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I M Murraylyon - One of the best experts on this subject based on the ideXlab platform.
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reversible plane xanthoma Vasculitis and peliosis hepatis in giant lymph node hyperplasia castleman s disease a case report and review of the Cutaneous manifestations of giant lymph node hyperplasia
Journal of The American Academy of Dermatology, 1992Co-Authors: D Sherman, B Ramsay, N A Theodorou, D Woodrow, F P Paradinas, J J Cream, I M MurraylyonAbstract:A patient is described who had generalized plane xanthomas, Cutaneous Vasculitis, peliosis hepatis, and intraabdominal giant lymph node hyperplasia of the plasma cell type. After excision of the abdominal mass, the xanthonnas resolved and the liver returned to its normal size, but the patient continued to develop skin lesions. A review is presented of the Cutaneous manifestations of giant lymph node hyperplasia.
Andrew J Carlson - One of the best experts on this subject based on the ideXlab platform.
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nomenclature of Cutaneous Vasculitis dermatologic addendum to the 2012 revised international chapel hill consensus conference nomenclature of vasculitides
Arthritis & Rheumatism, 2018Co-Authors: Cord Sunderkotter, Ko-ron Chen, Andrew J Carlson, Bernhard Zelger, Luis Requena, Warren W Piette, Jan P Dutz, Peter Lamprecht, Alfred Mahr, Elisabeth AbererAbstract:Objective To prepare a dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC2012) to address vasculitides affecting the skin (D-CHCC). The goal was to standardize the names and definitions for Cutaneous Vasculitis. Methods A nominal group technique with a facilitator was used to reach consensus on the D-CHCC nomenclature, using multiple face-to-face meetings, e-mail discussions, and teleconferences. Results Standardized names, definitions, and descriptions were adopted for Cutaneous components of systemic vasculitides (e.g., Cutaneous IgA Vasculitis as a component of systemic IgA Vasculitis), skin-limited variants of systemic vasculitides (e.g., skin-limited IgA Vasculitis, drug-induced skin-limited antineutrophil cytoplasmic antibody-associated Vasculitis), and Cutaneous single-organ vasculitides that have no systemic counterparts (e.g., nodular Vasculitis). Cutaneous vasculitides that were not included in the CHCC2012 nomenclature were introduced. Conclusion Standardized names and definitions are a prerequisite for developing validated classification and diagnostic criteria for Cutaneous Vasculitis. Accurate identification of specifically defined variants of systemic and skin-limited vasculitides requires knowledgeable integration of data from clinical, laboratory, and pathologic studies. This proposed nomenclature of vasculitides affecting the skin, the D-CHCC, provides a standard framework both for clinicians and for investigators.
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the histological assessment of Cutaneous Vasculitis
Histopathology, 2010Co-Authors: Andrew J CarlsonAbstract:Carlson J A (2010) Histopathology56, 3–23 The histological assessment of Cutaneous Vasculitis Vasculitis is defined as inflammation directed at vessels, which compromises or destroys the vessel wall leading to haemorrhagic and/or ischaemic events. Skin biopsy is the gold standard for the diagnosis of Cutaneous Vasculitis, whose manifestations include urticaria, infiltrative erythema, petechiae, purpura, purpuric papules, haemorrhagic vesicles and bullae, nodules, livedo racemosa, deep (punched out) ulcers and digital gangrene. These varied morphologies are a direct reflection of size of the vessels and extent of the vascular bed affected, ranging from a Vasculitis affecting few superficial, small vessels in petechial eruptions to extensive pan-dermal small vessel Vasculitis in haemorrhagic bullae to muscular vessel Vasculitis in lower extremity nodules with livedo racemosa. Skin biopsy, extending to subcutis and taken from the earliest, most symptomatic, reddish or purpuric lesion is crucial for obtaining a high-yielding diagnostic sample. Based on histology, Vasculitis can be classified on the size of vessels affected and the dominant immune cell mediating the inflammation (e.g. neutrophilic, granulomatous, lymphocytic, or eosinophilic). Disruption of small vessels by inflammatory cells, deposition of fibrin within the lumen and/or vessel wall coupled with nuclear debris allows for the confident recognition of small vessel, mostly neutrophilic Vasculitis (also known as leukocytoclastic Vasculitis). In contrast, muscular vessel Vasculitis can be identified solely by infiltration of its wall by inflammatory cells. Extravasation of red blood cells (purpura) and necrosis are supportive, but not diagnostic of Vasculitis as they are also seen in haemorrhagic and/or vaso-occlusive disorders (pseudoVasculitis). Vasculitic foci associated with extravascular granulomas (palisaded neutrophilic and granulomatous dermatitis), tissue eosinophilia, or tissue neutrophilia signal the risk for, or co-existence of systemic disease. This essential histological information coupled with direct immunofluorescence and anti-neutrophil cytoplasmic data and clinical findings enables more precise and accurate diagnosis of localized and systemic Vasculitis syndromes.
