Necrosing livedo reticularis in a patient with recurrent pulmonary hemorrhage
Identifieur interne : 000407 ( Istex/Corpus ); précédent : 000406; suivant : 000408Necrosing livedo reticularis in a patient with recurrent pulmonary hemorrhage
Auteurs : David M. Aronoff ; Jeffrey P. CallenSource :
- Journal of the American Academy of Dermatology [ 0190-9622 ] ; 1997.
English descriptors
- Teeft :
- Alveolar hemorrhage, American academy, Anticardiolipin, Anticoagulant, Antiphospholipid, Antiphospholipid antibodies, Antiphospholipid antibody syndrome, Biopsy specimen, Bland thrombi, Chest examination, Chlorambucil, Cutaneous, Dermatol, Dermatology, Differential diagnosis, Hemorrhage, Hydroxychloroquine, Livedo, Livedo reticularis, Lower extremities, Lung hemorrhage, Lupus, Lupus anticoagulant, Necrosing, Necrosing livedo reticularis, Oral chlorambucil, Pulmonary capillaritis, Pulmonary emboli, Pulmonary hemorrhage, Renal, Reticularis, Rheumatol, Syndrome, Systemic lupus erythematosus, Thrombosis, Ulceration, Venous thrombosis.
Abstract
Abstract: The antiphospholipid antibody (APS) syndrome is characterized by antiphospholipid antibodies (lupus anticoagulant [LA] or anticardiolipin [aCL], a recurrent arterial and venous thrombosis, recurrent fetal loss, and thrombocytopenia. Pulmonary hemorrhage is an unusual complication. We describe a 32-year-old woman with a history of recurrent pulmonary hemorrhage and transient renal dysfunction. Her original diagnosis was Goodpasture’s syndrome, and she was treated with immunosuppressive drugs. Necrosing livedo reticularis of the legs subsequently developed. The presence of aCL and LA in the patient’s serum, the finding of noninflammatory microthrombi in the dermal capillaries, and the lack of laboratory or pathologic features of Goodpasture’s syndrome, confirmed a diagnosis of APS in this patient. (J Am Acad Dermatol 1997;37:300-2.)
Url:
DOI: 10.1016/S0190-9622(97)80375-1
Links to Exploration step
ISTEX:42DFCB9B3F3D6AD387870001483C1528BA44C59ELe document en format XML
<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Necrosing livedo reticularis in a patient with recurrent pulmonary hemorrhage</title><author><name sortKey="Aronoff, David M" sort="Aronoff, David M" uniqKey="Aronoff D" first="David M." last="Aronoff">David M. Aronoff</name></author><author><name sortKey="Callen, Jeffrey P" sort="Callen, Jeffrey P" uniqKey="Callen J" first="Jeffrey P." last="Callen">Jeffrey P. Callen</name></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:42DFCB9B3F3D6AD387870001483C1528BA44C59E</idno><date when="1997" year="1997">1997</date><idno type="doi">10.1016/S0190-9622(97)80375-1</idno><idno type="url">https://api.istex.fr/ark:/67375/6H6-ZQN2MWSX-Z/fulltext.pdf</idno><idno type="wicri:Area/Istex/Corpus">000407</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">000407</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Necrosing livedo reticularis in a patient with recurrent pulmonary hemorrhage</title><author><name sortKey="Aronoff, David M" sort="Aronoff, David M" uniqKey="Aronoff D" first="David M." last="Aronoff">David M. Aronoff</name></author><author><name sortKey="Callen, Jeffrey P" sort="Callen, Jeffrey P" uniqKey="Callen J" first="Jeffrey P." last="Callen">Jeffrey P. Callen</name></author></analytic><monogr></monogr><series><title level="j">Journal of the American Academy of Dermatology</title><title level="j" type="abbrev">YMJD</title><idno type="ISSN">0190-9622</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="1997">1997</date><biblScope unit="volume">37</biblScope><biblScope unit="issue">2</biblScope><biblScope unit="supplement">P2</biblScope><biblScope unit="page" from="300">300</biblScope><biblScope unit="page" to="302">302</biblScope></imprint><idno type="ISSN">0190-9622</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0190-9622</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>Alveolar hemorrhage</term><term>American