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Granulomatous Disease (granulomatous + disease)
Kinds of Granulomatous Disease Selected AbstractsLupus Erythematosus-Like Lesions by Voriconazole in an Infant with Chronic Granulomatous DiseasePEDIATRIC DERMATOLOGY, Issue 1 2010ELISABETH GOMEZ-MOYANO M.D. The lesions disappeared with termination of the treatment. [source] Granulomatous diseases of the noseINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 12 2009Omid Zargari MD First page of article [source] Trigeminal Trophic Syndrome,Report of Four Cases and Review of the LiteratureDERMATOLOGIC SURGERY, Issue 5 2004Parrish Sadeghi MD Background. Trigeminal trophic syndrome is a unilateral, frequently crescent-shaped neurotrophic ulceration of the face occurring after injury to the trigeminal nerve. The appearance of the ulcers resembles other disease entities such as granulomatous disease, neoplasm, vasculitis, infection, and factitial dermatitis. Objectives. The objectives of this study are to increase awareness of this disorder and to emphasize the importance of eliciting a thorough neurologic history when evaluating facial ulcerations. Methods. Four cases are reported and, using MEDLINE, the English and non-English literature from 1982 to 2002 is reviewed. Results. Including this report, there have been 60 cases of trigeminal trophic syndrome reported from 1982 to 2002. The age at presentation ranged from 14 months to 93 years. Time of onset from injury to the trigeminal ganglion or its branches and the development of the ulcers ranged from 2 weeks to 30 years. One-third of the patients had undergone trigeminal nerve ablation for the treatment of trigeminal neuralgia and another third had a history of stroke. Other causes included craniotomy, head trauma, herpes infection. Conclusion. The majority of cases of trigeminal trophic syndrome are associated with a history of stroke or trigeminal nerve ablation. Successful surgical outcome can be achieved if the underlying neurologic pathology is addressed before the reconstructive procedure. [source] TLR3 modulates immunopathology during a Schistosoma mansoni egg-driven Th2 response in the lungEUROPEAN JOURNAL OF IMMUNOLOGY, Issue 12 2008Amrita D. Joshi Abstract We examined the role of TLR3 in Th2-driven pulmonary granulomatous disease, using wildtype (TLR3+/+) and TLR3 gene-deficient (TLR3,/,) mice in a well-established model of Schistosoma mansoni egg-induced pulmonary granuloma. The intravenous bolus injection of S. mansoni eggs into S. mansoni -sensitized TLR3+/+ mice was associated with an increase in TLR3 transcript expression in alveolar macrophages and ex vivo spleen and lung cultures at day 8 after egg injection. Lungs from TLR3,/, mice showed an increase in granuloma size, greater collagen deposition around the granuloma, and increased Th2 cytokine and chemokine levels compared with similarly sensitized and challenged TLR3+/+ mice. Macrophages from TLR3,/, mice exhibited an M2 phenotype characterized by increased arginase and CCL2 expression. Significantly greater numbers of CD4+CD25+ T cells were present in the lungs of TLR3,/, mice compared with TLR3+/+ mice at day 8 after egg embolization. Cells derived from granulomatous lung and lung draining lymph nodes of TLR3,/, mice released significantly higher levels of IL-17 levels relative to TLR3+/+ cells. Thus, our data suggest that TLR3 has a major regulatory role during a Th2-driven granulomatous response as its absence enhanced immunopathology. [source] Complementation of NADPH oxidase in p67-phox-deficient CGD patientsFEBS JOURNAL, Issue 4 2000p67-phox/p40-phox interaction Chronic granulomatous disease (CGD) is due to a functional defect of the O2, generating NADPH oxidase of phagocytes. Epstein,Barr-virus-immortalized B lymphocytes express all the constituents of oxidase with activity 100 times less than that of neutrophils. As in neutrophils, oxidase activity of Epstein,Barr-virus-immortalized B lymphocytes was shown to be defective in the different forms of CGD; these cells were used as a model for the complementation studies of two p67-phox-deficient CGD patients. Reconstitution of oxidase activity was performed in vitro by using a heterologous cell-free assay consisting of membrane-suspended or solubilized and purified cytochrome b558 that was associated with cytosol or with the isolated cytosolic-activating factors (p67-phox, p47-phox, p40-phox) from healthy