Frequency of epistaxis and telangiectasia in patients with hereditary hemorrhagic telangiectasia (HHT) in comparison with the general population: Curaçao diagnostic criteria revisited.

PURPOSE: The Curaçao criteria are well-established diagnostic criteria for hereditary hemorrhagic telangiectasia (HHT), but they lack details regarding a predictive presentation of epistaxis and telangiectasias. This study collects and compares data in HHT and population cohorts to inform the application of these criteria. METHODS: In-person interviews regarding epistaxis and targeted examination for telangiectases in a general population cohort (n = 204) and an HHT cohort (n = 432) were conducted. RESULTS: Frequency of epistaxis, rather than intensity or duration, was the best discriminator of HHT. A cutoff of ≥4 nosebleeds per year alone yielded a diagnostic sensitivity of 97%, and specificity of 84%. The mean number of telangiectases at the sites investigated was 0.4 in the general population cohort and 26.5 in the HHT cohort. The most distinctive sites for telangiectases in HHT were lips and palmar fingers, whereas telangiectases of the face and dorsum of the hand were comparable in both cohorts. CONCLUSION: We propose that the Curaçao criteria be modified to include the following cutoffs: (1) epistaxis frequency of ≥4 nosebleeds per year and (2) telangiectasia count of at least 2 in characteristic locations (palmar aspect of fingers, lips, and oral cavity), and that cutaneous telangiectases at other sites not be considered relevant for diagnostic purposes.

Cutaneous findings in patients with chronic venous insufficiency.

BACKGROUND: Venous hypertension causes many different cutaneous findings such as varicosities, telangiectasia, edema, and pigmentation, dermatitis, and venous ulcers on the skin. OBJECTIVE: This study aims to investigate the cutaneous signs and symptoms of chronic venous insufficiency (CVI) and to examine their contribution to early diagnosis. METHODS: A total of 150 patients were included in the study who applied to the dermatology outpatient clinic and were diagnosed with skin disease related to CVI or had skin findings. Patient's age, gender, complaints, occupation, additional diseases, drug usage, history of prolonged standing and travel, smoking habit, number of pregnancies, history of varicose veins in the family, dermatological diagnosis/findings, and venous Doppler ultrasonography reports were examined retrospectively. RESULTS: 56% of patients were women. Mean age was 56.69 ± 13.6 years. Overall, 82.7% of total patients had at least one skin finding. The most frequent skin findings except varicose veins were insufficiency dermatitis accounted for 32.7% of patients, telangiectasia, and pigmentation which were comprised 25.3%, 19.3% of the total number of patients respectively. In addition to this, 48.7% of patients had itching problems and 32.7% had pain. Moreover, 46% of patients presented superficial vein insufficiency, while 8.7% had deep vein insufficiency. For 47.3% of patients, vein diameter dilation was observed and 11.3% suffered from perforating vein insufficiency. In terms of Clinical-Etiologic-Anatomic-Pathophysiologic (CEAP) classification, scores of 52% of the patients were C3 and lower, while scores of 48% of total patients were C4a and higher. CONCLUSIONS: Early diagnosis and treatment of chronic venous insufficiency could prevent further chronic processes such as venous ulceration which is an advanced CVI finding. Thus, assessing the early skin findings might be important to identify the underlying venous insufficiency disease.

Epidemiology of rosacea in a population-based study of 161,269 German employees.