academy</term><term>Anticardiolipin</term><term>Anticoagulant</term><term>Antiphospholipid</term><term>Antiphospholipid antibodies</term><term>Antiphospholipid antibody syndrome</term><term>Biopsy specimen</term><term>Bland thrombi</term><term>Chest examination</term><term>Chlorambucil</term><term>Cutaneous</term><term>Dermatol</term><term>Dermatology</term><term>Differential diagnosis</term><term>Hemorrhage</term><term>Hydroxychloroquine</term><term>Livedo</term><term>Livedo reticularis</term><term>Lower extremities</term><term>Lung hemorrhage</term><term>Lupus</term><term>Lupus anticoagulant</term><term>Necrosing</term><term>Necrosing livedo reticularis</term><term>Oral chlorambucil</term><term>Pulmonary capillaritis</term><term>Pulmonary emboli</term><term>Pulmonary hemorrhage</term><term>Renal</term><term>Reticularis</term><term>Rheumatol</term><term>Syndrome</term><term>Systemic lupus erythematosus</term><term>Thrombosis</term><term>Ulceration</term><term>Venous thrombosis</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Abstract: The antiphospholipid antibody (APS) syndrome is characterized by antiphospholipid antibodies (lupus anticoagulant [LA] or anticardiolipin [aCL], a recurrent arterial and venous thrombosis, recurrent fetal loss, and thrombocytopenia. Pulmonary hemorrhage is an unusual complication. We describe a 32-year-old woman with a history of recurrent pulmonary hemorrhage and transient renal dysfunction. Her original diagnosis was Goodpasture’s syndrome, and she was treated with immunosuppressive drugs. Necrosing livedo reticularis of the legs subsequently developed. The presence of aCL and LA in the patient’s serum, the finding of noninflammatory microthrombi in the dermal capillaries, and the lack of laboratory or pathologic features of Goodpasture’s syndrome, confirmed a diagnosis of APS in this patient. (J Am Acad Dermatol 1997;37:300-2.)</div></front></TEI><istex><corpusName>elsevier</corpusName><keywords><teeft><json:string>antiphospholipid</json:string><json:string>livedo</json:string><json:string>reticularis</json:string><json:string>lupus</json:string><json:string>pulmonary hemorrhage</json:string><json:string>anticardiolipin</json:string><json:string>antiphospholipid antibody syndrome</json:string><json:string>chlorambucil</json:string><json:string>anticoagulant</json:string><json:string>rheumatol</json:string><json:string>antiphospholipid antibodies</json:string><json:string>livedo reticularis</json:string><json:string>syndrome</json:string><json:string>ulceration</json:string><json:string>necrosing</json:string><json:string>dermatol</json:string><json:string>necrosing livedo reticularis</json:string><json:string>lupus anticoagulant</json:string><json:string>cutaneous</json:string><json:string>dermatology</json:string><json:string>hydroxychloroquine</json:string><json:string>lung hemorrhage</json:string><json:string>thrombosis</json:string><json:string>bland thrombi</json:string><json:string>alveolar hemorrhage</json:string><json:string>oral chlorambucil</json:string><json:string>venous thrombosis</json:string><json:string>lower extremities</json:string><json:string>chest examination</json:string><json:string>american academy</json:string><json:string>differential diagnosis</json:string><json:string>systemic lupus erythematosus</json:string><json:string>biopsy specimen</json:string><json:string>pulmonary emboli</json:string><json:string>pulmonary capillaritis</json:string><json:string>renal</json:string><json:string>hemorrhage</json:string></teeft></keywords><author><json:item><name>David M. Aronoff MD</name></json:item><json:item><name>Jeffrey P. Callen MD</name></json:item></author><arkIstex>ark:/67375/6H6-ZQN2MWSX-Z</arkIstex><language><json:string>eng</json:string></language><originalGenre><json:string>Full-length article</json:string></originalGenre><abstract>Abstract: The antiphospholipid antibody (APS) syndrome is characterized by antiphospholipid antibodies (lupus anticoagulant [LA] or anticardiolipin [aCL], a recurrent arterial