Necrotizing vasculitis with a polyarteritis nodosa‐like pattern and selective immunoglobulin A deficiency: case report and review of the literature
Identifieur interne : 001980 ( Main/Exploration ); précédent : 001979; suivant : 001981Necrotizing vasculitis with a polyarteritis nodosa‐like pattern and selective immunoglobulin A deficiency: case report and review of the literature
Auteurs : Sabela Paradela ; Felipe Sacristán [Espagne] ; Manuel Almagro ; Víctor G. Prieto [États-Unis] ; Sara M. Kantrow [États-Unis] ; Eduardo FonsecaSource :
- Journal of Cutaneous Pathology [ 0303-6987 ] ; 2008-09.
English descriptors
- Teeft :
- Acute diarrhea, Anaphylactic transfusion reactions, Autoimmune, Autoimmune disease, Blackwell munksgaard, Case report, Clin, Clin diagn, Clin immunol, Clin immunol immunopathol, Clin pathol, Cutan pathol, Deficiency, Differential diagnosis, Fibrinoid necrosis, Genetic approach, Granulomatous, Granulomatous involvement, Human immunodeficiency virus, Human immunodeficiency virus infection, Igad, Igad patients, Immune complexes, Immunodeficiency, Immunoglobulin, Immunol, Lesion, Lower extremities, Medical history, Mural infiltration, Necrotizing, Necrotizing vasculitis, Normal serum levels, Original magnification, Painful cutaneous lesions, Polyarteritis pattern, Primary antibody deficiency, Primary immunodeficiency disease, Primary immunodeficiency diseases, Rheumatoid arthritis, Rheumatol, Sarcoidosis, Selective igad, Selective immunoglobulin, Serum autoantibodies, Serum levels, Serum protein electrophoresis, Severe infection, Sudden onset, Tract infections, Vasculitis.
Abstract
Selective immunoglobulin A deficiency (IgAD) is a primary immunodeficiency disease characterized by low levels (< 7 mg/dl) of serum immunoglobulin (Ig) A and normal serum levels of IgG and IgM. Patients with IgAD have increased risk for recurrent respiratory and gastrointestinal infections, autoimmune disease, asthma and allergy. A 26‐year‐old woman was admitted with sudden onset of painful cutaneous lesions on her lower extremities, pyrexia and arthromyalgia. Her medical history was remarkable for recurrent respiratory tract infections, self‐limited episodes of acute diarrhea, atopy, splenomegaly and a 4‐year history of a lung granulomatous lesion. Laboratory and imaging tests ruled out severe life‐threatening infection, connective tissue disease and neoplasm. Serum protein electrophoresis showed a low IgA serum level (6.67 mg/dl), with normal serum levels of IgG and IgM, conducting to a diagnosis of selective IgAD. A skin biopsy showed necrotizing vasculitis without any sign of internal organ disease. We report a patient with IgAD and granulomatous involvement of lungs, spleen and medium‐sized arteries of the skin. Although IgAD results from a failure of B‐cell differentiation, we propose that deregulated immune response with production of cross‐reactive antibodies and hyperstimulation of T cells and macrophages could contribute to this widespread granulomatous reaction.