BACKGROUND: Rosacea is a common chronic skin condition, but data on its epidemiology and related comorbidities are scarce. OBJECTIVES: To analyze the prevalence and associated cutaneous comorbidities of rosacea in Germany. METHODS: Voluntary dermatological full-body examinations were conducted between 2001 and 2016 in more than 500 German companies by experienced dermatologists and documented electronically. Point-prevalence rates were calculated, and associations were tested with chi-squared tests and logistic regression analysis. RESULTS: A total of 161,269 participants (mean age was 43.2 ± 10.9 years; 55.5% male) were included; 2.1% had rosacea (men: 2.1%, women 2.1%, mean age 50.7 ± 9.3 years). The prevalence of rosacea increased significantly with age (16-29 years: 0.3%; 30-39 years: 0.9%; 40-49 years: 2.0%; 50-59 years: 3.5%; 60-70 years: 5.7%). Furthermore, there was a significant decreasing prevalence from skin type I toward type IV (skin type I: 3.2%; II: 2.2%; III: 1.5%; IV: 0.4%). The most frequent dermatological comorbidities were: telangiectasia (OR = 2.5), folliculitis (OR = 1.8), seborrheic dermatitis (OR = 1.6), acne (OR = 1.6), tinea pedis (OR = 1.4), psoriasis (OR = 1.4), spider veins (OR = 1.1), and hemangioma (OR = 1.1). CONCLUSIONS: Rosacea is a common skin condition that is most prevalent above the age of 65 years. Rosacea patients have an increased risk for associated comorbidities. Therefore, the diagnostic and therapeutic process for rosacea patients must ensure an integrated, complete dermatological approach in terms of medical care.

Multiple cutaneous telangiectasias after the use of trastuzumab: a case report

We aim to report a particular case of cutaneous telangiectasias on the arms after immunotherapy with trastuzumab plus paclitaxel to treat breast cancer. New oncology therapies reflect a major advance in cancer treatment. They greatly increase survival; however, they still cause certain adverse cutaneous events that should be taken into account for their proper management.

Therapeutic Opportunities for Intestinal Angioectasia- Targeting PPARγ and Oxidative Stress.

Recurrent and acute bleeding from intestinal tract angioectasia (AEC) presents a major challenge for clinical intervention. Current treatments are empiric, with frequent poor clinical outcomes. Improvements in understanding the pathophysiology of these lesions will help guide treatment. Using data from the US Food and Drug Administration (FDA)'s Adverse Event Reporting System (FAERS), we analyzed 12 million patient reports to identify drugs inversely correlated with gastrointestinal bleeding and potentially limiting AEC severity. FAERS analysis revealed that drugs used in patients with diabetes and those targeting PPARγ-related mechanisms were associated with decreased AEC phenotypes (P < 0.0001). Electronic health records (EHRs) at University of Cincinnati Hospital were analyzed to validate FAERS analysis. EHR data showed a 5.6% decrease in risk of AEC and associated phenotypes in patients on PPARγ agonists. Murine knockout models of AEC phenotypes were used to construct a gene-regulatory network of candidate drug targets and pathways, which revealed that wound healing, vasculature development and regulation of oxidative stress were impacted in AEC pathophysiology. Human colonic tissue was examined for expression differences across key pathway proteins, PPARγ, HIF1α, VEGF, and TGFß1. In vitro analysis of human AEC tissues showed lower expression of PPARγ and TGFß1 compared with controls (0.55 ± 0.07 and 0.49 ± 0.05). National Center for Biotechnology Information (NCBI) Gene Expression Omnibus (GEO) RNA-Seq data was analyzed to substantiate human tissue findings. This integrative discovery approach showing altered expression of key genes involved in oxidative stress and injury repair mechanisms presents novel insight into AEC etiology, which will improve targeted mechanistic studies and more optimal medical therapy for AEC.

Weekly radiotherapy in elderly breast cancer patients: a comparison between two hypofractionation schedules.

PURPOSE: Weekly irradiation in breast cancer in elderly patients is a treatment option, whose tolerance may be influenced by the fractionation used. The objective of this study is to compare the tolerance and long-term side effects of two different fractionations. MATERIALS AND METHODS: 47 elderly patients were recruited after conservative or radical treatment that also received irradiation with a dose per fraction of 6.25 Gy or 5 Gy for one session per week, 6 sessions in total. The long-term tolerance results are compared by assessing toxicity using CTCAE version 5.0 scales for dermatitis, telangectasia, fibrosis and pain of the irradiated breast. In addition, objective parameters of skin status (erythema, hyperpigmentation, elasticity and hydration) by a multi-probe MultiSkin Test-Center system were obtained and compared between groups. RESULTS: After an average follow-up of 5 years, all patients were free of disease and with complete local control. A total of 20 patients with 6.25 Gy fractionation and 27 patients with 5 Gy fractionation have been included. Patients treated with lower fractionation had a lower incidence of dermatitis, telangectasia, fibrosis, or local pain. The decrease in elasticity measured by the multi-probe system was smaller with the fractionation of 5 Gy. No differences were observed in the other objective parameters. CONCLUSION: Weekly irradiation with 5 Gy fractionation is better tolerated than with higher fractionation.

