Pulmonary embolism versus pulmonary vasculitis in Hughes-Stovin syndrome: Characteristic computed tomography pulmonary angiographic findings and diagnostic and therapeutic implications. HSS International Study Group.

BACKGROUND AND AIM: Hughes-Stovin syndrome (HSS) is a rare systemic vasculitis with widespread venous/arterial thrombosis and pulmonary vasculitis. Distinguishing between pulmonary embolism (PE) and in-situ thrombosis in the early stages of HSS is challenging. The aim of the study is to compare clinical, laboratory, and computed tomography pulmonary angiography (CTPA) characteristics in patients diagnosed with PE versus those with HSS. METHODS: This retrospective study included 40 HSS patients with complete CTPA studies available, previously published by the HSS study group, and 50 patients diagnosed with PE from a single center. Demographics, clinical and laboratory findings, vascular thrombotic events, were compared between both groups. The CTPA findings were reviewed, with emphasis on the distribution, adherence to the mural wall, pulmonary infarction, ground glass opacification, and intra-alveolar hemorrhage. Pulmonary artery aneurysms (PAAs) in HSS were assessed and classified. RESULTS: The mean age of HSS patients was 35 ± 12.3 years, in PE 58.4 ± 17 (p < 0.0001). Among PE 39(78 %) had co-morbidities, among HSS none. In contrast to PE, in HSS both major venous and arterial thrombotic events are seen.. Various patterns of PAAs were observed in the HSS group, which were entirely absent in PE. Parenchymal hemorrhage was also more frequent in HSS compared to PE (P < 0.001). CONCLUSION: Major vascular thrombosis with arterial aneurysms formation are characteristic of HSS. PE typically appear loosely-adherent and mobile whereas "in-situ thrombosis" seen in HSS is tightly-adherent to the mural wall. Mural wall enhancement and PAAs are distinctive pulmonary findings in HSS. The latter findings have significant therapeutic ramifications.

Pulmonary vasculitis in Hughes-Stovin syndrome (HSS): a reference atlas and computed tomography pulmonary angiography guide-a report by the HSS International Study Group.

INTRODUCTION: Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread venous/arterial thrombosis and pulmonary artery aneurysms (PAAs), which is associated with serious morbidity and mortality. All fatalities reported in HSS resulted from unpredictable fatal suffocating hemoptysis. Therefore, it is necessary to recognize pulmonary complications at an early stage of the disease. OBJECTIVES: The aims of this study are to develop a reference atlas of images depicting the characteristic features of HSS by computed tomography pulmonary angiography (CTPA). To make a guide for physicians by developing a classification of PAAs according to the severity and risk of complications associated with each distinct lesion type. METHODS: The Members of the HSS International Study Group (HSSISG) collected 42 cases, with high-quality CTPA images in one radiology station and made reconstructions from the source images. These detailed CTPA studies were reviewed for final image selection and approved by HSSISG board members. We classified these findings according to the clinical course of the patients. RESULTS: This atlas describes the CTPA images that best define the wide spectrum of pulmonary vasculitis observed in HSS. Pulmonary aneurysms were classified into six radiographic patterns: from true stable PAA with adherent in-situ thrombosis to unstable leaking PAA, BAA and/or PAP with loss of aneurysmal wall definition (most prone to rupture), also CTPA images demonstrating right ventricular strain and intracardiac thrombosis. CONCLUSION: The HSSISG reference atlas is a guide for physicians regarding the CTPA radiological findings, essential for early diagnosis and management of HSS-related pulmonary vasculitis. Key Points • The Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by extensive vascular thrombosis and pulmonary artery aneurysms (PAAs) that can lead to significant morbidity and mortality. • All fatalities reported in HSS were related to unpredictable massive hemoptysis; therefore, it is critical to recognize pulmonary complications at an early stage of the disease. • The HSS International Study Group reference atlas  classifies pulmonary vasculitis in HSS at 6 different stages of the disease process and defines the different radiological patterns of pulmonary vasculitis notably pulmonary artery aneurysms, as detected by computed tomography pulmonary angiography (CTPA). • The main aim of the classification is to make a guide for physicians about this rare syndrome. Such a scheme has never been reached before since the first description of the syndrome by Hughes and Stovin since 1959. This classification will form the basis for future recommendations regarding diagnosis and treatment of this syndrome.

