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Dermatology for the pediatric skin of color population is the application of dermatology to the genetically diverse and distinctive segment of the pediatric population that includes children of non‐White racial and ethnic groups with increased pigmentation including individuals of Asian, LatinX, African, Native American, Pacific Island descent, Indigenous Peoples, among others, with overlap in particular individuals, and mixtures thereof. Treating children of color is a unique skill set within the field of pediatric dermatology, requiring knowledge and sensitivity. The discipline of pediatric skin of color can be challenging. Difficulty in diagnosis of common conditions stems from underlying pigmentation, variations in common hairstyling practices, and differences in demographics of cutaneous disease, whereas some conditions are more common in children of color, other conditions have nuances in clinical appearance and/or therapeutics with regard to skin color. This article is the first in a series of two articles looking at recently published skin‐related issues of high concern in children of color. Conditions reviewed in Part 1 include (1) hairstyling hair‐related concerns (traction alopecia, central centrifugal cicatricial alopecia, endocrine disruption), (2) autoimmune concerns (cutaneous lupus, vitiligo), and (3) infections (tinea capitis, progressive macular hypomelanosis). [ABSTRACT FROM AUTHOR]

Background/Objectives: Clinically amyopathic juvenile dermatomyositis (CAJDM) is an uncommon but important subset of patients with juvenile dermatomyositis, characterized by pathognomonic cutaneous findings without clinically evident muscle weakness. With limited data available and lack of standardized management guidelines for CAJDM, we sought to describe common features, including early indicators that may be associated with progression of muscle disease, and review the course and treatment of these patients. Methods: A retrospective chart review of patients with CAJDM was conducted at four North American academic centers between the years 2000 and 2015. Results: Twenty‐nine patients were included, of whom 21 (72%) were female. After a median follow‐up of 4 years (IQR 1.8‐5.8 years), 5 of the 29 (17%) patients with CAJDM evolved into classic juvenile dermatomyositis. Median time to develop weakness was 12 months (IQR 8‐19 months) after diagnosis. The skin disease of CAJDM patients who did not develop weakness was often found to be recalcitrant with 58% of them requiring multiple systemic therapies to control their cutaneous disease. Conclusion: These results highlight the need for long‐term monitoring for the development of myositis in CAJDM and for prospective studies on treatment of recalcitrant skin disease. [ABSTRACT FROM AUTHOR]

Background: Collagen vascular disorders (CVDs) are autoimmune disorders with multisystem involvement. Clinical liver involvement is not a characteristic feature though histological involvement could be frequent. Liver disease in CVDs could be the consequence of various factors. Aim: The aim was to analyze the histological spectrum of liver in collagen vascular disorders (CVDs) at autopsy. Materials and Methods: Thirty-six autopsy livers negative for hepatitis B or C virus were studied in CVD cases with no known association with chronic liver disease or vascular thrombosis or hematological disorder. Cirrhotic and normal livers were used as controls. The paired t-test, one-way ANOVA, and two-sided Dunnett t-test were used for comparison (< 0.05). None of the control cases showed any abnormal vessels. Results: There were 21 systemic lupus erythematosus (SLE), 7 rheumatoid arthritis (RA), 5 systemic sclerosis (SSc), and 3 polyarteritis nodosa (PAN) cases (M:F = 11:25, age range 23-60 years). Histology: Diffuse nodular regenerative hyperplasia of liver (NRHL) was seen in 10 cases, and 6 (5 SLE and 1 RA) had numerous abnormal thin-walled vessels in intermediate- and small-sized portal tracts with no vascular occlusion or inflammation. Moderate sized portal tracts showed more interface and lobular inflammation. The main portal vein and its major branches were normal. None of these six cases had increased transmainases (P>0.05). Most SLE cases had increased transaminases (P