or CGD patients. In p67-phox-deficient CGD patients, two cytosolic factors are deficient or missing: p67-phox and p40-phox. Not more than 20% of oxidase activity was recovered by complementing the cytosol of p67-phox-deficient patients with recombinant p67-phox. On the contrary, a complete restoration of oxidase activity was observed when, instead of cytosol, the cytosolic factors were added in the cell-free assay after isolation in combination with cytochrome b558 purified from neutrophil membrane. Moreover, the simultaneous addition of recombinant p67-phox and recombinant p40-phox reversed the previous complementation in a p40-phox dose-dependent process. These results suggest that in the reconstitution of oxidase activity, p67-phox is the limiting factor; the efficiency of complementation depends on the membrane tissue and the cytosolic environment. In vitro, the transition from the resting to the activated state of oxidase, which results from assembling, requires the dissociation of p40-phox from p67-phox for efficient oxidase activity. In the process, p40-phox could function as a negative regulatory factor and stabilize the resting state. [source] Characterization of 11 novel mutations in the X-linked chronic granulomatous disease (CYBB gene)HUMAN MUTATION, Issue 2 2001Bénédicte Gérard Abstract The most frequent form of chronic granulomatous disease (CGD) is caused by inactivation of the CYBB gene, which encodes the gp91-phox subunit of phagocyte NADPH oxidase. This defect prevents phagocytes from producing reactive oxygen species and thus from eradicating bacterial and fungal infections. We investigated 16 unrelated male patients with suspected X-linked CGD and gp91-phox deficiency. A mutation was found in the CYBB gene of all 16 patients, and 11 of these mutations were novel. Eleven patients (69%) had a point mutation (84G>A in two unrelated patients, and 177C>G, 217C>T, 388C>T, 676C>T, 691C>T, 868C>T, 919A>C, 1384G>T and T1514G in one case each, yielding W28X, C59W, R73X, R130X, R226X, Q231X, R290X, T307P, E462X, L505R gp-91phox). One patient had an in-frame deletion removing two amino acids (R54 and A55). Finally, insertions or duplications were found in four patients (from +1 to +31 bases). Overall, 12 (75%) of the mutations led to the production of a truncated protein. No clear correlation was found between clinical manifestations and genomic/biochemical alterations. Thirteen mothers could be tested, and all were carriers. Hum Mutat 18:163, 2001. © 2001 Wiley-Liss, Inc. [source] Sarcoidosis presenting with granulomatous uveitis induced by pegylated interferon and ribavirin therapy for hepatitis CINTERNAL MEDICINE JOURNAL, Issue 3 2008K. K. L. Yan Abstract Sarcoidosis is a systemic granulomatous disease that is triggered by an autoimmune process, and is now a well recognized but uncommon complication of antiviral therapy for Hepatitis C virus (HCV) infection, likely related to its immunomodulatory effects. The clinical presentation of HCV related sarcoidosis is as varied as systemic sarcoidosis, but ocular presentation alone has not been reported previously. We present a 23 year-old female who developed visual disturbances due to ocular sarcoidosis during the course of antiviral therapy for chronic HCV infection. Our case presentation is then followed by a review of the literature on the topic. We aim to stress the importance of screening for eye problems in following HCV patients undergoing antiviral therapy, and raise clinicians' awareness of sarcoidosis as a possible cause for eye problems even in the absence of respiratory complaints. [source] Lupus erythematosus-like lesions in a carrier of X-linked chronic granulomatous disease: A case report and personal considerationsINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 11 2004Caterina Foti MD No abstract is available for this article. [source] Cutaneous sarcoidosis: updates in the pathogenesisJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 7 2010MM Ali Abstract Sarcoidosis is a multiorgan granulomatous disease in which the skin is one of the frequently involved target organs. Cutaneous involvement occurs in a third of patients with sarcoidosis and has protean manifestations. More than a century has passed since the initial description of sarcoidosis, but its cause continues to be an enigma. Recent studies have introduced several new insights into the pathogenesis of this disease. The aim of this literature