A critical analysis of 57 cases of Hughes-Stovin syndrome (HSS). A report by the HSS International Study Group (HSSISG).

BACKGROUND: Hughes-Stovin syndrome (HSS) is a systemic disease characterized by widespread vascular thrombosis and pulmonary vasculitis with serious morbidity and mortality. The HSS International Study Group is a multidisciplinary taskforce aiming to study HSS, in order to generate consensus recommendations regarding diagnosis and treatment. METHODS: We included 57 published cases of HSS (43 males) and collected data regarding: clinical presentation, associated complications, hemoptysis severity, laboratory and computed tomography pulmonary angiography (CTPA) findings, treatment modalities and cause of death. RESULTS: At initial presentation, DVT was observed in 29(33.3 %), thrombophlebitis in 3(5.3%), hemoptysis in 24(42.1%), and diplopia and seizures in 1 patient each. During the course of disease, DVT occurred in 48(84.2%) patients, and superficial thrombophlebitis was observed in 29(50.9%). Hemoptysis occurred in 53(93.0%) patients and was fatal in 12(21.1%). Pulmonary artery (PA) aneurysms (PAAs) were bilateral in 53(93%) patients. PAA were located within the main PA in 11(19.3%), lobar in 50(87.7%), interlobar in 13(22.8%) and segmental in 42(73.7%). Fatal outcomes were more common in patients with inferior vena cava thrombosis (p = 0.039) and ruptured PAAs (p < 0.001). Death was less common in patients treated with corticosteroids (p < 0.001), cyclophosphamide (p < 0.008), azathioprine (p < 0.008), combined immune modulators (p < 0.001). No patients had uveitis; 6(10.5%) had genital ulcers and 11(19.3%) had oral ulcers. CONCLUSIONS: HSS may lead to serious morbidity and mortality if left untreated. PAAs, adherent in-situ thrombosis and aneurysmal wall enhancement are characteristic CTPA signs of HSS pulmonary vasculitis. Combined immune modulators contribute to favorable outcomes.

Does influenza subtype H1N1 have a place in the etiology of pityriasis rosea?

INTRODUCTION: Pityriasis rosea (PR) is an acute, inflammatory skin disease of unknown cause. Various infectious agents including viruses have been proposed as causative agents and presence of influenza subtype H1N1 was shown in case reports with PR, but the relation was not conclusive. We hypothesited that there may be a relation between PR and H1N1, since both of them are prevalent in the same period as winter or season transitions. AIM: To investigate the effect of the H1N1 virus in PR in this study. MATERIAL AND METHODS: Twenty-one female and 12 male PR patients who applied to Kirikkale University Faculty of Medicine Hospital Dermatology Outpatient Clinic were included in the study. Influenza subtype H1N1 IgM and IgG antibodies were detected by enzyme immunoassay (EIA) in sera of patients; tissue biopsy specimens were examined for influenza subtype H1N1 RNA by PCR. RESULTS: Seven (23%) of the 33 patients had positive IgM and IgG antibodies. Influenza subtype H1N1 RNA was not detected in the tissue samples of 33 PR patients. CONCLUSIONS: According to the results of this study, we can say that influenza subtype H1N1 does not play a role in PR etiology.

CAG polymorphism in the androgen receptor gene in women may be associated with nodulocystic acne.

INTRODUCTION: Acne vulgaris (AV) is a multifactorial, inflammatory disease of the pilosebaceous unit. Hormones play a major role in the pathogenesis of acne. In cases of hyperandrogenism; hirsutism, acne, seborrhoea and alopecia appear in women. However, severe acne can also be seen without evidence of hyperandrogenism. In this case, hypersensitivity of the androgen receptor gene (ARG) encoded in the X chromosome, which is the only receptor for androgens, can be considered. ARG contains a polymorphic CAG triple loop encoding the polyglutamine pathway at the 5'end of exon 1. AIM: To investigate CAG repeat polymorphism in the ARG in nodulocystic acne patients in Turkish population. MATERIAL AND METHODS: This prospective clinical study was conducted between 2016 and 2017 in accordance with the tenets of the Declaration of Helsinki. DNA isolation from blood was performed using the RTA® Genomic DNA Isolation Kit. The fragment lengths obtained from the device to determine CAG repeat numbers were analysed based on -288 bp length 22 CAG repeat content. RESULTS: A total of 199 subjects; 100 patients (51 males, 49 females) and 99 controls (49 males, 50 females) were included in the study. The mean allele length in the patient group was 19.34; and 19.7 in the control group. There was a statistically significant difference between female patients and the control group, when the patients and control groups were compared by gender (p = 0.0059). CONCLUSIONS: The CAG trinucleotide repeat count in the ARG may be associated with acne, without hirsutism findings.