A reliable skin toxicity predictor in permanent breast seed implant brachytherapy.

PURPOSE: To establish skin dose-outcome relationships using a reliable metric in permanent breast seed implant (PBSI). METHODS: Sixty-seven consecutive patients who underwent PBSI at our institution were included. Skin doses were calculated using two skin dose indices: maximum point dose to the skin surface, Dmax, and D0.2cc for a 2-mm internal skin rind (a surrogate to the dose to 1 cm2 area of skin) from CT-based postoperative treatment plans. Toxicity data were extracted from patients' charts and photographs. The associations between skin dose and skin toxicity were investigated using the analysis of variance, and the predictive performance of skin dose measures was evaluated using receiver operating characteristic curves. RESULTS: For acute reactions, 49.3% of patients had Grade 1, 4.5% Grade 2, and 1.5% Grade 3 toxicity. For telangiectasia at 3 years, very minor and minimally apparent telangiectasia was observed in 25% of patients. Moderate but asymptomatic telangiectasia was observed in 9.1% of cases. Both metrics were significantly associated with the occurrence of acute toxicity and telangiectasia at 3 years (p < 0.01). The predictive values for Dmax and D0.2cc were 0.779 and 0.763, respectively, (p < 0.0001) for acute skin toxicity and 0.786 and 0.810 for telangiectasia (p < 0.0002). Extreme dose outliers (up to 878 Gy) and a high variability were observed for Dmax but not for D0.2cc, illustrating the superior reliability of D0.2cc. CONCLUSION: D0.2cc, as an alternate skin dose measure to Dmax, is a robust metric for measuring skin dose that is simple to calculate, yet is clinically relevant and not prone to inaccuracies inherent to point dose measurement.

Pulmonary Arteriovenous Malformations in Non-hereditary Hemorrhagic Telangiectasia Patients: An 18-Year Retrospective Study.

PURPOSE: Pulmonary arteriovenous malformations (PAVMs) are most commonly associated with hereditary hemorrhagic telangiectasia (HHT). Patients with PAVMs can present with serious complications including stroke, transient ischemic attack (TIA), and brain abscess. PAVMs are rare in non-HHT patients and little is known about this patient population. The aim of this retrospective study is to better understand clinical presentation and outcomes of PAVMs occurring exclusively in non-HHT patients. METHODS: Non-HHT patients with PAVMs at the Mayo Clinic-Rochester between 01/01/2000 and 12/31/2018 were reviewed. Patients with Curacao score > 1 were excluded. Demographics, imaging characteristics, neurological complications, and follow-up imaging were analyzed. RESULTS: Seventy-seven patients with PAVMs were identified. The mean age at diagnosis was 48.2 ± 18.3 years with female preponderance (59.7%). The majority of PAVMs had lower lobe predominance (66.7%) and were simple and single in 75.3% and 89.6% of cases, respectively. Most patients were asymptomatic (46.8%) with dyspnea being the most common symptom (28.6%). Neurologic complications occurred in 19.5% of patients. The majority of PAVMs were idiopathic (61%). Thirty patients (39%) had one or more possible risk factors including previous thoracic surgery (23.4%), congenital heart disease (19.5%), and chest trauma (10.4%). Embolization was performed in 37 (48.1%) patients and only 4 (5.2%) underwent surgical resection. CONCLUSIONS: Non-HHT PAVMs occur more commonly in females, are most commonly simple and single, and have lower lobe predominance and a high rate of neurologic complications. Potential predisposing risk factors were identified in about 40% of the cases. Clinicians should be aware of the risk of PAVM development in patients with history of chest trauma, congenital heart disease, lung infection/abscess, and thoracic surgery.

Twenty-Year Incidence of Macular Telangiectasia Type 2 and Associated Systemic Comorbidities in Olmsted County, Minnesota.