Background: For a number of decades, hairy cell leukemia (HCL) has been linked with polyarthritis, vasculitis, Symptomatic cytopenias and thrombosis in the medical literature. Notwithstanding, the significance of these associations has not been well understood. Therefore, we have decided to analyze them further. Methods: We provide herein a comprehensive literature review of the prevalence of autoimmune disorders in patients with HCL. Most relevant publications were identified through searching the PubMed/Medline database for articles published from inception to February 2014. Findings: Perhaps due to the rarity of HCL, scientific literature on autoimmune conditions in patients with HCL consists mainly of published case series and isolated reports. Our analysis identified increased prevalence of various autoimmune conditions in patients with HCL, including various vasculitides, immune cytopenias and antiphospholipid antibody syndrome (APS) among others. Conclusions: Presence of certain autoimmune disorders should increase the suspicion of HCL in an appropriate clinicolaboratory context. Conversely, the diagnosis of HCL should prompt early recognition of certain autoimmune disorders if clinical suspicion exists. While some of these autoimmune diseases are thought to be secondary to the dysfunctional immune response associated with underlying malignant process, others could be primary and might even play a role in the HCL pathogenesis. The autoimmune complications can pose important clinical problems for the HCL patients. Therefore, a catalogue of these problems is important for alerting physicians to watch for them and diagnose them promptly. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: To validate a threshold-based prototype software application (MeVis PULMO 3D) for quantification of chronic interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA) using variable threshold settings for segmentation of diseased lung areas. Methods: Twenty-two patients with rheumatoid arthritis were included and underwent thin-section CT (4×1.25mm collimation). CT scans were assessed by two observers for extent of ILD (EoILD), and twice by MeVis PULMO 3D for each protocol. MeVis PULMO 3D used four segmentation threshold (ST) settings (ST=−740, −780, −800 and −840HU). Pulmonary function tests were obtained in all patients. Statistical evaluation used 95% limits of agreement (LoA) and linear regression analysis. Results: There was total concordance between the software measurements. Interobserver agreement was good (LoA=−28.36 to 17.58%). EoILD by readers correlated strongly with DLCO (r =−0.702, p <0.0001) and moderately with FVC (r =−0.523, p =0.018). There was close correlation between readers and MeVis PULMO 3D with best results for ST <780HU (EoILD vs. MeVis PULMO 3D: r =0.650 for ST=−800 and −840HU, respectively; p =0.002). MeVis PULMO 3D correlated best with DLCO at ST of −800HU (r =−0.44, −0.49, −0.58 and −0.57 for ST=−740, −780, −800 and −840, respectively; p =0.007–0.05) and moderately with FVC (r =−0.44, −0.51, −0.59 and −0.45 for ST=−740, −780, −800 and −840), respectively; p =0.007–0.05). Conclusion: The MeVis PULMO 3D system used holds promise to become a valuable instrument for quantification of chronic ILD in patients with RA when using the threshold value of −800HU, with evidence of the closest correlations, both with human observers and physiologic impairment. [Copyright &y& Elsevier]

Background T-lymphocyte dysfunction has been seldom investigated in collagen vascular disorders. The search for dominant T-cell clones has been scarcely reported, although the presence of such clones might be expected in disorders showing immune responses directed against a variety of autoantigens. Objectives We conducted a systematic search for dominant T-cell clones in peripheral blood in patients with collagen vascular disorders. Patients and methods Ninety-seven patients with collagen vascular disorders were studied (7 cutaneous and 38 systemic lupus erythematosus; 8 multiple morphea; 12 regional scleroderma; 32 systemic sclerosis of the CREST type). A dominant T-cell clone was searched for in peripheral blood by polymerase chain reaction targeting the T-cell receptor γ chain followed by a size analysis of amplified fragments. Peripheral blood from patients with nonlymphocyte-dependent disorders and matched by age and sex was assessed in the same conditions. Results in both groups were compared using nonparametric statistical tests. Results Overall, a circulating dominant T-cell clone was found in 52% of patients compared with 16.9% in controls. More precisely, such a dominant clone was present in 43% and 37% of cutaneous and systemic lupus erythematosus, respectively, in 75% of multiple morphea, 75% of regional scleroderma and 60% of CREST syndrome patients. The percentages in all subsets of patients were significantly higher than in the control group. Conclusions The presence of a dominant T-cell clone in peripheral blood is significantly more frequent in collagen vascular disorders than in controls, especially in patients with scleroderma, whatever the clinical subset, which suggests T-cell involvement in the immune response dysfunction in these diseases classically characterized by disturbances of B lymphocytes. The relevance of such a dominant clone regarding diagnosis, pathomechanisms, long-term outcome and visceral prognosis of these diseases as well as therapeutic decisions remains to be evaluated. [ABSTRACT FROM AUTHOR]

Abstract: Background: The collagen vascular diseases are a collection of conditions, which are thought to be secondary to pathologic alterations in the immune system. Deposition of immune complexes in blood vessel walls resulting in either ischemia or thrombosis is the most widely accepted pathologic mechanism. The lack of familiarity with this subgroup of disease can lead to unnecessary surgical intervention. Data sources: A Medline search was performed of all the English-language literature. Further references were obtained through cross-referencing the bibliography cited in each work. Conclusion: Clinical manifestations are varied and complications include constipation, fecal incontinence, pseudoobstruction, perforation, hemorrhage, and mesenteric ischemia. Colorectal manifestations typically follow dermal presentations. Management should be conservative especially for pseudo-obstructions. Surgical intervention increases morbidity and should be chosen when absolutely necessary. Because of the high incidence of colorectal malignancies in patients with dermatomyositis, aggressive screening should be performed. [Copyright &y& Elsevier]