and venous thrombosis, recurrent fetal loss, and thrombocytopenia. Pulmonary hemorrhage is an unusual complication. We describe a 32-year-old woman with a history of recurrent pulmonary hemorrhage and transient renal dysfunction. Her original diagnosis was Goodpasture’s syndrome, and she was treated with immunosuppressive drugs. Necrosing livedo reticularis of the legs subsequently developed. The presence of aCL and LA in the patient’s serum, the finding of noninflammatory microthrombi in the dermal capillaries, and the lack of laboratory or pathologic features of Goodpasture’s syndrome, confirmed a diagnosis of APS in this patient. (J Am Acad Dermatol 1997;37:300-2.)</abstract><qualityIndicators><score>4.768</score><pdfWordCount>1400</pdfWordCount><pdfCharCount>9847</pdfCharCount><pdfVersion>1.4</pdfVersion><pdfPageCount>3</pdfPageCount><pdfPageSize>576 x 792 pts</pdfPageSize><refBibsNative>true</refBibsNative><abstractWordCount>114</abstractWordCount><abstractCharCount>852</abstractCharCount><keywordCount>0</keywordCount></qualityIndicators><title>Necrosing livedo reticularis in a patient with recurrent pulmonary hemorrhage</title><pii><json:string>S0190-9622(97)80375-1</json:string></pii><corporate><json:item><name>From the Division of Dermatology, Department of Medicine, University of Louisville, School of Medicine.</name></json:item></corporate><genre><json:string>research-article</json:string></genre><host><title>Journal of the American Academy of Dermatology</title><language><json:string>unknown</json:string></language><publicationDate>1997</publicationDate><issn><json:string>0190-9622</json:string></issn><pii><json:string>S0190-9622(97)X8373-2</json:string></pii><volume>37</volume><issue>2</issue><pages><first>300</first><last>302</last></pages><genre><json:string>journal</json:string></genre></host><namedEntities><unitex><date><json:string>1997</json:string><json:string>16/4/80005</json:string></date><geogName></geogName><orgName><json:string>AmericanAcademyof Dermatology, Inc.</json:string><json:string>Department of Medicine,Universityof Louisville,School of Medicine</json:string><json:string>Division of Dermatology</json:string></orgName><orgName_funder></orgName_funder><orgName_provider></orgName_provider><persName><json:string>E. 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Callen, MD, 310 E. Broadway, #200, Louisville, KY 40202.</note><note>0190-9622/97/$5.00 + 0 16/4/80005</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Necrosing livedo reticularis in a patient with recurrent pulmonary hemorrhage</title><author role="org"><orgName>From the Division of Dermatology, Department of Medicine, University of Louisville, School of Medicine.</orgName></author><author xml:id="author-0000"><persName><forename type="first">David M.</forename><surname>Aronoff</surname></persName><roleName type="degree">MD</roleName></author><author xml:id="author-0001"><persName><forename type="first">Jeffrey P.</forename><surname>Callen</surname></persName><roleName type="degree">MD</roleName></author><idno type="istex">42DFCB9B3F3D6AD387870001483C1528BA44C59E</idno><idno type="ark">ark:/67375/6H6-ZQN2MWSX-Z</idno><idno type="DOI">10.1016/S0190-9622(97)80375-1</idno><idno type="PII">S0190-9622(97)80375-1</idno></analytic><monogr><title level="j">Journal of the American Academy of Dermatology</title><title level="j" type="abbrev">YMJD</title><idno type="pISSN">0190-9622</idno><idno type="PII">S0190-9622(97)X8373-2</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="1997"></date><biblScope unit="volume">37</biblScope><biblScope unit="issue">2</biblScope><biblScope unit="supplement">P2</biblScope><biblScope unit="page" from="300">300</biblScope><biblScope unit="page" to="302">302</biblScope></imprint></monogr></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>1997</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>Abstract: The antiphospholipid antibody (APS) syndrome is characterized by antiphospholipid antibodies (lupus anticoagulant [LA] or anticardiolipin [aCL], a recurrent arterial and venous thrombosis, recurrent fetal loss, and thrombocytopenia. Pulmonary hemorrhage is an unusual complication. We describe a 32-year-old woman with a history of recurrent pulmonary hemorrhage and transient renal dysfunction. Her original diagnosis was Goodpasture’s syndrome, and she was treated with immunosuppressive drugs. Necrosing livedo reticularis of the legs subsequently developed. The presence of aCL and LA in the patient’s serum, the finding of noninflammatory microthrombi in the dermal capillaries, and the lack of laboratory or pathologic features of Goodpasture’s syndrome, confirmed a diagnosis of APS in this patient. (J Am Acad Dermatol 1997;37:300-2.)</p></abstract></profileDesc><revisionDesc><change when="1997">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><extension>txt</extension><original>false</original><mimetype>text/plain</mimetype><uri>https://api.istex.fr/ark:/67375/6H6-ZQN2MWSX-Z/fulltext.txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Elsevier doc found" wicri:toSee="Elsevier, no converted or simple article"><istex:xmlDeclaration>version="1.0" encoding="utf-8"</istex:xmlDeclaration><istex:docType PUBLIC="-//ES//DTD journal article DTD version 5.0.1//EN//XML" URI="art501.dtd" name="istex:docType"><istex:entity SYSTEM="gr1" NDATA="IMAGE" name="gr1"></istex:entity><istex:entity SYSTEM="gr2" NDATA="IMAGE" name="gr2"></istex:entity></istex:docType><istex:document><article docsubtype="fla" xml:lang="en"><item-info><jid>YMJD</jid><aid>97803751</aid><ce:pii>S0190-9622(97)80375-1</ce:pii><ce:doi>10.1016/S0190-9622(97)80375-1</ce:doi><ce:copyright type="other" year="1997">American Academy of Dermatology, Inc</ce:copyright></item-info><head><ce:article-footnote><ce:label>☆</ce:label><ce:note-para>Reprint requests: Jeffrey P. Callen, MD, 310 E. Broadway, #200, Louisville, KY 40202.</ce:note-para></ce:article-footnote><ce:article-footnote><ce:label>☆☆</ce:label><ce:note-para>0190-9622/97/$5.00 + 0 <ce:bold>16/4/80005</ce:bold></ce:note-para></ce:article-footnote><ce:title>Necrosing livedo reticularis in a patient with recurrent pulmonary hemorrhage</ce:title><ce:author-group><ce:author><ce:given-name>David M.</ce:given-name><ce:surname>Aronoff</ce:surname><ce:degrees>MD</ce:degrees></ce:author><ce:author><ce:given-name>Jeffrey P.</ce:given-name><ce:surname>Callen</ce:surname><ce:degrees>MD</ce:degrees></ce:author><ce:collaboration><ce:text>From the Division of Dermatology, Department of Medicine, University of Louisville, School of Medicine.</ce:text></ce:collaboration></ce:author-group><ce:abstract><ce:section-title>Abstract</ce:section-title><ce:abstract-sec><ce:simple-para>The antiphospholipid antibody (APS) syndrome is characterized by antiphospholipid antibodies (lupus anticoagulant [LA] or anticardiolipin [aCL], a recurrent arterial and venous thrombosis, recurrent fetal loss, and thrombocytopenia. Pulmonary hemorrhage is an unusual complication. We describe a 32-year-old woman with a history of recurrent pulmonary hemorrhage and transient renal dysfunction. Her original diagnosis was Goodpasture’s syndrome, and she was treated with immunosuppressive drugs. Necrosing livedo reticularis of the legs subsequently developed. The presence of aCL and LA in the patient’s serum, the finding of noninflammatory microthrombi in the dermal capillaries, and the lack of laboratory or pathologic features of Goodpasture’s syndrome, confirmed a diagnosis of APS in this patient. (J Am Acad Dermatol 1997;37:300-2.)</ce:simple-para></ce:abstract-sec></ce:abstract></head><body><ce:sections><ce:para>The antiphospholipid antibody syndrome (APS) is characterized by circulating antiphospholipid immunoglobins, thrombocytopenia, and intravascular thrombi. <ce:cross-ref refid="bib1"><ce:sup>1</ce:sup></ce:cross-ref> Pulmonary arteritis and renal failure from microvascular thrombosis may also occur. <ce:cross-refs refid="bib2 bib3 bib4 bib5 bib6"><ce:sup>2-6</ce:sup></ce:cross-refs> We describe a patient with APS with recurrent pulmonary hemorrhage and transient hematuria and proteinuria.