in BP patients and most of them used rituximab (41.7%). None of the participants had MS or Alzheimer MRI patterns. There was no difference between groups in terms of GCA and MTA scores. Furthermore, the frequency of partially empty sella did not differ significantly between BP patients and controls (p = 0.461); nevertheless, grade-3 Fazekas was significantly higher in BP patients compared to controls (25% vs. 0%, p = 0.019). Of note, one BP patient had an epidermoid cyst and another had moderate enlargement of three ventricles. Also, new infarcts were observed in two and old infarcts in four BP patients. Although the majority of abnormal brain MRI findings were more frequent in BP patients compared to controls, only grade-3 Fazekas was significantly higher and acute infarcts were exclusively observed in BP patients.

Evaluation of combination use of phenytoin cream 1% and fractional CO2 laser in treatment of stable nonsegmental vitiligo: A pilot study.

Vitiligo is an autoimmune disorder of melanocyte characterized by macular and depigmented patches. There are several treatment modalities for this disease, including the use of corticosteroids, calcineurin inhibitors, vitamin D analogous and topical phenytoin. Combination therapy utilizing fractional CO2 laser with different topical agents has been used to enhance treatment response with promising results. In this study, we aimed to evaluate the effect of fractional CO2 laser in combination with topical phenytoin. In this study, 25 patients (11 females and 14 males) with age of 18-59 (mean age of 31.12) with nonsegmental stable vitiligo were recruited with insufficient response to at least 1-year treatment with a monotherapy using topical corticosteroids, calcineurin inhibitors, and/or NB-UVB phototherapy. Patients were treated with a combination of fractional CO2 laser (10,600 nm, pulse energy 30-50 mJ, MIXEL, South Korea, Rating: 220VAC, 3A, 50/60 Hz) with monthly intervals for six sessions and application of phenytoin 1% cream twice daily. Photography was done before and after treatment with Wood's lamp. The severity of disease using VASI score was calculated and compared before and after treatment. The mean VASI score before treatment was 0.55, and sixth month after treatment increased to 1.97 (p-value < 0.001). Patients were divided into three groups based on the vitiligo subtype: acral, upper extremities, and trunk. VASI score was measured in each group: VASI score before and after treatment was 0.50 and 1.48 in acral areas, 0.45 and 2.04 in upper extremities and 0.79 and 3.39 in trunk, respectively. This study revealed that combination therapy with phenytoin and fractional CO2 laser is effective in treatment of vitiligo not only in the upper extremities and trunk, but also interestingly in the acral areas.

Ichthyosis follicularis syndromes in patients with mutations in GJB2.

Ichthyosis follicularis (IF) manifests as generalized spiny follicular projections found in syndromic diseases secondary to SREBF1 and MBTPS2 mutations. We sought the genetic cause of IF in two distinct families from a cohort of 180 patients with ichthyosis. In Family 1, the proband (Patient 1) presented with IF, bilateral sensorineural hearing loss and punctate palmoplantar keratoderma. Using DNA from peripheral blood lymphocytes, two compound heterozygous mutations, c.526A>G and c.35delG, were discovered in GJB2. In Family 2, the proband (Patient 2) presented with a previously unreported IF phenotype in the context of keratitis-ichthyosis-deafness syndrome, and whole-exome sequencing found a de novo heterozygous mutation, c.148G>A in GJB2. Histopathology was consistent with porokeratotic eccrine ostial and dermal duct naevus (PEODDN) and IF in Patients 1 and 2, respectively. Our findings add to the clinical and histopathological spectrum of IF and emphasize the association of PEODDN-like entities with GJB2 variants.

Acquired lymphangioma circumscriptum secondary to tuberculosis: A rare case report.

Lymphangioma circumscriptum is a benign lymphatic malformation that usually presents at birth or early childhood. Acquired oral lymphangioma has been reported secondary to radiation therapy and denture-induced trauma due to damage to previously normal lymphatics. To the best of our knowledge, this is the first report of acquired oral lymphangioma due to childhood tuberculous adenitis.

High-sensitivity C-reactive protein as a biomarker in detecting subclinical atherosclerosis in psoriasis.