review was to provide a comprehensive overview on the current updates in the pathogenesis of sarcoidosis. This review has revealed that several genetic polymorphisms are associated with an increased risk of developing sarcoidosis, suggesting that genetic susceptibility to sarcoidosis is probably polygenic. Environmental factors may also modify the susceptibility to sarcoidosis. Evidence favouring an infectious aetiology has been accumulating, but the results of studies are conflicting. The current concept is that the pathogenesis of sarcoidosis involves a T-helper-1-mediated immune response to environmental antigens in a genetically susceptible host. The studies carried out on sarcoidosis have largely focused on the pulmonary aspects and have been mainly conducted by respiratory physicians. In contrast, research conducted on the cutaneous aspects of sarcoidosis is comparatively limited. Although tremendous advances have been made, there is a significant gap between the vast knowledge accumulated on sarcoidosis in recent years and the understanding of this disease. [source] "End-stage" Pulmonary Fibrosis in SarcoidosisMOUNT SINAI JOURNAL OF MEDICINE: A JOURNAL OF PERSONALIZED AND TRANSLATIONAL MEDICINE, Issue 1 2009Alvin T. Teirstein MD Abstract Pulmonary fibrosis is an unusual "end stage" in patients with sarcoidosis. Fibrosis occurs in a minority of patients, and presents with a unique physiologic combination of airways dysfunction (obstruction) superimposed on the more common restrictive dysfunction. Imagin techniques are essential to the diagnosis, assessment and treatment of pulmonary fibrosis. Standard chest radiographs and CT scans may reveal streaks, bullae, cephalad retraction of the hilar areas, deviation of the trachea and tented diaphragm. Positive gallium and PET scans indicate residual reversible granulomatous disease and are important guides to therapy decisions. Treatment, usually with corticosteroids, is effective in those patients with positive scans, but fibrosis does not improve with any treatment. With severe functional impariment and patient disability, pulmonary hypertension and right heart failure may supervene for which the patient will require treatment. Oxygen, careful diuresis, sildenafil and bosentan may be salutary. These patients are candidates for lung transplantation. Mt Sinai J Med 76:30,36, © 2009 Mount Sinai School of Medicine [source] Allogeneic bone marrow transplantation with reduced intensity conditioning for chronic granulomatous disease complicated by invasive Aspergillus infectionPEDIATRIC BLOOD & CANCER, Issue 3 2006Jairam Sastry MBBS, MRCPCH Abstract Chronic granulomatous disease (CGD) is a rare disorder characterized by recurrent infections, often resulting in impaired quality of life and death. Allogeneic BMT provides a definitive cure for CGD, but carries a significant risk of mortality and morbidity. The risk is higher for those who have invasive fungal infection prior to transplant. Reduced intensity conditioning (RIC) is associated with less toxicity from the conditioning agents and may provide an alternative option for all non-malignant diseases. We report a case of successful allogeneic BMT after RIC for a case of X-linked CGD complicated by severe invasive aspergillosis (IA). Pediatr Blood Cancer 2006;47:327,329. © 2006 Wiley-Liss, Inc. [source] Chronic granulomatous disease presenting with disseminated intracranial aspergillosisPEDIATRIC BLOOD & CANCER, Issue 1 2006Abdul Alsultan MD Abstract We describe an 8-year-old boy who presented with multiple unresectable aspergillus brain abscesses as the initial presentation of X-linked chronic granulomatous disease (CGD). He failed initial therapy with amphotericin B, but was subsequently salvaged with voriconazole. CGD should be considered in the differential diagnosis for all children presenting with invasive fungal infections, particularly, those involving the central nervous system (CNS). Whereas, optimal pharmacologic therapy is still unknown for CNS aspergillosis, voriconazole may have an advantage due to its ability to cross the blood brain barrier and excellent oral absorption and bioavailability. © 2005 Wiley-Liss, Inc. [source] Celiac disease and pulmonary hemosiderosis in a patient with chronic granulomatous diseasePEDIATRIC PULMONOLOGY, Issue 4 2004Dominik Hartl MD Abstract We report on a patient with the hitherto undescribed combination of chronic granulomatous disease, pulmonary hemosiderosis, and celiac disease. The hemosiderosis resolved with a gluten-free diet and glucocorticosteroid pulse therapy, but the restrictive lung function pattern remained unchanged. Lung function improved markedly by immunosuppression with daily glucocorticosteroid and azathioprine treatment. Pediatr Pulmonol. 