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Solid facial edema preceding a diagnosis of retro-orbital B-cell lymphoma

Identifieur interne : 005F21 ( Istex/Corpus ); précédent : 005F20; suivant : 005F22

Solid facial edema preceding a diagnosis of retro-orbital B-cell lymphoma

Auteurs : Laryssa R. Dragan ; Joseph M. Baron ; Scott Stern ; James C. Shaw

Source :

RBID : ISTEX:CB10C1A56300B04BC3348B35BF692A0044755233

Abstract

Persistent solid facial edema is a rare condition of unknown cause. Although acute facial edema has been associated with numerous disease processes such as infections, neoplasms, immune disorders, inflammation, neuropathic processes, drugs, mechanical obstructions, and trauma, solid facial edema has most often been associated with acne vulgaris. We report the first case, to our knowledge, of solid facial edema preceding a diagnosis of a subcutaneous scalp and orbital/periorbital B-cell lymphoma. (J Am Acad Dermatol 2000;42:872-4.)

Url:
DOI: 10.1016/S0190-9622(00)90258-5

Links to Exploration step

ISTEX:CB10C1A56300B04BC3348B35BF692A0044755233

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<ce:note-para>Reprint requests: James C. Shaw, MD, University of Chicago, Section of Dermatology, 5841 S Maryland Ave, MC 5067, Chicago, IL 60637. E-mail address: jshaw@medicine.bsd.uchicago.edu</ce:note-para>
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<ce:title>Solid facial edema preceding a diagnosis of retro-orbital B-cell lymphoma</ce:title>
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<ce:given-name>Laryssa R.</ce:given-name>
<ce:surname>Dragan</ce:surname>
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<ce:simple-para>Persistent solid facial edema is a rare condition of unknown cause. Although acute facial edema has been associated with numerous disease processes such as infections, neoplasms, immune disorders, inflammation, neuropathic processes, drugs, mechanical obstructions, and trauma, solid facial edema has most often been associated with acne vulgaris. We report the first case, to our knowledge, of solid facial edema preceding a diagnosis of a subcutaneous scalp and orbital/periorbital B-cell lymphoma. (J Am Acad Dermatol 2000;42:872-4.)</ce:simple-para>
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<ce:section-title>CASE REPORT</ce:section-title>
<ce:para>A 70-year-old white man presented for evaluation of persistent erythema and edema involving the cheeks, which had been present for 18 months (Fig 1,
<ce:italic>A</ce:italic>
).
<ce:display>
<ce:figure id="fig1">
<ce:label>Fig. 1</ce:label>
<ce:caption>
<ce:simple-para>
<ce:bold>A,</ce:bold>
Firm, erythematous swelling of face was noted on patient’s cheeks bilaterally.
<ce:bold>B,</ce:bold>
Biopsy specimen from right cheek revealed markedly edematous dermis.</ce:simple-para>
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A biopsy specimen from the right cheek (which was more swollen than the left cheek) revealed a somewhat thinned epidermis overlying a markedly edematous-appearing dermis, findings consistent with solid facial edema (
<ce:cross-ref refid="fig1">Fig 1,
<ce:italic>B</ce:italic>
</ce:cross-ref>
).</ce:para>
<ce:para>On subsequent visits examination of the patient revealed that in addition to persistent facial edema, swelling had developed in the temporal regions bilaterally. A new finding of significant bilateral proptosis, which was more prominent on the right, was also noted. The patient also reported that he had developed intermittent diplopia and ocular pain.</ce:para>
<ce:para>A magnetic resonance image revealed multiple enhancing masses involving both orbits, the periorbital regions, and the region of the lacrimal gland. Multiple subcutaneous lesions of the scalp were also noted. Biopsy specimens from the left temporal and right frontal scalp revealed a normal-appearing epidermis and dermis, and subcutaneous dense, monomorphic infiltrates of small lymphocytes with round to slightly irregular nuclei and moderate amounts of clear cytoplasm (Fig 2).
<ce:display>
<ce:figure>
<ce:label>Fig. 2</ce:label>
<ce:caption>
<ce:simple-para>Histology from lesion on patient’s temporal area shows subcutaneous nodule composed of lymphoid cells.</ce:simple-para>
</ce:caption>
<ce:link locator="gr2"></ce:link>
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Immunohistochemical stains revealed that most of the lymphocytes were of B-cell lineage (CD20 positive, CD5 negative). A bone marrow biopsy specimen revealed multiple atypical lymphoid aggregates, phenotypically composed of both B and T lymphocytes, which were suspicious for lymphoma because of their multifocality. The process appeared to be low grade and consistent with the diagnosis of marginal zone lymphoma. During the search for the extent of lymph-adenopathy, a 1.5-cm left kidney mass was noted on computed tomography of the abdomen. A surgical wedge resection of the mass revealed it to be a renal cell carcinoma. This procedure was considered definitive treatment for the tumor.</ce:para>
<ce:para>After establishment of the patient’s diagnosis of B-cell lymphoma on the basis of subcutaneous nodule biopsies, and presumed involvement of the orbit and periorbital areas with the same process, the patient was treated with a palliative course of radiotherapy to his involved scalp (2200 cGy over 2 weeks) and bilateral orbits (3000 cGy over 3 weeks). Slow, but dramatic improvement in the patient’s solid facial edema was seen over approximately 6 months after treatment, although some very slight residual soft swelling and mild erythema remains in the right cheek. There has been significant resolution of his ocular symptoms as well.</ce:para>
</ce:section>
<ce:section>
<ce:section-title>DISCUSSION</ce:section-title>
<ce:para>Although acute facial edema has been associated with numerous disease processes,
<ce:cross-refs refid="bib1 bib2 bib3 bib4 bib5 bib6 bib7 bib8 bib9 bib10 bib11 bib12 bib13 bib14">
<ce:sup>1-14</ce:sup>
</ce:cross-refs>
solid facial edema is a rare entity most often associated with acne.