BACKGROUND AND OBJECTIVE: To identify the incidence and associated systemic and ocular risk factors of macular telangiectasia (MacTel) type 2 using a population-based study. PATIENTS AND METHODS: In this retrospective cohort study, patients with MacTel type 2 were identified using the Rochester Epidemiology Project. An age- and sex-matched 4:1 cohort of healthy control patients was created to identify systemic medical comorbidities, medications, and ophthalmic risk factors with MacTel type 2. RESULTS: Over a 20-year time period, 18 patients with MacTel type 2 were identified. The overall incidence was 0.0007% per year. Adjusting for the age and sex of the Caucasian population in the 2010 U.S. census, the overall incidence was 0.0008% (95% CI, 0.4-1.2) per year. There were significantly more MacTel type 2 patients with type 2 diabetes (P = .007), hyperlipidemia (P = .03), and hypertriglyceridemia (P = .004). CONCLUSION: The incidence of MacTel type 2 in Olmsted County, Minnesota, is 0.7 per 100,000 persons per year. These patients are more likely to have type 2 diabetes and high cholesterol. The use of aspirin and nonsteroidal anti-inflammatory drugs may be protective factors. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:S35-S42.].

The First Case of TEMPI Syndrome in Japan.

TEMPI syndrome, a disease entity comprising telangiectasia, erythrocytosis with high erythropoietin, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting, was first described by Sykes et al. in 2011. To our knowledge, only 15 cases have been reported worldwide, none of which were in Japan. We herein report a 47-year-old man who had intractable ascites for 2 and a half years and was referred to our department for a peritoneovenous shunt. In addition to ascites, he had telangiectasia, high erythropoietin, monoclonal gammopathy, and perinephric fluid collection. Thus, this is the first case of TEMPI syndrome in Japan.

Comparison of clinical presentation and incidence of cardiopulmonary complications between male and female Thai patients with early systemic sclerosis: inception cohort study.

OBJECTIVES: To determine the prevalence of clinical manifestations and incidence rate of cardiopulmonary complications in a comparison between men and women with early SSc. METHODS: An inception cohort of early-SSc patients at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, between January 2010 and June 2016, was used. All patients were assessed for clinical manifestations and underwent ECG, echocardiography, and HRCT at the study entry and then annually. RESULTS: One hundred and fifteen patients (46 male, 90 dcSSc) with a mean (SD) disease duration of 11.6 months (8.8) at cohort entry were enrolled during a mean (SD) observational period of 3.8 years (1.6). At enrollment, the male group had a higher prevalence of dcSSc subtype (91.3% vs. 69.5%, p = 0.006), hypo-hyperpigmentation (84.8% vs. 65.2%, p = 0.021), myositis (26.1% vs. 10.1%, p = 0.024), and right ventricular dysfunction [RVD] (8.7% vs. 0%, p = 0.024) compared with women. At the last visit, the male group had a higher cumulative prevalence of digital ulcers (47.8% vs. 27.5%, p = 0.026), telangiectasia (93.5% vs. 69.6%, p = 0.002), joint contracture (69.6% vs. 43.5%, p = 0.006), tendon friction rub (39.1% vs. 20.3%, p = 0.027), LVEF < 50% (21.7% vs. 8.7%, p = 0.048), and RVD (34.8% vs. 7.2%, p < 0.001). The male group had a significantly higher incidence rate of RVD (8.21 vs. 1.99 per 100 person-years, p = 0.006) and interstitial lung disease [ILD] (65.25 vs. 40.36 per 100 person-years, p = 0.022) compared to women. CONCLUSIONS: In this study cohort, it was found that men with SSc had more severe clinical manifestations and higher incidence rate of RVD and ILD compared to women. Increased awareness of cardiopulmonary complications in men even in early phase of SSc is crucial. KEY POINTS: • Male patients with SSc have more severe disease manifestations compared to women. • Even in the early phase of the disease, men were found to have higher incidence rates of right ventricular dysfunction and interstitial lung disease than women. • Increased awareness regarding cardiopulmonary complications in men with early SSc is crucial for effective management of these complications.

Cutaneous sequelae in neonatal lupus: A retrospective cohort study.