2004; 38:344,348. © 2004 Wiley-Liss, Inc. [source] Reduced-intensity allogeneic hematopoietic cell transplantation: Graft versus tumor effects with decreased toxicityPEDIATRIC TRANSPLANTATION, Issue 3 2003Jennifer E. Schwartz Abstract: The potentially curative role of allogeneic hematopoietic cell transplantation (HCT) in neoplastic and non-neoplastic diseases is offset by the substantial risks of morbidity and mortality from complications of the intensive myeloablative and immunosuppressive preparative regimen. These regimen-related toxicities have restricted allogeneic HCT to young, otherwise healthy individuals without comorbid diseases. Pediatric patients undergoing conventional allogeneic HCT have lower procedure-related mortality but are at risk for non-fatal late effects of the high-dose pretransplant chemoradiotherapy, such as growth retardation, sterility and other endocrine dysfunction. Evaluation of reduced-intensity preparative regimens is the major focus of current clinical research in allogeneic HCT. Reduced-intensity HCT (RI-HCT) relies on the use of immunosuppressive but non-myeloablative agents that allow engraftment of donor cells, which provide adoptive allogeneic cellular immunotherapy and graft versus tumor (GVT) effects, with decreased regimen-related toxicities. Although the experience with RI-HCT in pediatric patients is very limited at this time, results in adults indicate that attenuated-dose preparative regimens allow older patients and those with organ dysfunction to undergo successful allogeneic HCT with acceptable morbidity and mortality. In adults, the potency of the allogeneic GVT effect varies among neoplastic diseases, with better results observed in patients with indolent hematological malignancies or renal cell carcinoma. The effectiveness of RI-HCT as treatment for children with hemoglobinopathies, chronic granulomatous disease and cellular immunodeficiencies is encouraging, and the role of reduced-intensity preparative regimens for allogeneic HCT in pediatric malignancies is under investigation. [source] Acute lymphoblastic leukemia in a patient with chronic granulomatous disease and a novel mutation in CYBB: First reportAMERICAN JOURNAL OF HEMATOLOGY, Issue 1 2005Baruch Wolach Abstract We report for the first time a child with chronic granulomatous disease (CGD) who developed acute lymphoblastic leukemia (ALL). The diagnosis of CGD was made at the age of 4 months, by studies of his neutrophil functions. The superoxide production of the cells was negligible, as was the bactericidal activity. He was found to have a deficiency of the gp91phox subunit of the leukocyte NADPH oxidase, with the X-linked inheritance of the disease. DNA analysis revealed a C nucleotide insertion between C1028 and T1029. This insertion has not been described before and causes a frameshift and a premature stop codon at amino-acid position 347. The mother was found to be a carrier of this mutation. At the age of 16 months, the patient developed T-cell ALL. He was treated for 2 years, and today, 10 years since the diagnosis, he is disease-free. During the course of ALL and later, he suffered from recurrent severe pyogenic infections, but careful detection of the etiological agent and promptly instituted specific treatment resulted in his complete recovery. Although primary immune deficiencies have been reported to have an increased tendency to develop malignancies, until now there have been no reports of CGD patients with ALL. Am. J. Hematol. 