Psoriasis is known to be associated with increased risk of cardiovascular diseases. High-sensitivity C-reactive protein (hs-CRP) is a marker of inflammation and an independent risk factor for atherosclerosis. We aimed to assess the correlation between hs-CRP and subclinical atherosclerosis in psoriatic patients. In 60 patients with moderate to severe psoriasis and 60 age- and gender matched healthy controls, we evaluated the serum hs-CRP level and mean intima-media thickness of the common carotid artery (MIMT-CCA). Psoriatic patients had higher levels of hs-CRP (median, 2.25 mg/L; IQR, 0.98-3.80; and range, 0.29-11.60) than did those in the control group (median, 1.03 mg/L; IQR, 0.36-2.15; and range, 0.10-3.35). Psoriatic patients also had higher mean MIMT (0.74 ± 0.19 and 0.54 ± 0.12, respectively, and P < .0001) compared with healthy subjects. The serum level of hs-CRP was significantly correlated with MIMT (P < .0001). Our results indicate that psoriatic patients have a higher risk of subclinical atherosclerosis and hs-CRP may be a useful marker for future risk of cardiovascular diseases in these patients. So, not only does anti-inflammatory drugs play a key role in the treatment of psoriasis, but also they may reduce the risk of cardiovascular diseases by decreasing level of inflammatory markers including hs-CRP.

Methylprednisolone pulse therapy plus adjuvant therapy for pemphigus vulgaris: an analysis of 10 years' experience on 312 patients.

Steroid pulse therapy has shown satisfactory efficacy and safety in treating pemphigus vulgaris (PV). However, there is a paucity of data about the efficacy and safety of methylprednisolone, despite its frequent administration. The aim of this study is to evaluate the efficacy and safety of steroid pulse therapy in treating PV. In this 10-year retrospective cohort study, 312 patients with PV, who had received methylprednisolone pulse therapy, were included. Data of pulse therapy sessions, adjuvant medications, dosages, remission rates, complications, and mortalities were collected from all patients. A total of 276 patients out of 312 underwent the final follow-up at least 6 months after the last session of pulse therapy. Complete remission off therapy was achieved in 83 patients (30%), and 152 patients (55%) had complete remission on therapy. About 29 (10.5%) patients had lesions of pemphigus at the time of the study follow-up, and 26.8% of remained patients were on the minimal therapy. Methylprednisolone pulse therapy could be considered as an option for proper control of PV in severe cases. It might lead to shorter periods of hospitalization and reduce the need to take long-term high-dose oral steroid therapy.

Coexistence of mucous membrane pemphigoid, vitiligo, and hypothyroidism: A second report of a new multiple autoimmune syndrome.

Mucous membrane pemphigoid (MMP) is a rare immunobullous disease affecting the mucous membrane and skin. An association of MMP with other autoimmune disease is uncommon. Multiple autoimmune syndrome (MAS) is a subclass of autoimmune disorders with a coexistence of three or more autoimmune disorder in a single patient. The second report of the association of vitiligo, autoimmune thyroid disease, and MMP shows that this combination can be considered as a new type of MAS, although its mechanism and pathology are unknown.

Presentation of vitiligo in a case of COVID-19 infection concomitant with receiving remdesivir.

The occurrence of vitiligo following COVID-19 infection and vaccination is well-documented. The mitochondrial dysfunction of melanocytes in vitiligo and the potential impact of RDV on mitochondria raise concerns about RDV possibly causing vitiligo.

A rare case of cicatricial pemphigoid confined to the scalp with associated photodamage.

We present a case of Brunsting-Perry characterized as an erythematous erosive plaque on photodamaged scalp skin, flaring after a recent prolonged sun exposure. Subsequent progression with blister formation led to the correct diagnosis, highlighting the need to consider cicatricial pemphigoid in eroded lesions without blisters, particularly on photodamaged skin.

Comparing quality of life, anxiety, depression, sleep disturbance, and associated factors in vitiligo and alopecia areata patients.

INTRODUCTION AND OBJECTIVES: Vitiligo and alopecia areata (AA) are two autoimmune skin diseases that affect patients' quality of life (QoL) and give rise to psychosocial complications, such as depression, negative self-image, less joyful social engagements, and low self-esteem. These two disorders have common and uncommon characteristics. Therefore, in this study, we tried to evaluate the similarities and differences in the psychological parameters including quality of life, sleep disturbance, anxiety, and depression levels between, vitiligo and AA patients. MATERIALS AND METHODS: Patients with either vitiligo or AA visiting the outpatient dermatology clinic from November 2017 to December 2020 have been included in this study. Persian versions of three questionnaires including the dermatology life quality index (DLQI), hospital anxiety and depression scale (HADS), and Pittsburgh sleep quality index (PSQI), have been used to assess the QoL, sleep disturbance, anxiety, and depression levels in patients. RESULTS: In total, 188 patients, including 94 (50%) cases of AA and 94 (50%) patients with vitiligo, met the criteria. In AA patients, a significantly higher DLQI score was found (p-value = 0.002) compared to the vitiligo cases, which means a better QoL in vitiligo patients. Additionally, AA patients had higher scores of anxiety (P-value<0.001) and depression (p-Value<0.001). However, sleep disturbance (64.9% of AA patients vs. 59.3% of vitiligo patients; p-Value = 0.4888) was not significantly different between the two groups. CONCLUSIONS: Our data showed lower QoL and higher levels of anxiety and depression in AA patients compared to vitiligo cases, but no difference was seen in sleep disturbance in the PSQI-P score.