BACKGROUND: Cutaneous eruptions in neonatal lupus erythematosus (NLE) are thought to be self-resolving. Limited literature suggests cutaneous changes may persist. OBJECTIVE: To characterize cutaneous residua in NLE and identify predictors for their development. METHODS: A retrospective cohort study of patients with cutaneous NLE born between January 1980 and May 2017 was performed. Primary outcome was the proportion of patients with cutaneous residua. Secondary outcomes included associations/predictors of sequelae. RESULTS: At the last follow-up, at a mean age of 4 years (range, 0.5-18.7 years), 34% of 106 patients had cutaneous sequelae, 13% had telangiectasia, 17% had dyspigmentation, and 9% had atrophic scarring. Scarring at the last follow-up was significantly associated with the presence of skin lesions at birth (P < .001). LIMITATIONS: This study was limited by the retrospective design, short follow-up duration in a subset of patients, and small sample size. CONCLUSION: Cutaneous NLE can exhibit long-term cutaneous residua. These findings underlie the importance of accurate diagnosis, long-term monitoring, and appropriate counseling.

Epidemiology and determinants of facial telangiectasia: a cross-sectional study.

BACKGROUND: Telangiectasia or red veins are one of the prominent features of facial skin ageing. To date, there are few studies investigating the determinants of telangiectasia. OBJECTIVES: We investigated lifestyle and physiological factors associated with facial telangiectasia in a large prospective Dutch cohort study. METHODS: Telangiectasia was quantified digitally from standardized facial photographs of 2842 North European participants (56.8% female, median age 66.9) from the Rotterdam Study, collected in 2010-2013. Effect estimates from multivariable linear regressions are presented as the percentage difference in the mean value of telangiectasia area per unit increase of a determinant (%Δ) with corresponding 95% CI. RESULTS: Significant determinants were older age [1.7%Δ per year (95% CI 1.4, 2.0)], female sex [18.3%Δ (95% CI 13.2, 23.6)], smoking [current versus never 38.4%Δ (95% CI 30.3, 47.0); former versus never 11.6%Δ (95% CI 6.6, 16.9)], a high susceptibility to sunburn [10.2%Δ (95% CI 5.4, 15.3)] and light skin colour [pale versus white-to-olive 31.4%Δ (95% CI 19.7, 44.1]; white vs. white-to-olive 9.2%Δ (95% CI 2.8, 16.0)]. CONCLUSIONS: In this large cohort study, we confirmed known and described new determinants of facial telangiectasia.

Atrophic and hypertrophic photoaging: Clinical, histologic, and molecular features of 2 distinct phenotypes of photoaged skin.

BACKGROUND: Exposure to the sun causes premature skin aging, known as photoaging. Clinical features of photoaging vary widely among individuals. In one form, skin appears thin with telangiectasia, and in another form, skin appears thickened with coarse wrinkles. Etiologic, clinical, and therapeutic distinctions among different forms of photoaging remain largely unknown. OBJECTIVE: To characterize the clinical, histologic, and molecular features of hypertrophic and atrophic photoaging. METHODS: In total, 53 individuals were clinically classified as having primarily atrophic or hypertrophic photoaging or neither (controls). Participants' demographic and sun exposure-related lifestyle data were captured by questionnaire. Fifteen clinical features of participants were qualitatively or quantitively scored. Facial biopsies were analyzed for gene expression and histologic characteristics. RESULTS: Actinic and seborrheic keratosis, telangiectasia, and prior incidence of skin cancers were statistically significantly greater and photoaging scale severity, coarse wrinkles, thickness, and sallowness were significantly reduced in atrophic versus hypertrophic groups. Histology also revealed significantly less elastotic material in atrophic photoaging. Gene expression of matrix metalloproteinases and collagens did not differ between the 2 forms of photoaging. LIMITATIONS: The study was not designed to identify other possible subtypes of photoaging. CONCLUSION: Systematic, categorical, and quantitative clinical and histologic assessments distinguish atrophic and hypertrophic photoaging.

Clinical and radiological outcomes of infantile hemangioma treated with oral propranolol: A long-term follow-up study.