80:50,54, 2005. © 2005 Wiley-Liss, Inc. [source] Unusual multifocal granulomatous disease caused by actinomycetous bacteria in a nestling Derbyan parrot (Psittacula derbiana)AUSTRALIAN VETERINARY JOURNAL, Issue 1-2 2009FJ Park A nestling Derbyan parrot (Psittacula derbiana) was presented with unusual subcutaneous swellings of the thigh regions, and poor growth. Histological examination revealed actinomycetous bacteria associated with multifocal systemic granulomas. The clinical and pathological findings of the case are presented, and some relevant aspects of actinomycetous bacterial infections in mammals and birds are discussed. Although granulomatous disease is encountered at times in avian species, the actinomycetous bacteria (Nocardia and Actinomyces spp.) have rarely been reported in association with multifocal granulomatous disease in birds. [source] Generalised granulomatous disease in a horseAUSTRALIAN VETERINARY JOURNAL, Issue 1-2 2004JE AXON A 6-year-old gelding was referred with a 3-month history of recurrent fever, inappetance, lethargy and weight loss. On clinical examination major findings were depression, thin condition, thrombophlebitis, nodules on the scrotal skin, leukocytosis, hyperfibrinogenaemia and hyperglobulinaemia. Pleural fluid and areas of lung consolidation were seen on ultrasonographic examination of the thorax. A diagnosis of chronic respiratory disease was made. Initially there was a response to antibiotic therapy but the horse was presented 3 months later with continued weight loss, recurrent fever and multifocal skin lesions, characterised by scales, crusts and nodules, affecting the nasal bridge, jugular grooves, ventral neck, withers, scrotum, prepuce, and medial gaskins. Histological evaluation of skin biopsies indicated a granulomatous reaction. On ultra-sonographic examination of the thorax multiple hypoechoic lesions consistent with granulomas were seen in both lungs. A diagnosis of generalised granulomatous disease was made. The horse was euthanased at the owner's request. On necropsy examination the main findings were multiple nodules of fibrotic granulomatous inflammation in the lung, heart, liver, gastrointestinal tract and mesenteric lymph nodes, supporting the diagnosis of generalised granulomatous disease. [source] Multilevel Mixture Cure Models with Random EffectsBIOMETRICAL JOURNAL, Issue 3 2009Xin Lai Abstract This paper extends the multilevel survival model by allowing the existence of cured fraction in the model. Random effects induced by the multilevel clustering structure are specified in the linear predictors in both hazard function and cured probability parts. Adopting the generalized linear mixed model (GLMM) approach to formulate the problem, parameter estimation is achieved by maximizing a best linear unbiased prediction (BLUP) type log-likelihood at the initial step of estimation, and is then extended to obtain residual maximum likelihood (REML) estimators of the variance component. The proposed multilevel mixture cure model is applied to analyze the (i) child survival study data with multilevel clustering and (ii) chronic granulomatous disease (CGD) data on recurrent infections as illustrations. A simulation study is carried out to evaluate the performance of the REML estimators and assess the accuracy of the standard error estimates. [source] Multivariate Survival Trees: A Maximum Likelihood Approach Based on Frailty ModelsBIOMETRICS, Issue 1 2004Xiaogang Su Summary. A method of constructing trees for correlated failure times is put forward. It adopts the backfitting idea of classification and regression trees (CART) (Breiman et al., 1984, in Classification and Regression Trees). The tree method is developed based on the maximized likelihoods associated with the gamma frailty model and standard likelihood-related techniques are incorporated. The proposed method is assessed through simulations conducted under a variety of model configurations and illustrated using the chronic granulomatous disease (CGD) study data. [source] Characterization of six novel mutations in CYBA: the gene causing autosomal recessive chronic granulomatous disease,BRITISH JOURNAL OF HAEMATOLOGY, Issue 6 2008Shahram Teimourian Summary One of the rarest forms of chronic granulomatous disease (CGD) is caused by mutations in CYBA, which encodes the p22-phox subunit of the phagocyte NADPH oxidase, leading to defective intracellular killing. This study investigated eight patients (six males and two females) from seven consanguineous, unrelated families with clinical CGD, positive family history and p22-phox deficiency. Mutation analysis of CYBA showed six different novel mutations: deletion of exons 3, 4 and 5; a missense mutation in exon 6 (c.373G>A); a splice site mutation in intron 5 (c.369+1G>A); a frameshift in exon 6 (c.385delGAGC); a frameshift in exon 3 (c.174delG); and a frameshift in exon 4 (c.223delC). [source] Differential modulation of innate immune cell functions by the Burkholderia cepacia complex: Burkholderia cenocepacia but not Burkholderia