Clinical significance of prognostic nutritional index (PNI) in malignant melanoma.

PURPOSE: Nutrition and inflammation play a crucial role in the development of cancer. The prognostic value of the prognostic nutritional index (PNI) has been confirmed in some types of human cancers. However, few studies are available indicating its prognostic power in patients with malignant melanoma (MM). Thus, we aimed to identify baseline peripheral blood biomarkers to predict the outcome of MM patients MATERIAL AND METHODS: Data of 101 patients with MM were evaluated retrospectively. Associations between clinical and histopathological parameters with overall survival (OS) and progression-free survival (PFS) were analyzed using Kaplan-Meier curves and compared by the log-rank test. The optimal cutoff values were determined by a receiver operating characteristic (ROC) curve analysis. Neutrophil-lymphocyte ratio (NLR), systemic immune-inflammation index (SII) and PNI were grouped based on a cutoff points 2.18, 547.1, and 40.1, respectively. Univariate and multivariate analyses were used to assess their prognostic values for overall survival (OS). RESULTS: Lower NLR ( < 2.18), SII ( < 547.1) and higher PNI ( ≥ 40.1) were linked with a longer PFS and OS in patients with MM, as reflected in the Kaplan-Meier analyses. In univariate analysis, TNM stage, Breslow thickness, Clark stage, ulceration, Ki67 status, LDH, NLR, SII, and PNI were significantly associated with OS. Multivariate analysis identified TNM stage, ulceration, LDH and PNI as an independent predictor of OS in patients with MM. CONCLUSION: PNI can be regarded as a novel independent prognostic factor for predicting OS in MM.

Treatment of multiple eccrine hidrocystoma with oral isotretinoin following erbium-yttrium aluminum garnet laser.

Eccrine hidrocystomes (EH) are benign cystic tumors of dermal eccrine ducts. There is still no gold standard treatment option for EH because of the large number of lesions, the risk of cicatrization and the different treatment options offered. We offer combined oral isotretinoin and following erbium-yttrium aluminum garnet laser treatment as an alternative treatment option in difficult patients with EH.

Anti-inflammatory activity of Anthemis tricolor Boiss.

INTRODUCTION: Traditionally, Anthemis genus has been used for the treatment of gastrointestinal disorders, haemorrhoid and stomach ache in Europe. There are several in-vitro and in-vivo studies that showed the pharmacological properties of Anthemisspecies such as antibacterial, anti-inflammatory, antiedemic and hepatoprotective properties. AIM: Our study referred to the model of inflammation formed in rats; we aimed to evaluate anti-inflammatory properties of A. tricolor extracts. MATERIAL AND METHODS: Five different extracts of A. tricolor were tested against the negative control and 2 different topical corticosteroids (betamethasone and hydrocortisone); n-hexane and sesquiterpene extracts of A. tricolorBois showed significantly reduced erythema compared to the negative control. RESULTS: Only n-hexane and sesquiterpene extracts of A. tricolor have the anti-inflammatory effect. CONCLUSIONS: This is the first report on anti-inflammatory activity of A. tricolor and we showed an anti-inflammatory effect of n-hexane and sesquiterpene extract of A. tricolor with UV erythema test as a result, hence A. tricolor extracts can be used for the treatment of inflammatory skin diseases due to anti-inflammatory activity.

Rationale for the autologous serum skin test in acute versus chronic urticaria.

