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Jeffrey P. Callen - One of the best experts on this subject based on the ideXlab platform.
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treatment of severe Cutaneous small vessel Vasculitis with mycophenolate mofetil
Archives of Dermatology, 2012Co-Authors: Tye M Haeberle, William Brodie Adams, Jeffrey P. CallenAbstract:A 46-year-old man presented with a 5-week history of painful purpuric ulcerative lesions on his legs (Figure1). A skin biopsy specimen taken by his referring physician exhibited perivascular infiltration of polymorphonuclear leukocytes, fibrinoid deposition on the vessel walls, and extravasation of erythrocytes that was diagnostic of Small-Vessel Vasculitis (leukocytoclastic Vasculitis). No involvement of the deep dermis or subCutaneous tissue was noted in the pathology report. A prior 10-day course of prednisone had proven ineffective. His medical history was positive for intermittent hematuria that predated his Vasculitis by several years. The hematuria was microscopic and had been worked up by a urologist and determined to be without systemic involvement. There were no other positive findings on review of systems and no evidence of systemic Vasculitis. Medications at the onset of his Vasculitis included sertraline, ibuprofen, and zolpidem, none of which was new. Laboratory evaluation revealed no abnormalities in his complete blood cell count, comprehensive metabolic panel, hepatitis profile, erythrocyte sedimentation rate, or serum protein electrophoresis. Serologic findings were negative for cryoglobulins; ANCA; and anti-Smith, antiScl-70, anti-Ro (SS-A), anti-La (SS-B), antihistone, anti– Jo-1, and anticentromeric antibodies. Urinalysis findings were positive for 2 blood, 2 protein, and 11 to 30 red blood cells per high-powered field, consistent with the microscopic hematuria in the patient’s history. Findings of direct immunofluorescence studies of a biopsy specimen taken from the presumed newest lesion on the left thigh were scantly positive for IgM in blood vessels and negative for IgA and IgG; C3 findings were moderately positive in a few vessels and in a discontinuous granular pattern along the basement membrane. Treatment was initiated with colchicine, 0.6 mg twice daily, along with dapsone, 150 mg/d. This regimen blocked the development of new lesions, but there was no change in the ulcerations with these therapies. During the course of his treatment, the patient developed abdominal pain, which he attributed to dapsone, and therapy was discontinued.
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Is Cutaneous Small-Vessel Vasculitis a Marker of Internal Malignancy?
NEJM Journal Watch, 2011Co-Authors: Jeffrey P. CallenAbstract:An association between Cutaneous Vasculitis and internal malignancy has often been reported, especially between Vasculitis and lymphomas or malignancy of the hematopoietic system. A link to solid organ tumors has also been suggested, but is this association real? Should clinicians evaluate patients with Vasculitis for malignancy? In the 1950s, Curth suggested criteria to evaluate the association between skin disease and internal malignancy, including concurrent onset, parallel course, …
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Tissue eosinophilia as an indicator of drug-induced Cutaneous Small-Vessel Vasculitis
Archives of dermatology, 2006Co-Authors: Soon Bahrami, Janine C. Malone, Kelli G. Webb, Jeffrey P. CallenAbstract:Objective To determine whether tissue eosinophilia is a reliable indicator of a drug-induced etiology in biopsy samples demonstrating leukocytoclastic Vasculitis. Design Retrospective medical record review with concurrent histopathologic analysis. Setting University-affiliated dermatology practice. Patients Sixty-three patients with Cutaneous Small-Vessel Vasculitis meeting specific inclusion criteria were divided into drug-induced (n = 16) and non–drug-induced (n = 47) groups. Main Outcome Measures Corresponding histopathologic material was reviewed by a dermatopathologist masked to the etiologic associations. An eosinophil ratio was calculated for each patient, derived from the mean eosinophil score (averaging eosinophil counts from 10 high-power histologic fields), and expressed in relation to the intensity of inflammation in the histopathologic slides examined. Eosinophilia ratios were compared for both groups using the Mann-Whitney test. Results A significant difference was found in mean eosinophil ratios in the drug-induced vs non–drug-induced groups (5.20 vs 1.05; P = .01). Vascular fibrin deposition was present in both groups and was not found to be significantly different ( P = .78). Clinical evidence of systemic Vasculitis was present in 2 patients (13%) in the drug-induced group vs 15 (32%) in the non–drug-induced group. Fourteen patients (88%) in the drug-induced group had a short-term disease course vs 27 (57%) in the non–drug-induced group. Conclusions Tissue eosinophilia is established as a reliable indicator of drug induction in Cutaneous small vessel Vasculitis. Drug-induced Small-Vessel Vasculitis generally follows a short-term disease course without development of systemic involvement. This information may be useful for guiding management decisions, especially when the etiology is unclear.