<ce:cross-refs refid="bib15 bib16 bib17 bib18 bib19 bib20 bib21">
<ce:sup>15-21</ce:sup>
</ce:cross-refs>
The name “solid facial edema,” from Connelly and Winkelmann
<ce:cross-ref refid="bib17">
<ce:sup>17</ce:sup>
</ce:cross-ref>
reflects a firm, nonpitting edema usually involving the central face. Although intermittent or acute facial edema has been reported with malignancy, there are no published reports of solid facial edema in association with a B-cell lymphoma, such as that seen in our patient.</ce:para>
<ce:para>Treatments for solid facial edema have included prednisone, isotretinoin, ketotifen, and clofazimine.
<ce:cross-refs refid="bib15 bib16 bib17 bib18 bib19 bib20 bib21">
<ce:sup>15-21</ce:sup>
</ce:cross-refs>
Although improvement has been reported with these drugs, recurrence is common after treatment is stopped, and many cases persist during treatment.
<ce:cross-ref refid="bib16">
<ce:sup>16</ce:sup>
</ce:cross-ref>
Systemic antibiotics generally have not been successful.</ce:para>
<ce:para>Although several hypotheses have been proposed regarding the pathogenesis of solid facial edema, the cause remains unknown. Speculation exists that the edema is produced by repeated inflammation, which leads to obstruction of lymphatic vessels or fibrosis.
<ce:cross-refs refid="bib15 bib17">
<ce:sup>15,17</ce:sup>
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An interesting report of solid facial edema occurring in twins raises the possibility that genetic factors may also play a role in the pathogenesis.
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In addition, a leiomyosarcoma in the superior vena cava and azygos vein has been described as causing facial edema, as has Kaposi’s sarcoma.
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In a report of recurrent facial edema associated with a T-cell lymphoma of a cervical lymph node, the authors propose that the edema could be secondary to secretion of cytokines by tumor, rather than from mechanical lymphatic obstruction by the tumor.
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In each of the aforementioned cases, the facial edema resolved when the primary process was treated with chemotherapy, radiation, or surgery.</ce:para>
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<titleInfo lang="en">
<title>Solid facial edema preceding a diagnosis of retro-orbital B-cell lymphoma</title>
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<titleInfo type="alternative" lang="en" contentType="CDATA">
<title>Solid facial edema preceding a diagnosis of retro-orbital B-cell lymphoma</title>
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<name type="personal">
<namePart type="given">Laryssa R.</namePart>
<namePart type="family">Dragan</namePart>
<namePart type="termsOfAddress">MDa</namePart>
<affiliation>Chicago, IllinoisFrom the Section of Dermatology,a Section of Hematology and Oncology,b and Department of Medicine,c University of Chicago</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
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</name>
<name type="personal">
<namePart type="given">Joseph M.</namePart>
<namePart type="family">Baron</namePart>
<namePart type="termsOfAddress">MDb</namePart>
<affiliation>Chicago, IllinoisFrom the Section of Dermatology,a Section of Hematology and Oncology,b and Department of Medicine,c University of Chicago</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Scott</namePart>
<namePart type="family">Stern</namePart>
<namePart type="termsOfAddress">MDc</namePart>
<affiliation>Chicago, IllinoisFrom the Section of Dermatology,a Section of Hematology and Oncology,b and Department of Medicine,c University of Chicago</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">James C.</namePart>
<namePart type="family">Shaw</namePart>
<namePart type="termsOfAddress">MDa</namePart>
<affiliation>Chicago, IllinoisFrom the Section of Dermatology,a Section of Hematology and Oncology,b and Department of Medicine,c University of Chicago</affiliation>
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<abstract lang="en">Persistent solid facial edema is a rare condition of unknown cause. Although acute facial edema has been associated with numerous disease processes such as infections, neoplasms, immune disorders, inflammation, neuropathic processes, drugs, mechanical obstructions, and trauma, solid facial edema has most often been associated with acne vulgaris. We report the first case, to our knowledge, of solid facial edema preceding a diagnosis of a subcutaneous scalp and orbital/periorbital B-cell lymphoma. (J Am Acad Dermatol 2000;42:872-4.)</abstract>
<note>This supplement is made possible through an educational grant from Ortho Dermatological to the American Academy of Dermatology.</note>
<note>Reprint requests: James C. Shaw, MD, University of Chicago, Section of Dermatology, 5841 S Maryland Ave, MC 5067, Chicago, IL 60637. E-mail address: jshaw@medicine.bsd.uchicago.edu</note>
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<title>Journal of the American Academy of Dermatology</title>
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<title>YMJD</title>
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<originInfo>
<dateIssued encoding="w3cdtf">200005</dateIssued>
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<identifier type="ISSN">0190-9622</identifier>
<identifier type="PII">S0190-9622(00)X0121-1</identifier>
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<date>200005</date>
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<number>42</number>
<caption>vol.</caption>
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<number>5</number>
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<end>938</end>
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<extent unit="pages">
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<accessCondition type="use and reproduction" contentType="copyright">©2000 American Academy of Dermatology, Inc</accessCondition>
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