INTRODUCTION: Autologous serum skin test (ASST) is a rapid, in-vivo clinical test to detect functional autoantibodies in patients with chronic spontaneous urticaria (CSU), but the rationale for its use in acute urticaria (AU) is unknown. AIM: To evaluate the efficacy of ASST among patients with AU or CSU. MATERIAL AND METHODS: Treatment-naïve adult (age ≥ 18 years) patients with a diagnosis of AU (< 6 weeks' duration) and CSU were enrolled prospectively in a cross-sectional study. Healthy age- and sex-matched subjects served as controls. Besides a detailed history and physical examination, ASST, total immunoglobulin E (IgE), freeT3 (fT3), freeT4 (fT4), anti-thyroglobulin, and anti-TPO levels were assessed in all subjects. RESULTS: Of 101 subjects, mean age was 34.35 ±12.68 years and the study comprised 58.4% of females with no difference between AU (n = 27), CSU (n = 46), and control groups (n = 28). The ratio of positivity in ASST was similar between AU (25.9%) and CSU groups (21.7%), but higher than in controls (10.7%, p = 0.33 for all). The ratio of patients with high total IgE levels (> 100 IU/ml) in AU (85.2%) and CSU (65.2%) groups was similar (p = 0.06), but significantly higher than in the control group (10.7%) (p< 0.001 and p< 0.001). The CSU group had significantly higher abnormal thyroid test results (45.7%) than AU (14.8%) and control groups (3.6%) (p = 0.01 and p< 0.001), whereas patients with clinically diagnosed thyroiditis were only in the CSU group (6.5%). In logistic regression analysis, there was no relation found among the possible risk factors for ASST, even if analysed separately as AU, CSU and control groups. CONCLUSIONS: Even though thyroid function test levels were found to be related with CSU, and total IgE was associated with urticaria, ASST was found to be of importance. This study confirms that ASST was insufficient to demonstrate autoimmunity and acute-chronic urticaria nature. Further tests indicating auto-antibodies in AU and CSU are needed.

Decreased choroidal thickness in vitiligo patients.

BACKGROUND: Vitiligo is a disease characterized by depigmented macules and patches that occur as a result of the loss of functional melanocytes from the affected skin through a mechanism which has not been elucidated yet. Destruction of pigment cells in vitiligo may not remain limited to the skin; the eyelashes, iris, ciliary body, choroid, retinal pigment epithelium and meninges may also be affected. This study aims to compare the choroidal thickness of patients with and without vitiligo using optical coherence tomography (OCT). METHODS: Spectral-domain optical coherence tomography (SD-OCT) (Retina Scan Advanced RS-3000 NIDEK, Japan) instrument (with λ = 840 nm, 27,000 A-scans/second and 5 µm axial resolution) was used for the imaging. Statistical analysis was performed using SPSS 21.0 software package. RESULTS: In all values except optic nevre area measurements, the choroidal thickness of all vitiligo patients was found out to be thinner compared to the control group. CONCLUSIONS: In vitiligo, the choroidal thickness may be affected by the loss of melanocytes.

Hughes-Stovin Syndrome as an Outcome of Behçet Disease or as a Different Entity.

Hughes-Stovin syndrome is a rare disorder of unknown etiology. Although the association between multiple pulmonary artery aneurysms and venous thrombosis of the lower limbs was reported by Beattie and Hall in 1911, it was not until 1962 that the eponym "Hughes-Stovin syndrome" was formally introduced in the medical literature. We describe 2 patients with Hughes-Stovin syndrome who presented with pulmonary artery aneurysm, thrombophlebitis, hemoptysis, and oral ulcers, review the manifestations of the disease, and compare its similarities with and differences from Behçet disease.

Macroglossia as a presenting feature of multiple myeloma.

Macroglossia has been very rarely reported as a first clinical sign of multiple myeloma.

Retrospective Analysis of Endemic Melasma Patients.

Melasma is an acquired diffuse hypermelanosis characterized by localized, symmetrical, irregular, light-to-dark brown maculae occurring in sun-exposed areas of skin. The aim of this retrospective study was to determine demographics of patients, analysis of etiologic factors, clinical features, efficacy and side effects of available topical treatments due to high incidence of melasma patients. In this study melasma patients in Birecik State Hospital were investigated retrospectively. Between January 2014 and October 2015, 1008 patients had diagnosis of melasma in 49,809 applications of 24,603 different patients who admitted to Dermatology Outpatient Clinics. Of the 1008 patients, 263 had completed 3-month treatment period. These patients did not receive treatment in June, July, August and September. All melasma patients were rural and dealing with agriculture. There was no significant difference between female and male patients in terms of age. Of the 253 female melasma patients, only 2 of them had not child and none of them were using hormone drug. Of the 263 patients with melasma, Fitzpatrick skin type was 3 in 79 (30%) patients, 4 in 184 (70%) patients. Sanliurfa city showed higher fertility rate, sun exposure, and skin type than Turkey as a whole. These predisposing factors may explain higher melasma occurrence in Sanliurfa. Patient information about preventive measures and treatment play important role in treatment of cosmetic condition. The most important measure seems to advise patients about sun-protection especially during pregnancy.