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Renal Involvement is Frequent in Patients with Cutaneous Small-Vessel Vasculitis in Crete
NEJM Journal Watch, 2002Co-Authors: Jeffrey P. CallenAbstract:Cutaneous Small-Vessel Vasculitis (CSVV) is one manifestation of a generalized disorder. In some patients with palpable purpura, systemic vasculitic
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Cutaneous Vasculitis: Relationship to systemic disease and therapy
Current Problems in Dermatology, 1993Co-Authors: Jeffrey P. CallenAbstract:Abstract Cutaneous Vasculitis is a marker of systemic disease. It may represent the sole manifestation of the disease process or may be considered merely a minor feature of a life-threatening vasculitic syndrome. Some of the confusion in the classification of Vasculitis exists because of the features that are shared by so-called specific syndromes. For example, when one considers patients with paraproteinemia, it is evident that a variety of the vasculitic syndromes can occur in these patients and that the paraprotein may be a manifestation of an associated collagen-vascular disease. Similarly, the patient with hypersensitivity Vasculitis may manifest the full gamut of a systemic Vasculitis including gastrointestinal bleeding and nephritis. Therefore, when approaching the patient in whom Cutaneous disease is a feature, one should consider the full array of vasculitic syndromes, and all organ systems that are potentially affected should be thoroughly evaluated. It appears that the syndrome with which each patient has been diagnosed remains stable. In other words, the patient with chronic Cutaneous (Small-Vessel) Vasculitis seems to have involvement of only the skin, even with long-term follow-up. The pathogenesis of Cutaneous Vasculitis, at least in part, has been linked to the presence of immune complexes in the circulation, which can become deposited in the tissues. However, additional factors including endothelial abnormalities, cytokines, and direct effects of inflammatory cells can also be involved in this complex cascade of events. Although corticosteroids seem to be effective in most vasculitic syndromes, the doses required often result in steroid-related side effects. Thus, it has been a goal in each vasculitic syndrome to find agents that are effective and potentially less toxic than corticosteroids. Immunosuppressive or cytotoxic agents have also demonstrated their effectiveness, but long-term use may result in a greater potential for a secondary malignancy. Other agents to be considered for patients with Vasculitis are antihistamines, antimalarials, dapsone and sulfonamides, and colchicine.
David A. Wetter - One of the best experts on this subject based on the ideXlab platform.
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leukocytoclastic Vasculitis in children clinical characteristics subtypes causes and direct immunofluorescence findings of 56 biopsy confirmed cases
Journal of The European Academy of Dermatology and Venereology, 2017Co-Authors: Emma F. Johnson, David A. Wetter, Julia S. Lehman, Jennifer L. Hand, Dawn Marie R. Davis, Megha M. TollefsonAbstract:Background Leukocytoclastic Vasculitis (LCV) in children is a complex group of conditions. Objectives This study presents the demographics, clinical features, direct immunofluorescence (DIF) results and suspected aetiologies of 56 biopsy-confirmed cases of leukocytoclastic Vasculitis in children. Methods Retrospective review of 56 children seen at Mayo Clinic in Rochester, Minnesota, from 1993 to 2013 with clinical features and Cutaneous biopsy consistent with LCV. Results Twenty-seven (48%) cases were found to be due to IgA Vasculitis (Henoch-Schonlein purpura). The remaining cases were found to be due to Cutaneous Small-Vessel Vasculitis (n = 19, 34%), urticarial Vasculitis (n = 5, 9%), ANCA-associated Vasculitis (n = 4, 7%) and acute haemorrhagic oedema of infancy (n = 1, 2%). IgA Vasculitis was found to be associated with abdominal pain (P = 0.008), whereas the non-IgA Vasculitis group was associated with headache (P = 0.052). Children with IgA Vasculitis had palpable purpura (P = <0.001), petechia (P = 0.057), vesicles (P = 0.009) and involvement of the buttock (P = 0.004) more frequently than the non-IgA Vasculitis group. On DIF, perivascular IgA was positive in IgA Vasculitis compared to non-IgA Vasculitis cases (P = <0.001), the other conjugates were similar between the two groups. Conclusion The most common subtype of biopsy-confirmed LCV in children is IgA Vasculitis. Clinical features, exam characteristics and DIF results can be helpful in determining the subtype of Cutaneous Vasculitis in children.