Infantile hemangiomas (IH) undergo rapid growth during early infancy followed by gradual involution. After involution, a part of IH remain as residual lesions. Since 2008, oral propranolol has been widely used in the treatment of IH. However, long-term outcome of IH treated with propranolol remains unknown. This study aimed to investigate the sequelae of IH treated with propranolol. In this study, propranolol was given at a dose of 2 mg/kg per day at the age of 3.8 ± 2.5 months and follow-up visits were arranged to continue at least through the age of 4 years. Types of sequela were recorded and classified as four degrees ("none", "minimal", "significant" and "severe" at last visit), then subsequent therapy was evaluated with the help of magnetic resonance imaging (MRI). A total of 73 patients with complete follow up were enrolled in the study. The most common types of sequela were telangiectasia, fibrofatty tissue and erythema. Significant and severe sequelae were observed in 72.4% of treated IH; superficial IH led to more but not significantly significant and severe sequelae than mixed IH (P > 0.05). Despite propranolol treatment, surgery was still needed in 37.5% of IH at a mean age of 70.3 months, and for the main reason of surgery, fibrofatty or hemangioma residua, MRI was useful for us to choose an appropriate surgical procedure.

Autoantibody profile and clinical patterns in 619 Italian patients with cutaneous lupus erythematosus.

BACKGROUND: Anti-nuclear antibodies (ANA), anti-extractable nuclear antigens (ENA) and anti-dsDNA antibodies are often associated with cutaneous lupus erythematosus (CLE), with variable frequency depending on skin subtype. However, specific data based on large case-series on the pathogenetic, diagnostic and prognostic meaning of such autoantibodies are still lacking. OBJECTIVE: To characterize the correlations between CLE subtypes as well as LE-non-specific skin lesions and their autoantibody pattern. METHODS: Epidemiological, clinical and immunopathological data of 619 Italian patients with CLE and LE-non-specific skin lesions were analysed. Differences in age, sex, clinical features and autoantibody profile were evaluated in each LE subgroup. RESULTS: Anti-nuclear antibodies (P < 0.0001), anti-dsDNA (P < 0.0001), ENA (P = 0.001), anti-Sm (P = 0.001), anti-RNP (P = 0.004) and anti-histone (P = 0.005) antibodies were associated with SLE. A strong association between ANA (P < 0.0001) and anti-dsDNA (P < 0.0001) and female gender was also found: positive ANA and positive anti-dsDNA had a higher prevalence among females. Chronic CLE resulted to be negatively associated with ENA (OR = 0.51, P < 0.0001), anti-Ro/SSA (OR = 0.49, P < 0.0001) and anti-dsDNA (OR = 0.37, P < 0.0001). Intermittent CLE resulted to be negatively associated with ENA (OR = 0.50, P = 0.007) and ANA (OR = 0.61, P = 0.025). Subacute CLE resulted to be associated with ENA (OR = 5.19, P < 0.0001), anti-Ro/SSA (OR = 3.83, P < 0.0001), anti-Smith (OR = 2.95, P = 0.004) and anti-RNP (OR = 3.18, P = 0.007). Acute CLE resulted to be strongly associated with anti-dsDNA (OR = 6.0, P < 0.0001) and ANA (OR = 18.1, P < 0.0001). LE-non-specific skin lesions resulted to be significantly associated with systemic involvement. Livedo reticularis was significantly associated with ENA (P = 0.007) and anti-Ro/SSA (P = 0.036). Palpable purpura and periungual telangiectasia were significantly associated with ANA. CONCLUSION: According to our findings, some well-known associations between CLE subtypes and autoantibody profile were confirmed; moreover, specific association between autoantibodies and LE-non-specific skin lesions was highlighted. A strict association between anti-ENA and anti-Ro/SSA antibodies and livedo reticularis, ANA and palpable purpura, and ANA and periungual telangiectasia was evidenced.

Telangiectatic Matting is Associated with Hypersensitivity and a Bleeding Tendency.