Url:
DOI: 10.1111/j.1600-0560.2007.00911.x
Affiliations:
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Kantrow</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Pathology, The University of Texas M.D. Anderson Cancer Center, Houston, TX</wicri:regionArea><placeName><region type="state">Texas</region></placeName></affiliation></author><author><name sortKey="Fonseca, Eduardo" sort="Fonseca, Eduardo" uniqKey="Fonseca E" first="Eduardo" last="Fonseca">Eduardo Fonseca</name><affiliation></affiliation><affiliation><wicri:noCountry code="no comma">E-mail: fonseca@canalejo.org</wicri:noCountry></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">Journal of Cutaneous Pathology</title><title level="j" type="alt">JOURNAL OF CUTANEOUS PATHOLOGY</title><idno type="ISSN">0303-6987</idno><idno type="eISSN">1600-0560</idno><imprint><biblScope unit="vol">35</biblScope><biblScope unit="issue">9</biblScope><biblScope unit="page" from="871">871</biblScope><biblScope unit="page" to="875">875</biblScope><biblScope unit="page-count">5</biblScope><publisher>Blackwell Publishing Ltd</publisher><pubPlace>Oxford, UK</pubPlace><date type="published" when="2008-09">2008-09</date></imprint><idno type="ISSN">0303-6987</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0303-6987</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>Acute diarrhea</term><term>Anaphylactic transfusion reactions</term><term>Autoimmune</term><term>Autoimmune disease</term><term>Blackwell munksgaard</term><term>Case report</term><term>Clin</term><term>Clin diagn</term><term>Clin immunol</term><term>Clin immunol immunopathol</term><term>Clin pathol</term><term>Cutan pathol</term><term>Deficiency</term><term>Differential diagnosis</term><term>Fibrinoid necrosis</term><term>Genetic approach</term><term>Granulomatous</term><term>Granulomatous involvement</term><term>Human immunodeficiency virus</term><term>Human immunodeficiency virus infection</term><term>Igad</term><term>Igad patients</term><term>Immune complexes</term><term>Immunodeficiency</term><term>Immunoglobulin</term><term>Immunol</term><term>Lesion</term><term>Lower extremities</term><term>Medical history</term><term>Mural infiltration</term><term>Necrotizing</term><term>Necrotizing vasculitis</term><term>Normal serum levels</term><term>Original magnification</term><term>Painful cutaneous lesions</term><term>Polyarteritis pattern</term><term>Primary antibody deficiency</term><term>Primary immunodeficiency disease</term><term>Primary immunodeficiency diseases</term><term>Rheumatoid arthritis</term><term>Rheumatol</term><term>Sarcoidosis</term><term>Selective igad</term><term>Selective immunoglobulin</term><term>Serum autoantibodies</term><term>Serum levels</term><term>Serum protein electrophoresis</term><term>Severe infection</term><term>Sudden onset</term><term>Tract infections</term><term>Vasculitis</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Selective immunoglobulin A deficiency (IgAD) is a primary immunodeficiency disease characterized by low levels (< 7 mg/dl) of serum immunoglobulin (Ig) A and normal serum levels of IgG and IgM. Patients with IgAD have increased risk for recurrent respiratory and gastrointestinal infections, autoimmune disease, asthma and allergy. A 26‐year‐old woman was admitted with sudden onset of painful cutaneous lesions on her lower extremities, pyrexia and arthromyalgia. Her medical history was remarkable for recurrent respiratory tract infections, self‐limited episodes of acute diarrhea, atopy, splenomegaly and a 4‐year history of a lung granulomatous lesion. Laboratory and imaging tests ruled out severe life‐threatening infection, connective tissue disease and neoplasm. Serum protein electrophoresis showed a low IgA serum level (6.67 mg/dl), with normal serum levels of IgG and IgM, conducting to a diagnosis of selective IgAD. A skin biopsy showed necrotizing vasculitis without any sign of internal organ disease. We report a patient with IgAD and granulomatous involvement of lungs, spleen and medium‐sized arteries of the skin. Although IgAD results from a failure of B‐cell differentiation, we propose that deregulated immune response with production of cross‐reactive antibodies and hyperstimulation of T cells and macrophages could contribute to this widespread granulomatous reaction.</div></front></TEI><affiliations><list><country><li>Espagne</li><li>États-Unis</li></country><region><li>Texas</li></region></list><tree><noCountry><name sortKey="Almagro, Manuel" sort="Almagro, Manuel" uniqKey="Almagro M" first="Manuel" last="Almagro">Manuel Almagro</name><name sortKey="Fonseca, Eduardo" sort="Fonseca, Eduardo" uniqKey="Fonseca E" first="Eduardo" last="Fonseca">Eduardo Fonseca</name><name sortKey="Paradela, Sabela" sort="Paradela, Sabela" uniqKey="Paradela S" first="Sabela" last="Paradela">Sabela Paradela</name></noCountry><country name="Espagne"><noRegion><name sortKey="Sacristan, Felipe" sort="Sacristan, Felipe" uniqKey="Sacristan F" first="Felipe" last="Sacristán">Felipe Sacristán</name></noRegion></country><country name="États-Unis"><region name="Texas"><name sortKey="Prieto, Victor G" sort="Prieto, Victor G" uniqKey="Prieto V" first="Víctor G." last="Prieto">Víctor G. Prieto</name></region><name sortKey="Kantrow, Sara M" sort="Kantrow, Sara M" uniqKey="Kantrow S" first="Sara M." last="Kantrow">Sara M. Kantrow</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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