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Leukocytoclastic Vasculitis in children: clinical characteristics, subtypes, causes and direct immunofluorescence findings of 56 biopsy‐confirmed cases
Journal of the European Academy of Dermatology and Venereology : JEADV, 2016Co-Authors: Emma F. Johnson, David A. Wetter, Julia S. Lehman, Jennifer L. Hand, Dawn Marie R. Davis, Megha M. TollefsonAbstract:Background Leukocytoclastic Vasculitis (LCV) in children is a complex group of conditions. Objectives This study presents the demographics, clinical features, direct immunofluorescence (DIF) results and suspected aetiologies of 56 biopsy-confirmed cases of leukocytoclastic Vasculitis in children. Methods Retrospective review of 56 children seen at Mayo Clinic in Rochester, Minnesota, from 1993 to 2013 with clinical features and Cutaneous biopsy consistent with LCV. Results Twenty-seven (48%) cases were found to be due to IgA Vasculitis (Henoch-Schonlein purpura). The remaining cases were found to be due to Cutaneous Small-Vessel Vasculitis (n = 19, 34%), urticarial Vasculitis (n = 5, 9%), ANCA-associated Vasculitis (n = 4, 7%) and acute haemorrhagic oedema of infancy (n = 1, 2%). IgA Vasculitis was found to be associated with abdominal pain (P = 0.008), whereas the non-IgA Vasculitis group was associated with headache (P = 0.052). Children with IgA Vasculitis had palpable purpura (P =
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histopathological findings in Cutaneous small vessel Vasculitis associated with solid organ malignancy
British Journal of Dermatology, 2014Co-Authors: Joshua O. Podjasek, David A. Wetter, Carilyn N. Wieland, Michael J. Camilleri, Christine M. LohseAbstract:Summary Background Histopathological findings in biopsy specimens from patients with Cutaneous Small-Vessel Vasculitis (CSVV) secondary to solid-organ malignancy have not been previously reported. Objectives We aimed to understand better the differences in histopathological findings between biopsy specimens from patients with CSVV associated with solid-organ malignancies and patients with CSVV secondary to other causes. Methods From a previously published group of patients with CSVV and solid-organ malignancy, we identified patients with available histopathology slides of biopsy specimens. We compared histopathological findings from these patients with those from 68 previously published patients with Henoch–Schonlein purpura not associated with solid-organ malignancy (60% male). Results We identified 15 patients (eight male, 53%) with available slides from biopsy specimens. The mean age of these patients with solid-organ malignancy-associated CSVV was 66·6 years, compared with 45·8 years in the Henoch–Schonlein purpura cases not associated with solid-organ malignancy (P < 0·001). Solid-organ malignancy-associated CSVV was less likely to demonstrate papillary dermal oedema (P = 0·04), papillary dermal inflammation (P < 0·001) and lymphocytes (P < 0·001), and more likely to have plasma cells (P = 0·02). Additionally, we detected nonsignificant differences in the presence of histiocytes (P = 0·05), intravascular thrombosis (P = 0·052) and microabscess formation (P = 0·06). Conclusions CSVV associated with solid-organ malignancies tended to have deeper dermal involvement and a different cellular milieu from cases not associated with solid-organ malignancies. In addition, the patients with CSVV with solid-organ malignancies were significantly older than those without. Prospective studies with age-matched controls are needed to determine the clinical significance of the histopathological differences in solid-organ malignancy-associated CSVV.
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Histopathological findings in Cutaneous Small-Vessel Vasculitis associated with solid-organ malignancy.