The effect of topical tranexamic acid with micro-needling and micro-needling alone in treatment of macular amyloidosis.

BACKGROUND: Macular amyloidosis is a form of primary localized cutaneous amyloidosis presented by pruritic pigmented macules in rippled or reticulate pattern. The aim of this study was to assess the efficacy of using topical tranexamic acid with micro-needling comparing to micro-needling alone in patients with macular amyloidosis. MATERIALS AND METHODS: Patients with bilaterally located macular amyloidosis on trunk or upper extremities were recruited in this trial. The skin lesions in all patients were divided into two parts which were randomly assigned to the group of treatment with micro-needling plus tranexamic acid and the group of micro-needling alone. There were four sessions of treatment with 2 weeks interval. The percentage of improvement in pigmentation (based on photographs and dermoscopy) and rippling of each group was determined by three blinded dermatologists. The level of patient satisfaction and reduction of pruritus was measured by a questionnaire and defined as a percentage. RESULTS: Twenty females were enrolled in this study. The mean (SD) patients' age was 39.7 (±10.13) years. Both groups showed improvement in pigmentation based on images, dermoscopy, and rippling pattern. Patients' satisfaction was 46.5% in tranexamic acid group and 47.5% in micro-needling alone. Nevertheless, there was no significant difference between both groups (p value >0.05). Interestingly, the pruritus improved 61.66% after four sessions of treatment in both groups. CONCLUSION: Micro-needling is a suitable modality for decreasing pruritus and pigmentation in macular amyloidosis. However, topical application of tranexamic acid does not lead to additional improvement.

Iranian psoriasis registry: Research protocol and preliminary results of a pilot study.

BACKGROUND: Psoriasis as a common cutaneous inflammatory disease affect many aspects of patients' life. Disease registries render it possible to collect valuable data regarding a disease prevalence and burden as well as long-term observations concerning possible therapeutic regimens. METHODS: This registry was designed for the ongoing systematic data collection on patients with psoriasis at two referral dermatology centers in Iran. The pilot phase of the registry was used to identify possible obstacles in the application and execution of systematic registration. RESULTS: A total of 281 patients were registered with the mean age of 42.02 years. The disease duration was 12.06 ± 10.90 years with the variety of clinical presentations. There was no significant difference between males and females in the age of disease onset (p = 0.53). Notably, 167 patients had children. Among them, 13 had children with psoriasis. The gender of the affected parent did not affect the possibility of psoriasis transmission to the child, and no significant difference was seen between the two sexes (P = 0.569). Regarding treatment, 99.4% of patients (n = 280) had used topical agents, 52.3% (n = 147) biologics, and 60.9% (n = 171) nonbiologic medications. CONCLUSION: Clinical trials report the efficacy and safety data regarding limited study populations in a restricted time window, and the results may differ from the general population. This highlights the importance of registry-based studies for collecting and analyzing longitudinal information. In terms of long-term disease complications such as malignancies, cardiovascular events, and serious adverse events, registry-based studies will help clinicians better recognize and manage each disease.

Facial pyoderma gangrenosum associated with fallopian tube carcinosarcoma.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis associated with underlying disorders. The association between PG and solid organ tumors (SM), including gynecologic cancers, has been previously reported. Here, we report a case of a 61-year-old woman with pyoderma gangrenosum on the posterior auricular region associated with an underlying fallopian tube carcinosarcoma: a rare and aggressive gynecologic malignancy. The patient's ulcer responded favorably to treatment, and surgical resection of the tumor was performed. The patient was then referred for further cancer management. No new lesions or recurrences were found over the 18 months of routine follow-up.

Perceived impact of COVID-19 pandemic on body dysmorphic disorder and anxiety among population seeking cosmetic procedures.