</ce:para><ce:section><ce:section-title>CASE REPORT</ce:section-title><ce:para>A 32-year-old woman had a sudden massive pulmonary hemorrhage. This was preceded 3 months earlier by myalgia, arthralgia, fever, chills, sweats, and a productive cough with blood-tinged sputum. Examination revealed an obese, febrile (99.8° F), mildly hypertensive (140/95 mm Hg) woman. Chest examination was abnormal and revealed diffusely coarse breath sounds bilaterally.</ce:para><ce:para>Laboratory findings included the following: hematocrit 33% (normal, 35% to 45%), Westergren erythrocyte sedimentation rate 78 mm/hr (normal, 0 to 20 mm/hr), and activated partial thomboplastin time 56 seconds (normal, 22 to 32 seconds). Urinalysis revealed microscopic hematuria and 1+ proteinuria, but no casts. Her blood urea nitrogen and creatinine levels were normal. Antinuclear antibody was positive 1:150 homogeneous, and an anticardiolipin IgG antibody was highly positive at 115.1 GPL units (normal, <20). A chest x-ray examination and computed tomography scan showed bilateral, diffuse, fluffy alveolar infiltrates. Anti-nDNA, anti-Sm, anti-U
<ce:inf>1</ce:inf> RNP, and anti-Ro (SS-A) antibodies were absent.</ce:para><ce:para>The patient received parenteral antibiotics for a possible pneumonia, and her symptoms abated. During the next several months she continued to have intermittent hemoptysis. A slightly elevated antiglomerular basement membrane antibody was noted and a working diagnosis of Goodpasture’s syndrome was made despite nonspecific histologic and immunopathologic findings in lung and kidney biopsy specimens. She was treated with prednisone, 60 mg/day, and cyclophosphamide, 100 mg/day. She had another episode of massive pulmonary hemorrhage accompanied by acute respiratory failure. Livedo reticularis was noted on her palms and soles. Her cyclophosphamide and prednisone therapy was continued in varying doses for the next 2 years. Her condition gradually improved but was complicated by hemorrhagic cystitis. Oral chlorambucil, 6 mg/day, was substituted for the cyclophosphamide. Her prednisone dosage was slowly reduced and eventually stopped, as was the chlorambucil.</ce:para><ce:para>Thereafter, the patient noted painful skin lesions on her legs and feet. Examination revealed a reticulated pattern of purpura on the dorsum of the feet that extended onto her toes bilaterally. She also had some areas of erythema and small ulcerations on the medial aspect of both lower extremities just above the ankles. Two-plus pitting edema was also noted on both lower extremities.</ce:para><ce:para>A biopsy specimen demonstrated bland thrombi within numerous capillary-sized vessels within the dermis (<ce:cross-ref refid="fig1">Fig. 1</ce:cross-ref>) .
<ce:display><ce:figure id="fig1"><ce:label>Fig. 1</ce:label><ce:caption><ce:simple-para>Biopsy specimen showing noninflammatory, bland thrombi filling multiple small vessels in dermis. (Hematoxylin-eosin stain; original magnification ×100.)</ce:simple-para></ce:caption> <ce:link locator="gr1"></ce:link></ce:figure></ce:display>Extravasated red blood cells and hemosiderin-laden macrophages were also noted. There was no inflammation or fibrinous degeneration of the vessel walls. Immunofluorescence microscopy was nonspecific. Laboratory studies for cryoglobulins and cryofibrinogen were negative, but the anticardiolipin IgG level was elevated.</ce:para><ce:para>These findings led to a diagnosis of primary APS. The patient was treated with oral enteric coated aspirin, 325 mg daily.</ce:para><ce:para>One year later she noted progressive small ulcerations of the skin on her ankles. Examination revealed slightly palpable, purpuric reticulated areas bilaterally on the legs, with more prominent erosions and crusts over the right medial malleolus (<ce:cross-ref refid="fig2">Fig. 2</ce:cross-ref>) .