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Cutaneous Vasculitis update neutrophilic muscular vessel and eosinophilic granulomatous and lymphocytic Vasculitis syndromes
American Journal of Dermatopathology, 2007Co-Authors: Andrew J Carlson, Ko-ron ChenAbstract:Most biopsies of Cutaneous Vasculitis will exhibit a small vessel neutrophilic Vasculitis [leukocytoclastic Vasculitis (LCV)] that is associated with immune complexes on direct immunofluorescence examination or, less commonly, antineutrophilic cytoplasmic antibodies (ANCA) by indirect immunofluorescence testing. Is in uncommon for skin biopsy to reveal solely a neutrophilic arteritis signifying the presence of Cutaneous polyarteritis nodosa or, if accompanied by significant lobular panniculitis, nodular Vasculitis/erythema induratum. In other cases, Cutaneous vascular damage (fibrinoid necrosis, muscular vessel wall disruption, or endarteritis obliterans) will be mediated by a nonneutrophilic inflammatory infiltrate. Eosinophilic Vasculitis can be a primary (idiopathic) process that overlaps with hypereosinophilic syndrome, or it can be a secondary Vasculitis associated with connective tissue disease or parasite infestation. Authentic Cutaneous granulomatous Vasculitis (versus Vasculitis with extravascular granulomas) can represent a Cutaneous manifestation of giant cell arteritis, an eruption secondary to systemic disease such as Crohn's disease or sarcoidosis, or a localized disorder, often a post-herpes zoster (HZ) phenomenon. Lymphocytic Vasculitis is a histologic reaction pattern that correlates with broad clinical differential diagnosis, which includes connective tissue disease - mostly systemic lupus erythematosus (SLE), endothelial infection by Rickettsia and viruses, idiopathic lichenoid dermatoses such as perniosis or ulcerative necrotic Mucha-Habermann disease, and angiocentric Cutaneous T-cell lymphomas. Skin biopsy extending into the subcutis, identifying the dominant inflammatory cell and caliber of vessels affected, extravascular histologic clues such as presence of lichenoid dermatitis or panniculitis, and correlation with clinical data allows for accurate diagnosis of these uncommon vasculitic entities.
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Cutaneous Vasculitis update small vessel neutrophilic Vasculitis syndromes
American Journal of Dermatopathology, 2006Co-Authors: Andrew J Carlson, Ko-ron ChenAbstract:A broad and diverse spectrum of vasculitic syndromes exists. These syndromes affect the skin with varying levels of associated systemic manifestations, running the gamut from a self-limited, localized, Cutaneous phenomenon to rapidly progressive, multiorgan disease. The majority of cases of Cutaneous Vasculitis will show a neutrophilic small vessel Vasculitis that can be either a primary (idiopathic) disorder (eg, Cutaneous leukocytoclastic angiitis) or a secondary disorder that is associated with drugs, infection (eg, streptococcal infection, viral hepatitis), or underlying disease (eg, connective tissue disease, malignancy). Biopsy is the gold standard for the diagnosis of Cutaneous Vasculitis and also necessary for the detection of Cutaneous vascular immune complexes by direct immunofluorescence. Based on the type of vessel disrupted by inflammation (small and/or muscular), the distribution of Vasculitis in the dermis and subcutis, and predominate inflammatory cell-type mediating vessel wall damage, a list of relevant differential diagnoses can be generated. This histologic information coupled with extravascular findings such as tissue eosinophilia, tissue neutrophilia, and/or granulomas, plus pathophysiologic markers such as direct immunofluorescent examination for immune complexes and serologic evaluation for antineutrophil cytoplasmic antibodies allows for more accurate diagnosis of specific vasculitic entities. Herein, we review both primary and secondary vasculitic syndromes that affect the skin and show a small vessel neutrophilic mediated Vasculitis.
Elisabeth Aberer - One of the best experts on this subject based on the ideXlab platform.