multivorans disrupts maturation and induces necrosis in human dendritic cellsCELLULAR MICROBIOLOGY, Issue 10 2008Kelly L. MacDonald Summary Burkholderia cepacia complex (BCC) bacteria cause pulmonary infections that can evolve into fatal overwhelming septicemia in chronic granulomatous disease or cystic fibrosis patients. Burkholderia cenocepacia and Burkholderia multivorans are responsible for the majority of BCC infections in cystic fibrosis patients, but B. cenocepacia is generally associated with a poorer prognosis than B. multivorans. The present study investigated whether these pathogens could modulate the normal functions of primary human monocyte-derived dendritic cells (DCs), important phagocytic cells that act as critical orchestrators of the immune response. Effects of the bacteria on maturation of DCs were determined using flow cytometry. DCs co-incubated for 24 h with B. cenocepacia, but not B. multivorans, had reduced expression of costimulatory molecules when compared with standard BCC lipopolysaccharide-matured DCs. B. cenocepacia, but not B. multivorans, also induced necrosis in DCs after 24 h, as determined by annexin V and propidium iodide staining. DC necrosis only occurred after phagocytosis of live B. cenocepacia; DCs exposed to heat-killed bacteria, bacterial supernatant or those pre-treated with cytochalasin D then exposed to live bacteria remained viable. The ability of B. cenocepacia to interfere with normal DC maturation and induce necrosis may contribute to its pathogenicity in susceptible hosts. [source] Decrease in phenotypic regulatory T cells in subsets of patients with common variable immunodeficiencyCLINICAL & EXPERIMENTAL IMMUNOLOGY, Issue 3 2009J. Horn Summary Common variable immunodeficiencies (CVID) are a heterogeneous group of antibody deficiency disorders complicated by autoimmune, lymphoproliferative and/or granulomatous manifestations, suggesting variations in immunoregulation. We sought to quantify regulatory CD4 T cells (Treg cells) in the blood of CVID patients and to correlate the frequency with clinical manifestations and classification subgroups. Blood samples from 99 CVID patients in Freiburg, London and Sydney, who had been phenotyped clinically and stratified according to their memory B cell phenotype (Freiburg and Paris classification schemes), were analysed for the proportion of Treg cells, defined either as CD25+/forkhead box P3 (FoxP3)+, CD25+/CD127low/FoxP3+ or CD25+/CD127low CD4+ T cells, and results compared with 49 healthy controls. Irrespective of the phenotype used to define them, there was a significant decrease in the Treg cell proportion in patients with granulomatous disease and immune cytopenias. This allowed the definition of a subgroup of CVID patients with abnormally low Treg cells, which had a higher rate of these two manifestations as well as autoimmune disease in general. There was also a significant reduction in the proportion of Treg cells in the Freiburg group Ia compared with other CVID patients and controls, but there were no differences between the Paris groups. The reduction in Treg cells in subsets of CVID patients may be relevant to their clinical manifestations, and may contribute to our understanding of the pathogenesis of CVID complications. [source] Oral biopsies from patients with orofacial granulomatosis with histology resembling Crohn's disease have a prominent Th1 environmentINFLAMMATORY BOWEL DISEASES, Issue 4 2007Jona Freysdottir BSc Abstract Background: Orofacial granulomatosis (OFG) is an idiopathic inflammatory disorder of children and young adults whose clinical symptoms include swelling of the lips or face, mucosal nodularity (cobblestoning), mucosal tags, hyperplasia of the gingivae, and aphthous oral ulcers. Whether some OFG patients with clinical and histological characteristics resembling Crohn's disease (CD) are a special group (oral CD) or true CD patients with symptoms reaching all the way to the oral mucosa remains to be determined. Methods: In this study oral biopsies from 10 patients with OFG were analyzed for the presence of T cells, T-cell subsets, B cells, and macrophages, as well as cytokines (IL-4, IL-10, IFN-,, IL-12, and TNF-,), chemokines (RANTES and MIP-1,), and chemokine receptors (CCR3, CCR5, and CXCR3). For comparison, oral tissues from 7 patients with other granulomatous diseases were included. Results: Compared with the non-OFG group, the OFG group had raised levels of