Localized Wegener’s granulomatosis
Identifieur interne : 001546 ( Main/Exploration ); précédent : 001545; suivant : 001547Localized Wegener’s granulomatosis
Auteurs : A. V. Marzano [Italie] ; Y. Balice [Italie] ; M. Papini [Italie] ; R. Testa [Italie] ; E. Berti [Italie] ; C. Crosti [Italie]Source :
- Journal of the European Academy of Dermatology and Venereology [ 0926-9959 ] ; 2011-12.
English descriptors
- Teeft :
- Acad dermatol, Aggressive behaviour, Authors journal, Cartilage, Clinical features, Cyclophosphamide, Dermatology, Erythematous plaque, Erythrocyte sedimentation rate, European academy, Exudating surface nostrils, Facial, Facial bones, Facial region, Fondazione irccs, Giant cells, Good response, Granda ospedale maggiore policlinico, Granulomatosis, Histopathologic traits, Lesion, Lesional skin, Leucocytoclastic vasculitis, Localized, Massive destruction, Nasal, Nasal cartilage, Nasal mucosa, Ndings, Neutrophil, Oral azathioprine, Oral cavity, Oral cyclophosphamide, Oral manifestations, Oral prednisone, Palatal, Palatal bone, Palatal mucosae, Partial remission, Persistent disease stage, Plaque, Prednisone, Respiratory tract, Skin lesion, Slight improvement, Smooth surface, Study group, Systemic involvement, Universita degli studi, Upper airway mucosa, Vasculitis, Venereology, Venereology marzano, Wegener granulomatosis.
Abstract
Background Wegener’s granulomatosis (WG) is a rare granulomatous necrotizing vasculitis of small and medium vessels which has predilection for upper airways, lungs and kidney. However, any other organ, including the skin and oral cavity, can be involved. Although mucocutaneous lesions are relatively common, they have only rarely been reported as localized manifestation of the disease. Objectives Our aim was to evaluate the type and sites of skin and mucosal lesions, clinical course and response to treatment, histologic features and laboratory findings in localized WG. Methods The medical records of three patients (two women and one man) with localized WG followed up at our hospitals for a mean time of 10 years were studied. Results All patients presented with facial plaques infiltrating the nasal and palatal mucosae and cartilages and, in one case, perforating the palatal bone. Anti‐neutrophil cytoplasmic antibodies, which are the marker for multisystem WG, were negative. The disease, refractory to various immunosuppressants, responded well, albeit incompletely, to prednisone plus cyclophosphamide. Limitations The limited number of patients is counterbalanced by the rarity of the disease. Conclusions Our cases may represent a rare distinctive subset of WG limited to the facial region and upper airway mucosa but showing a locally aggressive behaviour leading to cartilage and bony destruction.
Url:
DOI: 10.1111/j.1468-3083.2010.03970.x
Affiliations:
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Clinica Dermatologica e Venerologica di Terni, Dipartimento di Specialità Medico‐Chirurgiche e Sanità Pubblica, Università di Perugia, Terni</wicri:regionArea><wicri:noRegion>Terni</wicri:noRegion></affiliation></author><author><name sortKey="Berti, E" sort="Berti, E" uniqKey="Berti E" first="E." last="Berti">E. Berti</name><affiliation wicri:level="3"><country xml:lang="fr">Italie</country><wicri:regionArea>U.O. Dermatologia, Fondazione IRCCS Ca’ Granda – Ospedale Maggiore Policlinico, Dipartimento di Anestesiologia, Terapia Intensiva e Scienze Dermatologiche, Università degli Studi di Milano, Milan</wicri:regionArea><placeName><settlement type="city">Milan</settlement><region nuts="2">Lombardie</region></placeName></affiliation><affiliation wicri:level="3"><country xml:lang="fr">Italie</country><wicri:regionArea>Dipartimento di Medicina Clinica e Prevenzione, Università degli Studi di Milano‐Bicocca, Milan</wicri:regionArea><placeName><settlement type="city">Milan</settlement><region nuts="2">Lombardie</region></placeName></affiliation></author><author><name sortKey="Crosti, C" sort="Crosti, C" uniqKey="Crosti C" first="C." last="Crosti">C. Crosti</name><affiliation wicri:level="3"><country xml:lang="fr">Italie</country><wicri:regionArea>U.O. Dermatologia, Fondazione IRCCS Ca’ Granda – Ospedale Maggiore Policlinico, Dipartimento di Anestesiologia, Terapia Intensiva e Scienze Dermatologiche, Università degli Studi di Milano, Milan</wicri:regionArea><placeName><settlement type="city">Milan</settlement><region nuts="2">Lombardie</region></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">Journal of the European Academy of Dermatology and Venereology</title><title level="j" type="alt">JOURNAL OF EUROPEAN ACADEMY DERMATOLOGY VENEREOLOGY</title><idno type="ISSN">0926-9959</idno><idno