<ce:display><ce:figure id="fig2"><ce:label>Fig. 2</ce:label><ce:caption><ce:simple-para>Purpuric livedo reticularis with small areas of necrosis and ulceration.</ce:simple-para></ce:caption> <ce:link locator="gr2"></ce:link></ce:figure></ce:display>She was again given oral chlorambucil, 4 mg daily, with oral hydroxychloroquine, 200 mg twice a day.</ce:para><ce:para>Six months later, her cutaneous lesions were inactive. The chlorambucil was stopped, and aspirin and hydroxychloroquine were continued. She has had no further episodes of pulmonary hemorrhage or evidence of respiratory, renal, or cardiac dysfunction, but her skin lesions reactivated 6 months later.</ce:para></ce:section><ce:section><ce:section-title>DISCUSSION</ce:section-title><ce:para>APS is characterized by antiphospholipid antibodies (lupus anticoagulant or anticardiolipin), arterial and venous thrombosis, recurrent fetal loss, and thrombocytopenia. <ce:cross-ref refid="bib1"><ce:sup>1</ce:sup></ce:cross-ref> A variety of cutaneous manifestations occur in this syndrome. <ce:cross-ref refid="bib7"><ce:sup>7</ce:sup></ce:cross-ref> Livedo reticularis and leg ulcerations are both common. <ce:cross-ref refid="bib7"><ce:sup>7</ce:sup></ce:cross-ref> Pulmonary hemorrhage has been uncommonly associated with APS. <ce:cross-refs refid="bib2 bib3 bib4 bib5 bib6"><ce:sup>2-6</ce:sup></ce:cross-refs></ce:para><ce:para>Conditions that can manifest as livedo reticularis have in common an impairment of the cutaneous circulation. The differential diagnosis of necrosing livedo reticularis includes APS, cryoglobulinemia, polyarteritis nodosa, cryofibrinogenemia, and Wegener’s granulomatosis. Sneddon’s syndrome, a triad of livedo reticularis, hypertension, and neurologic thrombotic events, can also present with necrosing livedo reticularis. <ce:cross-ref refid="bib8"><ce:sup>8</ce:sup></ce:cross-ref> Emboli resulting from cholesterol crystals, atrial myxoma, or bacterial endocarditis may produce similar lesions.</ce:para><ce:para>The differential diagnosis of pulmonary hemorrhage includes focal diseases of the lung panenchyma and airways, as well as diffuse processes involving the pulmonary microvasculature and alveoli. Autoimmune diseases such as Goodpasture’s syndrome, Wegener’s granulomatosis, and systemic lupus erythematosus can involve multiple systems, including the lungs and kidneys. The combination of lung hemorrhage and nephritis is commonly presumed to be Goodpasture’s syndrome. However in a recent report by Niles et al., <ce:cross-ref refid="bib9"><ce:sup>9</ce:sup></ce:cross-ref> the majority of patients were found to have antineutrophil cytoplasmic antibodies rather than antiglomerular basement membrane antibodies. In a minority of patients, including those with an antineutrophil cytoplasmic antibody, Wegener’s granulomatosis was not found to be the cause of the pulmonary-renal syndrom. Within this report there were two patients with APS, one of whom had pulmonary emboli. Other causes of lung hemorrhage with nephritis include microscopic poly-arteritis, Henoch-Schönlein purpura, and mixed cryoglobulinemia.</ce:para><ce:para>Pulmonary hemorrhage in APS may be multifactorial. In a recent review, Asherson and Cervera <ce:cross-ref refid="bib10"><ce:sup>10</ce:sup></ce:cross-ref> identified eight reported pulmonary processes that complicate APS. We were unable to demonstrate evidence of pulmonary emboli or coagulopathy (other than APS) in our patient, and because her pulmonary, cardiac, and renal functions were not permanently impaired, we postulate that intraalveolar pulmonary hemorrhage was the cause.</ce:para><ce:para>The optimal treatment for patients with APS is unclear. The major manifestations of the illness result from recurrent arterial and venous thromboses. The cause of the thrombosis is not well understood. <ce:cross-ref refid="bib11"><ce:sup>11</ce:sup></ce:cross-ref> Evidence exists that the antiphospholipid antibodies themselves are directly harmful, but other studies suggest that the antibodies are merely markers for an underlying abnormality of a coagulation protein or platelets or endothelial cells. Given the lack of understanding of its pathogenesis, APS is often treated with an empiric regimen of immunosuppressive, antiplatelet, and anticoagulant drugs. <ce:cross-ref refid="bib12"><ce:sup>12</ce:sup></ce:cross-ref></ce:para><ce:para>The treatment of the leg ulcers in patients with APS is also varied. The successful use of intralesional corticosteroid injections has been reported. <ce:cross-ref refid="bib13"><ce:sup>13</ce:sup></ce:cross-ref> Heparin, in combination with intravenous thrombolytics such as urokinase and tissue plasminogen activator, has also been reported as effective. <ce:cross-ref refid="bib14"><ce:sup>14</ce:sup></ce:cross-ref> Our patient was treated with hydroxychloroquine and chlorambucil. 