The British journal of dermatology, 2014Co-Authors: Joshua O. Podjasek, David A. Wetter, Carilyn N. Wieland, Michael J. Camilleri, Christine M. LohseAbstract:Summary Background Histopathological findings in biopsy specimens from patients with Cutaneous Small-Vessel Vasculitis (CSVV) secondary to solid-organ malignancy have not been previously reported. Objectives We aimed to understand better the differences in histopathological findings between biopsy specimens from patients with CSVV associated with solid-organ malignancies and patients with CSVV secondary to other causes. Methods From a previously published group of patients with CSVV and solid-organ malignancy, we identified patients with available histopathology slides of biopsy specimens. We compared histopathological findings from these patients with those from 68 previously published patients with Henoch–Schonlein purpura not associated with solid-organ malignancy (60% male). Results We identified 15 patients (eight male, 53%) with available slides from biopsy specimens. The mean age of these patients with solid-organ malignancy-associated CSVV was 66·6 years, compared with 45·8 years in the Henoch–Schonlein purpura cases not associated with solid-organ malignancy (P
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A Practical Approach to the Diagnosis, Evaluation, and Management of Cutaneous Small-Vessel Vasculitis
American Journal of Clinical Dermatology, 2014Co-Authors: Megan R. Goeser, Valerie Laniosz, David A. WetterAbstract:Cutaneous Small-Vessel Vasculitis (CSVV) is a disorder characterized by neutrophilic inflammation predominantly limited to the superficial Cutaneous postcapillary venules. CSVV may be idiopathic or may have a defined cause such as infection, medication, connective tissue disease, or malignancy. CSVV may also be associated with extraCutaneous disease or systemic Vasculitis. The most common clinical presentation of CSVV consists of symmetrically distributed palpable purpura of the lower extremities. In general, lesional skin biopsy samples should be examined with light microscopy and direct immunofluorescence for adult patients with suspected CSVV. A complete history, review of systems, physical examination, and selected laboratory studies also should be performed to assess for inciting causes or extraCutaneous involvement of CSVV. Treatment varies and depends on the chronicity of CSVV, the severity of Cutaneous involvement, and the presence or absence of both an underlying cause and extraCutaneous involvement of CSVV. An isolated episode of CSVV associated with a known inciting factor may be managed by removal or treatment of the trigger, along with symptomatic measures. First-line systemic treatments for chronic, idiopathic CSVV include colchicine or dapsone, used singly or in combination. Recurrent, chronic, or severely symptomatic CSVV that does not respond to the aforementioned therapies may require initiation of an immunosuppressive agent such as azathioprine, mycophenolate mofetil, methotrexate, cyclosporine, or rituximab.
Ana Suarezvalle - One of the best experts on this subject based on the ideXlab platform.
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Cutaneous small vessel Vasculitis associated with novel 2019 coronavirus sars cov 2 infection covid 19
Journal of The European Academy of Dermatology and Venereology, 2020Co-Authors: Miguel Dominguezsantas, Borja Diazguimaraens, Garcia P Abellas, Morenogarcia C Del Real, Patricia Burgosblasco, Ana SuarezvalleAbstract:The severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) has rapidly become a global health issue. Although it is known to produce diverse Cutaneous manifestations, some of them have yet to be described. This letter reports new dermatologic findings associated with a confirmed COVID‐19 case.
Mohamed Effat - One of the best experts on this subject based on the ideXlab platform.
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Warfarin induced leukocytoclastic Vasculitis: an extraordinary side effect
Journal of Thrombosis and Thrombolysis, 2019Co-Authors: Dina Elantably, Ahmed Mourad, Ahmed Elantably, Mohamed EffatAbstract:Warfarin is one of the most commonly used anticoagulants in the management of thromboembolic events. Herein we report a rare case of warfarin induced leukocytoclastic Vasculitis in a patient with history of rheumatic heart disease and a mechanical mitral valve prosthesis who presented with heart failure and palpable purpura. Upon clinical suspicion of Cutaneous small vessel Vasculitis, a comprehensive laboratory panel was performed. Warfarin induced Vasculitis was suspected when withdrawal of warfarin, due to rising INR, led to improvement of the skin lesions. The diagnosis was finally confirmed when re-instatement of warfarin reproduced the skin lesions and a skin biopsy showed evidence for leukocytoclastic Vasculitis with eosinophilic infiltration. A third of cases of leukocytoclastic Vasculitis are due to drug hypersensitivity which being a diagnosis of exclusion with varying manifestations, requires a high index of clinical suspicion. Since drug induced leukocytoclastic Vasculitis may affect multiple organ systems and even cause mortality, clinicians must be aware of this rare adverse event, promptly discontinue the drug, and commence anti-inflammatory or immunosuppressive treatment when necessary.
Angelo V Marzano - One of the best experts on this subject based on the ideXlab platform.