type="eISSN">1468-3083</idno><imprint><biblScope unit="vol">25</biblScope><biblScope unit="issue">12</biblScope><biblScope unit="page" from="1466">1466</biblScope><biblScope unit="page" to="1470">1470</biblScope><biblScope unit="page-count">5</biblScope><publisher>Blackwell Publishing Ltd</publisher><pubPlace>Oxford, UK</pubPlace><date type="published" when="2011-12">2011-12</date></imprint><idno type="ISSN">0926-9959</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0926-9959</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>Acad dermatol</term><term>Aggressive behaviour</term><term>Authors journal</term><term>Cartilage</term><term>Clinical features</term><term>Cyclophosphamide</term><term>Dermatology</term><term>Erythematous plaque</term><term>Erythrocyte sedimentation rate</term><term>European academy</term><term>Exudating surface nostrils</term><term>Facial</term><term>Facial bones</term><term>Facial region</term><term>Fondazione irccs</term><term>Giant cells</term><term>Good response</term><term>Granda ospedale maggiore policlinico</term><term>Granulomatosis</term><term>Histopathologic traits</term><term>Lesion</term><term>Lesional skin</term><term>Leucocytoclastic vasculitis</term><term>Localized</term><term>Massive destruction</term><term>Nasal</term><term>Nasal cartilage</term><term>Nasal mucosa</term><term>Ndings</term><term>Neutrophil</term><term>Oral azathioprine</term><term>Oral cavity</term><term>Oral cyclophosphamide</term><term>Oral manifestations</term><term>Oral prednisone</term><term>Palatal</term><term>Palatal bone</term><term>Palatal mucosae</term><term>Partial remission</term><term>Persistent disease stage</term><term>Plaque</term><term>Prednisone</term><term>Respiratory tract</term><term>Skin lesion</term><term>Slight improvement</term><term>Smooth surface</term><term>Study group</term><term>Systemic involvement</term><term>Universita degli studi</term><term>Upper airway mucosa</term><term>Vasculitis</term><term>Venereology</term><term>Venereology marzano</term><term>Wegener granulomatosis</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Background Wegener’s granulomatosis (WG) is a rare granulomatous necrotizing vasculitis of small and medium vessels which has predilection for upper airways, lungs and kidney. However, any other organ, including the skin and oral cavity, can be involved. Although mucocutaneous lesions are relatively common, they have only rarely been reported as localized manifestation of the disease. Objectives Our aim was to evaluate the type and sites of skin and mucosal lesions, clinical course and response to treatment, histologic features and laboratory findings in localized WG. Methods The medical records of three patients (two women and one man) with localized WG followed up at our hospitals for a mean time of 10 years were studied. Results All patients presented with facial plaques infiltrating the nasal and palatal mucosae and cartilages and, in one case, perforating the palatal bone. Anti‐neutrophil cytoplasmic antibodies, which are the marker for multisystem WG, were negative. The disease, refractory to various immunosuppressants, responded well, albeit incompletely, to prednisone plus cyclophosphamide. Limitations The limited number of patients is counterbalanced by the rarity of the disease. Conclusions Our cases may represent a rare distinctive subset of WG limited to the facial region and upper airway mucosa but showing a locally aggressive behaviour leading to cartilage and bony destruction.</div></front></TEI><affiliations><list><country><li>Italie</li></country><region><li>Lombardie</li></region><settlement><li>Milan</li></settlement></list><tree><country name="Italie"><region name="Lombardie"><name sortKey="Marzano, A V" sort="Marzano, A V" uniqKey="Marzano A" first="A. V." last="Marzano">A. V. Marzano</name></region><name sortKey="Balice, Y" sort="Balice, Y" uniqKey="Balice Y" first="Y." last="Balice">Y. Balice</name><name sortKey="Berti, E" sort="Berti, E" uniqKey="Berti E" first="E." last="Berti">E. Berti</name><name sortKey="Berti, E" sort="Berti, E" uniqKey="Berti E" first="E." last="Berti">E. Berti</name><name sortKey="Crosti, C" sort="Crosti, C" uniqKey="Crosti C" first="C." last="Crosti">C. Crosti</name><name sortKey="Marzano, A V" sort="Marzano, A V" uniqKey="Marzano A" first="A. V." last="Marzano">A. V. Marzano</name><name sortKey="Papini, M" sort="Papini, M" uniqKey="Papini M" first="M." last="Papini">M. Papini</name><name sortKey="Testa, R" sort="Testa, R" uniqKey="Testa R" first="R." last="Testa">R. Testa</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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