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Rheum</sb:maintitle></sb:title><sb:volume-nr>37</sb:volume-nr></sb:series><sb:date>1994</sb:date></sb:issue><sb:pages><sb:first-page>S297</sb:first-page></sb:pages></sb:host></sb:reference></ce:bib-reference></ce:bibliography-sec></ce:bibliography></tail></article></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Necrosing livedo reticularis in a patient with recurrent pulmonary hemorrhage</title></titleInfo><titleInfo type="alternative" lang="en" contentType="CDATA"><title>Necrosing livedo reticularis in a patient with recurrent pulmonary hemorrhage</title></titleInfo><name type="corporate"><namePart>From the Division of Dermatology, Department of Medicine, University of Louisville, School of Medicine.</namePart></name><name type="personal"><namePart type="given">David M.</namePart><namePart type="family">Aronoff</namePart><namePart type="termsOfAddress">MD</namePart><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Jeffrey P.</namePart><namePart type="family">Callen</namePart><namePart type="termsOfAddress">MD</namePart><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="research-article" displayLabel="Full-length article" authority="ISTEX" authorityURI="https://content-type.data.istex.fr" valueURI="https://content-type.data.istex.fr/ark:/67375/XTP-1JC4F85T-7">research-article</genre><originInfo><publisher>ELSEVIER</publisher><dateIssued encoding="w3cdtf">1997</dateIssued><copyrightDate encoding="w3cdtf">1997</copyrightDate></originInfo><language><languageTerm type="code" authority="iso639-2b">eng</languageTerm><languageTerm type="code" authority="rfc3066">en</languageTerm></language><abstract lang="en">Abstract: The antiphospholipid antibody (APS) syndrome is characterized by antiphospholipid antibodies (lupus anticoagulant [LA] or anticardiolipin [aCL], a recurrent arterial and venous thrombosis, recurrent fetal loss, and thrombocytopenia. Pulmonary hemorrhage is an unusual complication. We describe a 32-year-old woman with a history of recurrent pulmonary hemorrhage and transient renal dysfunction. Her original diagnosis was Goodpasture’s syndrome, and she was treated with immunosuppressive drugs. Necrosing livedo reticularis of the legs subsequently developed. The presence of aCL and LA in the patient’s serum, the finding of noninflammatory microthrombi in the dermal capillaries, and the lack of laboratory or pathologic features of Goodpasture’s syndrome, confirmed a diagnosis of APS in this patient. (J Am Acad Dermatol 1997;37:300-2.)</abstract><note>Reprint requests: Jeffrey P. Callen, MD, 310 E. Broadway, #200, Louisville, KY 40202.</note><note>0190-9622/97/$5.00 + 0 16/4/80005</note><relatedItem type="host"><titleInfo><title>Journal of the American Academy of Dermatology</title></titleInfo><titleInfo type="abbreviated"><title>YMJD</title></titleInfo><genre type="journal" authority="ISTEX" authorityURI="https://publication-type.data.istex.fr" valueURI="https://publication-type.data.istex.fr/ark:/67375/JMC-0GLKJH51-B">journal</genre><originInfo><publisher>ELSEVIER</publisher><dateIssued encoding="w3cdtf">1997</dateIssued></originInfo><identifier type="ISSN">0190-9622</identifier><identifier type="PII">S0190-9622(97)X8373-2</identifier><part><date>1997</date><detail type="volume"><number>37</number><caption>vol.</caption></detail><detail type="issue"><number>2</number><caption>no.</caption></detail><detail type="supplement"><number>P2</number><caption>Suppl.</caption></detail><extent unit="issue-pages"><start>295</start><end>364</end></extent><extent unit="pages"><start>300</start><end>302</end></extent></part></relatedItem><identifier type="istex">42DFCB9B3F3D6AD387870001483C1528BA44C59E</identifier><identifier type="ark">ark:/67375/6H6-ZQN2MWSX-Z</identifier><identifier type="DOI">10.1016/S0190-9622(97)80375-1</identifier><identifier type="PII">S0190-9622(97)80375-1</identifier><accessCondition type="use and reproduction" contentType="copyright">©1997 American Academy of Dermatology, Inc</accessCondition><recordInfo><recordContentSource authority="ISTEX" authorityURI="https://loaded-corpus.data.istex.fr" valueURI="https://loaded-corpus.data.istex.fr/ark:/67375/XBH-HKKZVM7B-M">elsevier</recordContentSource><recordOrigin>American Academy of Dermatology, Inc, ©1997</recordOrigin></recordInfo></mods><json:item><extension>json</extension><original>false</original><mimetype>application/json</mimetype><uri>https://api.istex.fr/ark:/67375/6H6-ZQN2MWSX-Z/record.json</uri></json:item></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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