Easy bruising and bleeding are not only characteristic manifestations of clotting and platelet disorders, they are also prominent features in some heritable collagen disorders, such as the Ehlers–Danlos syndromes (EDS). The EDS comprise a heterogeneous group of connective tissue diseases sharing clinical manifestations in skin, ligaments and joints, blood vessels and internal organs. Most EDS subtypes are caused by mutations in genes encoding the fibrillar collagens type I, III and V, or in genes coding for enzymes involved in the post-translational modification of these collagens. Easy bruising is, to a variable degree, present in all subtypes of EDS, and is because of fragility of the capillaries and the perivascular connective tissues. Vascular fragility affecting medium-sized and large arteries and veins is typically observed in the vascular subtype of EDS, caused by a molecular defect in collagen type III, an important constituent of blood vessel walls and hollow organs. Extensive bruising, spontaneous arterial rupture, leading to severe internal bleeding or premature death, and rupture of hollow organs, such as the intestine or the gravid uterus are predominant features of this subtype. Haematological studies including evaluation of clotting factors, platelet aggregation and bleeding time are usually normal in patients with EDS, except for the Hess test (Rumple–Leede test), which may be abnormal, indicating capillary fragility. In some forms of EDS confirmation of the clinical diagnosis and subtype is possible with biochemical and molecular studies. [ABSTRACT FROM AUTHOR]

This paper describes a typical case of poikiloderma vasculare atrophicans (PVA) in a 48-year-old female. Histologically, the features were suggestive of PVA with the absence of Pautrier′s microabscess or atypical lymphoid cells. The biopsy specimen was positive for cluster of differentiation (CD) 8 on immunohistochemical staining. Its exact pathogenesis remains obscure, and it remains unclear whether PVA actually is mycosis fungoides (MF), a forme fruste of MF, or a distinct and benign dermatosis with CD8+ phenotype that can perhaps be labeled as PVA. However, it has a long benign clinical course without progression to tumor stage of MF in most cases, and its status within the spectrum of cutaneous T-cell lymphoma remains poorly understood. Yet it is imperative to distinguish PVA from poikilodermic MF.

This paper describes a typical case of poikiloderma vasculare atrophicans (PVA) in a 48‑year‑old female. Histologically, the features were suggestive of PVA with the absence of Pautrier's microabscess or atypical lymphoid cells. The biopsy specimen was positive for cluster of differentiation (CD) 8 on immunohistochemical staining. Its exact pathogenesis remains obscure, and it remains unclear whether PVA actually is mycosis fungoides (MF), a forme fruste of MF, or a distinct and benign dermatosis with CD8+ phenotype that can perhaps be labeled as PVA. However, it has a long benign clinical course without progression to tumor stage of MF in most cases, and its status within the spectrum of cutaneous T‑cell lymphoma remains poorly understood. Yet it is imperative to distinguish PVA from poikilodermic MF. [ABSTRACT FROM AUTHOR]

Background: Sarcoidosis is a non-caseating granulomatous disease, mostly affecting previously healthy persons in their fourth and fifth decade. In Belgium, there is a paucity of epidemiological data concerning sarcoidosis and it is unknown to what respect national data on sarcoidosis relates to the global epidemiology of the disease., Objectives: In this cohort study we describe the patient population in an academic center of reference, serving both as a regional care center and a center for a tertiary referral., Methods: We collected epidemiological data among 234 consecutive patients consulting the outpatient sarcoidosis clinic during a two-year time period. We manually explored the electronic patient file for data retrieval., Results: Out of the 234 patients, 140 are male (60%) and 94 are female (40%) patients. Forced vital capacity showed a median decline of 2% during follow-up, whereas median diffusion capacity increased with 4% over the same period of time. Within our study cohort, we observed a preponderance in employment as construction workers (14%), the chemical industry (6%) and in the metal processing industry (6%)., Conclusion: The current study reports on epidemiological findings among the largest cohort of sarcoidosis patients in Belgium published to date., Graphical abstract [Figure: see text].