INTRODUCTION: COVID-19-related anxiety and preventative actions like the widespread shutdown of beauty-related services emerged as a stressful condition for BDD patients seeking expert opinions. MATERIAL AND METHODS: We conducted a cross-sectional investigation to assess anxiety symptoms (by CDAS) and BDD (via BDD-YBOCS questionnaire) in patients referred to the esthetic clinic. RESULTS: A total of 120 individuals (106 females and 14 males; mean age: 45.53 ± 11.84) were evaluated. 13.3% of subjects were diagnosed with BDD; 79.2%, 17.5%, and 3.3% of them perceived none or mild, moderate, and severe symptoms of anxiety, respectively. CONCLUSION: Anxiety was not connected with BDD in our investigation. Further evaluation of other psychiatric disorders associated with anxiety and BDD was recommended.

Shared histological and immunohistological findings in two patients with generalized vitiligo associated with autoimmune atrophic gastritis.

The prevalence of autoimmune atrophic gastritis (AAG) in vitiligo patients was estimated at about 15%. In both conditions, a release of specific antibodies and an autoimmune destruction of target cells (melanocytes in vitiligo, parietal cells (PC) in AAG) mediated by CD8-T lymphocytes was demonstrated to perform a comparative histological study of vitiligo skin and AAG mucosa. In two patients with concomitant vitiligo and AAG, biopsies from the vitiligo lesions and gastric mucosa from corpus fundus were performed. Sections were immunostained with E-cadherin, Coll IV, CD8, CD20, CD4 antibodies. The skin sections also were stained with HES, HMB45, MITF. Common histological findings were found in both diseases. Adhesivity impairment with down expression of E-cadherin and Coll IV was objectivated. The protruding MITF+melanocytes and the detached PC were surrounded by an infiltrate including CD8 and CD4. CD8 was infiltrating the epidermis in close contact with the remaining melanocytes and the gastric glands around the remaining PC. In both diseases, the autoimmune process could be preceded by a detachment of either melanocytes in vitiligo or PC in AAG possibly in relation to an initial adhesivity impairment of these cells. Common autoimmune mechanisms could be suggested for both diseases.

A case of severe psoriatic erythroderma with COVID-19.

COVID-19 caused by the SARS-CoV-2, became pandemic very quickly. Management of severe dermatologic disorders in patients who require systemic immunosuppressive treatment is a major concern in COVID-19 pandemic era. Here, we report a 45-year-old homeless addicted male with second flare of psoriatic erythroderma and positive PCR test for COVID 19. His first attack occurred two months earlier, when he was screened for SARS-CoV-2 before admission which all evaluations showed nagative results. The patient was treated and relatively controlled with cyclosporine and therefore he was discharged. During this interval, he not only discontinued his medication, but also became SARS-CoV-2 positive. It seems that both factors participated in flare of his erythroderma.

Multiple Becker's Nevi in a Female Patient Without Musculoskeletal or Developmental Abnormalities: A Case Report and Review of the Literature.

Becker's nevus (BN) is one of the cutaneous hamartomatous lesions that often presents as an acquired, single unilateral pigmented hairy patch on the upper trunk, scapular region, or upper arm. Hereby we introduce a 13-year-old girl with the unusual manifestation of BN with multiple acquired, pigmented hairy patches for six months without any musculoskeletal or developmental involvements. After carefully reviewing the literature, to the best of our knowledge, this is the first case in this regard in Iran.

Tacrolimus (FK506) ointment combined with Nb-UVB could activate both hair follicle (HF) and dermal melanocyte precursors in vitiligo: the first histopathological and clinical study.

Topical Tacrolimus, especially when combined with Nb-UVB, has been proven clinically to be effective in the treatment of vitiligo. However, no histological study has evaluated the repigmentation mechanism of tacrolimus ointment in combination therapy with Nb-UVB. In this study, the histological findings in patients receiving Nb-UVB were compared with those receiving topical tacrolimus combined with Nb-UVB. Twenty patients were recruited and received Nb-UVB treatment. The first ten patients were selected for the combination therapy and instructed to apply tacrolimus 0.1% ointment twice daily on the specified lesion of interest. The remaining ten patients did not receive any other topical treatments. Skin biopsy was performed at baseline from the depigmented area and 2-3 months post-treatment from the repigmented area. Biopsy specimens were stained with haematoxylin-eosin-safran (HES), Fontana Masson, HMB45, Melan A, MITF, SOX10 and Nestin. Clinically, in the combination therapy group, interfollicular repigmentation in addition to the perifollicular and marginal pattern was observed. Histologically, in the combination therapy group, besides the migration of melanocytes from the bulge of the hair follicle seen in the monotherapy group, for the first time, we observed dermal melanocyte precursors located in mid- and superficial dermis.