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A project by the SIDeMaST Immunopathology Group on Cutaneous Vasculitis
Giornale italiano di dermatologia e venereologia : organo ufficiale Societa italiana di dermatologia e sifilografia, 2015Co-Authors: Manuela Papini, Pietro Quaglino, M. La Placa, Angelo V MarzanoAbstract:Vasculitides are a challenge to the clinician, in terms of both diagnosis and therapy. Multiple classi!cation systems have been implemented and the numerous classi!cation schemes re"ect the complexity of establishing a simple classi!cation that could be functional for daily care. Although Vasculitis classi!cation has become increasingly elaborated, some areas remain ill de!ned. Some forms of Vasculitis are still dif!cult to assign to a speci!c disease entity. Generally accepted operational criteria are available for many vasculitides, but for some entities there are no effective criteria. Moreover, diagnostic criteria for Vasculitis with suf!cient strength and/or con!dence that can be universally accepted are not yet available. The need for diagnostic criteria validated and agreed upon is particularly relevant in the context of Cutaneous Vasculitis. The project of the SIDeMaST Italian Group of Immunopathology on Cutaneous Vasculitis is a national prospective observational study designed to develop and validate diagnostic criteria and to improve and validate classi!cation criteria for Cutaneous small vessel Vasculitis also known as leukocytoclastic Vasculitis (CLV). Primary objective of the study will also be that of developing the Cutaneous Vasculitis Severity Index (CUVASI). Secondary objectives of the project will be: 1) de!nition of the etiological agents that are most frequently associated with CLV; 2) search for possible correlations between causative agent and peculiar clinical and/or histopathological aspects; 3) evaluation of immuno"uorescence pattern observed in this speci!c group of primitive Cutaneous Vasculitis in order to characterize the diagnostic sensitivity and speci!city of this technique; 4) identi!cation of a set of clinical investigations and laboratory tests to be performed for a correct CLV assessment. Actually 15 Italian dermatological clinics are contributing to the project and anticipated recruiting >100 patients with CLV. A pilot retrospective study to assess the feasibility of the project is going to be launched and if its results are positive then the pro
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skin involvement in Cutaneous and systemic Vasculitis
Autoimmunity Reviews, 2013Co-Authors: Angelo V Marzano, Pamela Vezzoli, Elisabetta BertiAbstract:Abstract Cutaneous vasculitides are a heterogeneous group of inflammatory disorders affecting skin blood vessels. They may be triggered by several factors, such as infection or drug, or may be related to underlying disease, notably connective tissue or malignancies. However, Vasculitis occurs without any demonstrable triggering agents in a relevant number of patients. On the other hand, vasculitic skin lesions may manifest as a component of Vasculitis affecting also internal organs; in someone of these patients, skin involvement occurs initially as the sole sign of disease, leading to consider Cutaneous Vasculitis a diagnosis of exclusion. In this review, we have focused on the most common variants of Cutaneous Vasculitis, including Cutaneous small vessel Vasculitis and urticarial Vasculitis as well as Henoch–Schonlein purpura, a systemic form in which however skin involvement often predominates. We have also argued on livedoid vasculopathy, a Cutaneous entity which, although nonfrankly vasculitic in origin, is frequently associated with connective tissue disease. Finally, we have analyzed the variety of Cutaneous manifestations that may develop during the course of the main systemic vasculitides, such as Wegener's granulomatosis, Churg–Strauss syndrome and polyarteritis nodosa.
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Skin involvement in Cutaneous and systemic Vasculitis
'Elsevier BV', 2013Co-Authors: Angelo V Marzano, Pamela Vezzoli, Elisabetta BertiAbstract:Cutaneous vasculitides are a heterogeneous group of inflammatory disorders affecting skin blood vessels. They may be triggered by several factors, such as infection or drug, or may be related to underlying disease, notably connective tissue or malignancies. However, Vasculitis occurs without any demonstrable triggering agents in a relevant number of patients. On the other hand, vasculitic skin lesions may manifest as a component of Vasculitis affecting also internal organs; in someone of these patients, skin involvement occurs initially as the sole sign of disease, leading to consider Cutaneous Vasculitis a diagnosis of exclusion. In this review, we have focused on the most common variants of Cutaneous Vasculitis, including Cutaneous small vessel Vasculitis and urticarial Vasculitis as well as Henoch-Sch\uf6nlein purpura, a systemic form in which however skin involvement often predominates. We have also argued on livedoid vasculopathy, a Cutaneous entity which, although nonfrankly vasculitic in origin, is frequently associated with connective tissue disease. Finally, we have analyzed the variety of Cutaneous manifestations that may develop during the course of the main systemic vasculitides, such as Wegener's granulomatosis, Churg-Strauss syndrome and polyarteritis nodosa. \ua9 2012 Elsevier B.V