Background: It has been suggested that the humoral immune system plays a role in the pathogenesis of cryptogenic fibrosing alveolitis (CFA). Although circulating autoantibodies to lung protein(s) have been suggested, none of the lung proteins have been characterised. The purpose of this study was to determine the antigen to which the serum from patients with pulmonary fibrosis reacted.Methods: The anti-A549 cell antibody was characterised in a patient with CFA using Western immunoblotting and immunohistochemical staining of A549 cells. As we identified that one of the antibodies against A549 cells was anti-cytokeratin 8, the expression of mRNA of cytokeratin 8 in A549 cells was evaluated. In addition, we attempted to establish an enzyme linked immunosorbent assay to measure the levels of anti-cytokeratin 8 antibody in the serum of patients with CFA and pulmonary fibrosis associated with collagen vascular disorders (PF-CVD).Results: Initially two anti-A549 cell antibodies were detected in the serum of patients with pulmonary fibrosis, one of which was characterised as anticytokeratin 8 antibody by Western immunoblotting. We were able to establish an ELISA to measure anti-cytokeratin 8 antibody and found significantly higher levels in patients with CFA and PF-CVD than in normal volunteers, patients with sarcoidosis, pneumonia, and pulmonary emphysema.Conclusions: One of the anti-A549 cell antibodies in the serum of patients with CFA was against cytokeratin 8. The serum levels of anti-cytokeratin 8 antibody were increased in patients with CFA and PF-CVD. These results suggest that anticytokeratin 8 antibody may be involved in the process of lung injury in pulmonary fibrosis. [ABSTRACT FROM AUTHOR]

Background: The collagen vascular disorders, particularly systemic sclerosis, dermatomyositis, systemic lupus erythematosus and mixed connective tissue disorder, are often characterized by microangiopathic abnormalities of the nail folds. Nail fold dermoscopy is a well-established technique to assess these vascular changes.Aims: To evaluate finger nail capillary vascular abnormalities by dermoscopy and their correlation with cutaneous and systemic involvement in the patients of collagen vascular disorders.Methods: This was a cross-sectional study involving patients of collagen vascular disorders presenting to Government Medical College, Amritsar over a period of 2 years. Nail fold dermoscopy was done in these patients and correlated with cutaneous and systemic involvement. Statistical analysis was done using SPSS 17.0 version.Results: A total of 30 patients were enrolled in the study. Sixteen (53.3%), 11 (36.7%) and 3 (10%) patients of systemic sclerosis, systemic lupus erythematosus and mixed connective tissue disorder, respectively were included for nail fold dermoscopy. The commonest change recorded in our study was dilated capillaries in 21 (70%) patients, followed by capillary dropouts in 17 (56.7%) patients and avascular areas in 16 (53.3%) patients. Of 17 patients presenting with sclerodactyly, active, early and late patterns were seen in 7 (41.2%), 2 (11.8%) and 7 (41.2%) patients, respectively. Out of 13 patients with respiratory involvement, active, early and late patterns were seen in 1, 1 and 7 (53.8%) patients, respectively (P value = 0.004).Limitations: Owing to lesser number of patients in our study, it is difficult to draw conclusive recommendations, and more studies with a larger sample size are required.Conclusion: Dermoscopy is a valuable tool not only to diagnose collagen vascular disorders but also for prognostication by correlating with systemic involvement. [ABSTRACT FROM AUTHOR]

Abstract: Introduction: Intracardiac thrombi (ICT), more commonly encountered at autopsy, are well documented with underlying cardiovascular disease. Occurrence of ICT in systemic diseases without an intrinsic cardiac disorder is rare. The aim of this autopsy study was to highlight such an occurrence. Methods: From 1996 to 2010, cases with ICT unrelated to primary cardiac disorders were selected at autopsy and analyzed. Clinical and investigational data were obtained from the medical records. The location, morphology, size, and histological appearance of the thrombi were noted. The thrombi were then classified on the basis of their location, nature, and histology (fresh and/or organized); this was correlated with the clinical setting. Results: Among a total of 11,724 autopsies performed in 15 years, 276 patients (2.4%) had ICT. Of these, 45 patients (0.4%) had ICT that were unrelated to primary cardiac diseases. There were 25 men and 20 women with a mean age of 46.1 years. Antemortem diagnosis was not made in any of these patients. Eight patients each (35.6%) showed isolated left-sided and multichambered involvement, while the rest of the hearts (64.4%) had thrombi in the right-sided chamber(s). The recognizable risk factors were underlying cancers (24.4%), prolonged immobilization (20%), systemic lupus erythematosus (6.7%), pregnancy (4.4%), nephropathy (4.4%), primary antiphospholipid antibody syndrome (2.2%), and ulcerative colitis (2.2%). However, 16 patients (35.7%) had no obvious predisposing factor, although investigations for prothrombotic markers had not been done. Diabetes mellitus, chronic alcoholism, and deep vein thrombosis of the lower limbs had been clinically documented in some of them. The cause of death in most patients (73.3%) had been related to pulmonary and/or systemic thromboembolism. Conclusions: This autopsy study emphasizes the great need for a higher index of suspicion of in situ thrombosis in the heart in hypercoagulable states so as to curtail the morbidity and mortality of the primary disease process. [Copyright &y& Elsevier]