CD4+ T cells, IFN-,, IL-10, and RANTES but reduced levels of CD68+ macrophages outside the granulomas, whereas within the granulomas the levels of CD3+ and CD4+ T cells and of IFN-, were raised, but the levels of IL-4 were decreased. These data are indicative of a Th1 environment within the oral OFG tissues, which resembles that already observed in gut CD tissues. Conclusions: Therefore, it can be concluded that some OFG patients have both histopathological and immunopathological features that resemble those observed in CD patients. (Inflamm Bowel Dis 2006) [source] Colonic sarcoidosis, infliximab, and tuberculosis: A cautionary taleINFLAMMATORY BOWEL DISEASES, Issue 4 2004Prof. Dario Sorrentino MD Abstract The antitumor necrosis factor, infliximab, has been recently shown to be effective in refractory sarcoidosis including the intestinal form of this disease. We have tried this therapy in a 55-year-old woman under immunosuppressive therapy for longstanding sarcoidosis presenting with abdominal pain apparently caused by a colonic localization of the disease. The latter diagnosis was based, as recommended, on the presence of nonnecrotizing granulomas in mucosal biopsies, the presence of systemic disease, and the careful exclusion of other granulomatous diseases, including tuberculosis. After the first IV infusion (10 mg/kg BW), she quickly improved, but the wellbeing lasted approximately 4 weeks. She then received another dose of infliximab, but she soon developed low-grade fever and weakness and shortly succumbed of miliary tuberculosis. Likely, infliximab precipitated a pre-existing mycobacterial infection of the intestine. Given the likelihood of underdiagnosing intestinal tuberculosis,and the risks associated with infliximab treatment,this case suggests that this drug should be used with extreme caution, if at all, when a diagnosis of colonic sarcoidosis is suspected. [source] Sarcoidosis of the skin , A dermatological puzzle: important differential diagnostic aspects and guidelines for clinical and histopathological recognitionJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 2 2010G Tchernev Abstract Sarcoidosis of the skin may have an extremely heterogeneous clinical presentation, so that the definitions of ,great imitator' and ,clinical chameleon' have long been used. There is, in fact, a large group of skin diseases that can enter the differential diagnosis with cutaneous sarcoid manifestations, either clinically or/and pathologically. As the clinical consequences and the prognosis of these groups of diseases are often very different, it is important to correctly plan the diagnostic workup. The diagnostic process in this case often presents a challenge as no single test is sufficiently specific, so that a certain diagnosis can be only made in the presence of a compatible clinical and radiographic picture, along with histopathological evidence of non-necrotizing, epithelioid cell granulomas, and exclusion of other potential aetiologies. For practical reasons, four main groups of skin conditions capable of mimicking sarcoidosis can be identified: (i) transmissible, infectious diseases; (ii) allergic and immunological manifestations of various aetiologies; (iii) granulomatous diseases of various aetiologies; and (iv) lymphomas and pseudolymphomas. The aim of this article is to describe the main clinical and histopathological findings of such disease entities, and to discuss the role of those features (morphological, pathological and laboratory) that can help distinguish them from sarcoidosis of the skin. [source] Granulomatous pyoderma gangrenosum: two unusual cases showing necrotizing granulomatous inflammationCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 8 2000Hyang-Joon Park We present two cases of pyoderma gangrenosum (PG) with unusual histopathological findings. The main histopathological feature of PG is usually massive neutrophilic infiltration; the neutrophil is thus the cytologic hallmark of PG. The occurrence of vasculitis is controversial. In our patients, in contrast, biopsy specimens revealed extensive granulomatous inflammation with massive tissue necrosis throughout the entire dermis and subcutaneous tissue and vascular involvement simulating many other granulomatous diseases. However, there was no evidence of systemic disease. Our cases may therefore represent a histopathologically distinct subset of PG. [source] | |||||||||||||||||||||||||||||||