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nomenclature of Cutaneous Vasculitis dermatologic addendum to the 2012 revised international chapel hill consensus conference nomenclature of vasculitides
Arthritis & Rheumatism, 2018Co-Authors: Cord Sunderkotter, Ko-ron Chen, Andrew J Carlson, Bernhard Zelger, Luis Requena, Warren W Piette, Jan P Dutz, Peter Lamprecht, Alfred Mahr, Elisabeth AbererAbstract:Objective To prepare a dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC2012) to address vasculitides affecting the skin (D-CHCC). The goal was to standardize the names and definitions for Cutaneous Vasculitis. Methods A nominal group technique with a facilitator was used to reach consensus on the D-CHCC nomenclature, using multiple face-to-face meetings, e-mail discussions, and teleconferences. Results Standardized names, definitions, and descriptions were adopted for Cutaneous components of systemic vasculitides (e.g., Cutaneous IgA Vasculitis as a component of systemic IgA Vasculitis), skin-limited variants of systemic vasculitides (e.g., skin-limited IgA Vasculitis, drug-induced skin-limited antineutrophil cytoplasmic antibody-associated Vasculitis), and Cutaneous single-organ vasculitides that have no systemic counterparts (e.g., nodular Vasculitis). Cutaneous vasculitides that were not included in the CHCC2012 nomenclature were introduced. Conclusion Standardized names and definitions are a prerequisite for developing validated classification and diagnostic criteria for Cutaneous Vasculitis. Accurate identification of specifically defined variants of systemic and skin-limited vasculitides requires knowledgeable integration of data from clinical, laboratory, and pathologic studies. This proposed nomenclature of vasculitides affecting the skin, the D-CHCC, provides a standard framework both for clinicians and for investigators.
Mariacarolina Salerno - One of the best experts on this subject based on the ideXlab platform.
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Cutaneous Vasculitis in patients with autoimmune polyendocrine syndrome type 1 report of a case and brief review of the literature
BMC Pediatrics, 2014Co-Authors: Nicola Improda, Donatella Capalbo, Emilia Cirillo, Manuela Cerbone, Andrea Esposito, Claudio Pignata, Mariacarolina SalernoAbstract:Autoimmune polyendocrine syndrome type 1, also known as autoimmune polyendocrinopathy-candidiasis-ectodermal-dystrophy, is a rare autosomal recessive disease due to pathogenic variants in the AIRE gene. Classic features of the syndrome are mucoCutaneous candidiasis, chronic idiopathic hypoparathyroidism and Addison disease. However, other endocrine and non-endocrine components, may occur with a different prevalence. In addition to ectodermal features, which are quite common features of the disease, APS 1 patients may experience other types of skin alterations, such as vasculitic skin rash. An early diagnosis of APS 1 can be very challenging, due to the high clinical heterogeneity, and a considerable delay may occur between the appearance of symptoms and the diagnosis. We report on a girl affected by APS 1 who presented with Cutaneous Vasculitis when she was seven-months old, some years before the onset of the common components of the disease. Clinical picture of APS 1 may be characterized by isolated rare or atypical autoimmune or immune-mediated manifestations, even years before the onset of the classic components of the disease. Among these uncommon features, skin rashes of variable form and duration may occur, most of them being associated with histopathological features of Vasculitis. Our case suggests that Cutaneous Vasculitis may represent a first sign of APS 1. The clinical significance of Cutaneous Vasculitis in the context of APS 1 is still debated. It may represent a rare, unusual, early component of the disease or a clinical manifestation secondarily related to the typical APS 1 components (i.e. autoimmune thyroid disease), which are frequently associated with rheumatologic-like signs and symptoms. Alternatively, it may be the expression of an independent disease co-occuring with APS 1. In conclusion, our case suggests that children presenting with unexplained vasculitic skin rash should be followed-up in order to early identify APS 1.
D Sherman - One of the best experts on this subject based on the ideXlab platform.
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reversible plane xanthoma Vasculitis and peliosis hepatis in giant lymph node hyperplasia castleman s disease a case report and review of the Cutaneous manifestations of giant lymph node hyperplasia
Journal of The American Academy of Dermatology, 1992Co-Authors: D Sherman, B Ramsay, N A Theodorou, D Woodrow, F P Paradinas, J J Cream, I M MurraylyonAbstract:A patient is described who had generalized plane xanthomas, Cutaneous Vasculitis, peliosis hepatis, and intraabdominal giant lymph node hyperplasia of the plasma cell type. After excision of the abdominal mass, the xanthonnas resolved and the liver returned to its normal size, but the patient continued to develop skin lesions. A review is presented of the Cutaneous manifestations of giant lymph node hyperplasia.