Evaluation of Optic Nerve with Strain and Shear Wave Elastography in Patients with Behçet's Disease and Healthy Subjects.

The objective of this study was to investigate the elasticity characteristics of the optic nerve using strain and shear wave elastography in patients with Behçet's disease and to compare the results with those of healthy volunteers. Forty-six optic nerves from patients with Behçet's disease and 54 optic nerves from healthy volunteers were investigated prospectively in this study using strain and shear wave elastography. There was a statistically significant difference in terms of elasticity patterns between patients and healthy volunteers (p < 0.001). Elastographic images of healthy volunteers revealed most optic nerves to be type 3 (51.8%); however, type 2 (40.7%) and type 1 (7.5%) were also observed. Elastographic examination of Behçet's disease patients revealed type 2 in 52.2%, type 1 in 43.5% and type 3 in 4.3% of patients. Statistically significant differences were observed between patients and healthy volunteers in the analysis of shear wave elastography values (p < 0.001). Receiver operating characteristic curve analysis was perfect (0.933) (95% CI = 0.885-0.980), and a cutoff value of 16.5 kPa shear had very high sensitivity and specificity for the patient group. Strain and shear wave elastography findings for the optic nerves of patients with Behçet's disease were significantly different from those for healthy volunteers.

The investigation of antimicrobial peptides expression and its related interaction with methotrexate treatment in patients with psoriasis vulgaris.

BACKGROUND: Psoriasis is a chronic, inflammatory and immune-mediated disease. Recently, the role of antimicrobial peptides (AMPs) such as human beta defensins (hBDs) in the pathogenesis of psoriasis has been investigated. We aimed to evaluate the expression profiles of hBD-1 and hBD-2 in psoriatic skin before and after methotrexate (MTX) therapy and to compare healthy controls. METHODS: Immunohistochemical expressions of hBD-1 and hBD-2 were assessed in 16 patients with psoriasis vulgaris and 20 normal skin biopsies from healthy controls. The patients were administered a 12 week of MTX and skin biopsy samples were obtained from the lesional skin of the patients pre-/posttreatment and normal body of the healthy controls. RESULTS: The median (range) Psoriasis Area and Severity Index (PASI) value was 21.6 (8.2-27.7) before the treatment whereas; 3.05 (1-23.4) after the treatment. hBD-1 expression in psoriasis patients was significantly higher as compared to the healthy controls before treatment (p < 0.01). No significant difference was observed between psoriasis patients and healthy controls in terms of hBD-2 expression before treatment (p > 0.05). No significant difference was observed between before-after MTX treatment in terms of hBD-1 and hBD-2 expression levels in psoriasis patients (p > 0.05). CONCLUSIONS: These findings suggest a role for hBD-1 in psoriasis pathogenesis. But MTX treatment does not affect on hBD-1 and hBD-2 expressions. Further studies are needed to assess the roles of these AMPs in psoriasis etiopathogenesis.

Management of a Recurrent Pyogenic Granuloma of the Inferior Lip with Pulsed Dye Laser: A Case Report.

Pyogenic granuloma (PG) is a common, acquired, benign vascular reactive proliferation. This article presents the unique and successful pulsed dye laser treatment of a labial PG on a 60-year-old patient.

Signet-ring Cells in the Skin: A Case of Late-onset Cutaneous Metastasis of Gastric Carcinoma and a Brief Review of Histological Approach.

Up to 10% of patients with visceral malignancies develop skin metastases during their clinical course and these metastases constitute about 2% of all skin cancers. Skin metastasis may be the first sign of a clinically silent visceral cancer or represent recurrence of an internal malignancy. In both situations, they are associated with poor prognosis, which can partly be attributed to underdiagnosis. In this paper, a case of relapsing gastric adenocarcinoma, which manifested itself as asymptomatic cutaneous papules and nodules on a patient's head and neck, is reported and histopathological approach to the cutaneous lesions containing signet-ring cell is briefly reviewed.