OBJECTIVE: The aim was to investigate the pathogenesis of telangiectatic matting (TM) and identify possible risk factors. METHODS: This study had two parts. The clinical records of consecutive patients were retrospectively analysed to identify risk factors for TM. In the second part, the haemostatic and coagulation profile of the subset of patients with TM were analysed and compared with controls using standard coagulation tests, platelet function and a global assay of coagulation (rotational thromboelastometry, ROTEM). RESULTS: In 352 consecutive patients presenting to a phlebology practice, 25 patients had TM (7.1%). All 25 patients were female with the median age of 45 (27-57) years. A comprehensive medical history was taken. Among 27 possible risk factors assessed, statistically significant associations included recurrent epistaxis, easy bruising, hypersensitivity (eczema, hives, hay fever, and rhinitis), previous treatment with sclerotherapy or endovenous laser for lower limb veins, and a family history of telangiectasias. Variables not associated with TM included oral contraceptive intake, hormone replacement therapy, and age. The haemostatic and coagulation profile of 12 patients (6 male and 6 female) with TM did not differ significantly from those without TM. CONCLUSION: TM is associated with both hypersensitivity and a bleeding tendency. This study revealed no significant increase in the incidence of haemostatic abnormalities in patients with TM compared with the control group. Given the significant association with hypersensitivity disorders, the underlying mast cell hyper-reactivity may contribute to both hypersensitivity and a bleeding tendency and predispose patients to TM.

Wilms tumor screening in diffuse capillary malformation with overgrowth and macrocephaly-capillary malformation: A retrospective study.

BACKGROUND: CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies) syndrome is associated with regional bony and/or soft tissue overgrowth, capillary malformation, and an increased risk for Wilms tumor. OBJECTIVE: To evaluate the frequency of Wilms tumor in patients with 2 similar conditions: diffuse capillary malformation with overgrowth (DCMO) and macrocephaly-capillary malformation (M-CM). METHODS: Culling our Vascular Anomalies Center database, we retrospectively reviewed patients in whom DCMO and M-CM had been diagnosed and who were evaluated between 1998 and 2016 for possible development of Wilms tumor. Patients younger than 8 years of age at their last visit and not seen in more than 2 years were contacted for follow-up. RESULTS: The study comprised 89 patients: 67 with DCMO, 17 with M-CM, and 5 with an indeterminate diagnosis. No case of Wilms tumor was found in these groups. LIMITATIONS: Some patients were younger than 8 years of age at last follow-up visit and the sample size was small. CONCLUSION: Patients with DCMO do not appear to be at increased risk for Wilms tumor. Screening is probably unnecessary in DCMO unless there is associated hemihypertrophy. Although there were no cases in our cohort, there are 2 reports of M-CM associated with Wilms tumor in the literature.

Dermatological comorbidity in psoriasis: results from a large-scale cohort of employees.

The field of dermatological comorbidity in psoriasis is only passively explored with contradictory results. Objective of this study was to further investigate the complex field of psoriasis and associated skin diseases by identifying skin comorbidity patterns in an extensive cohort of employees in Germany. Retrospective analysis of data deriving from occupational skin cancer screenings was conducted. From 2001 to 2014 German employees between 16 and 70 years from different branches underwent single whole-body screenings by trained dermatologists in their companies. All dermatological findings and need for treatment were documented. Point prevalence rates and their 95% confidence intervals were computed. Logistic regression analysis was performed to calculate odds ratios (OR) of single dermatological diseases to occur together with psoriasis controlled for age and sex. Data from 138,930 persons (56.5% male, mean age 43.2) were evaluated. Psoriasis point prevalence was 2.0%. Of those 20.6% had unmet treatment needs of their disease. Onychomycosis was the most frequent dermatological comorbidity with a prevalence of 7.8%. Regression analysis found rosacea (OR = 1.40, 95% CI 1.13-1.72) and telangiectasia (OR = 1.25, 95% CI 1.10-1.41) to be significantly associated with psoriasis. 17.2% of psoriasis patients had at least one further finding requiring treatment. The highest treatment needs were found for onychomycosis (3.4%), tinea pedis (3.1%), and verruca plantaris (1.0%). It can be concluded that persons with psoriasis are at increased risk to suffer from comorbid skin diseases, which should be considered in treatment regimens. Particular attention should be paid to fungal diseases of the feet.