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Rapid Evolving Unilateral Indurated Oozing Facial Plaques in a Patient with Head-and-Neck Cancer: Peripheral T-Cell Lymphoma Not Otherwise Specified (NOS)

Identifieur interne : 002361 ( Pmc/Corpus ); précédent : 002360; suivant : 002362

Rapid Evolving Unilateral Indurated Oozing Facial Plaques in a Patient with Head-and-Neck Cancer: Peripheral T-Cell Lymphoma Not Otherwise Specified (NOS)

Auteurs : Uwe Wollina ; Gesina Hansel ; Dana Langner ; André Koch ; Jacqueline Schönlebe ; Georgi Tchernev

Source :

RBID : PMC:5535662

Abstract

BACKGROUND

The sudden development of facial plaques and nodules may be an alarming clinical sign for underlying malignancies. Nevertheless, a broad range of inflammatory and infectious diseases must be considered as well in the differential diagnosis.

CASE REPORT

We report on a 53-year-old male patient with a left-sided cheek infiltration with oozing but no lymphadenopathy. He had a medical history of head-and-neck cancer. The primary differential diagnosis was herpes zoster with secondary impetiginization or pyoderma facial. About eight weeks later, the patient presented with progressive formation of nodules and plaques on the face and isotretinoin was stopped. Skin biopsy suggested mycosis fungoid and an oral treatment with bexarotene was started. After limited response for another eight weeks, he returned later with massive facial swelling, nodules and impetiginization. Another skin biopsy was performed to exclude diagnostic error or investigate possible disease progression. Microscopic evaluation and multiplex-polymerase chain reaction confirmed the diagnosis of peripheral T-cell lymphoma, not otherwise specified (PTL-NOS), stage Ia (T1 N0 M0). Imaging techniques excluded metastatic spread. By interdisciplinary tumour board, R-CHOP (rituximab, cyclophosphamide, hydroxyl-doxorubicin, vincristine, and prednisolone) was recommended and initiated by hemato-oncologists.

CONCLUSIONS

PLT-NOS confirmed in the present patient has a poor prognosis with a 5-year survival rate of less than 20%.


Url:
DOI: 10.3889/oamjms.2017.085
PubMed: 28785337
PubMed Central: 5535662

Links to Exploration step

PMC:5535662

Le document en format XML

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</sec>
<sec id="st2">
<title>CASE REPORT</title>
<p>We report on a 53-year-old male patient with a left-sided cheek infiltration with oozing but no lymphadenopathy. He had a medical history of head-and-neck cancer. The primary differential diagnosis was herpes zoster with secondary impetiginization or pyoderma facial. About eight weeks later, the patient presented with progressive formation of nodules and plaques on the face and isotretinoin was stopped. Skin biopsy suggested mycosis fungoid and an oral treatment with bexarotene was started. After limited response for another eight weeks, he returned later with massive facial swelling, nodules and impetiginization. Another skin biopsy was performed to exclude diagnostic error or investigate possible disease progression. Microscopic evaluation and multiplex-polymerase chain reaction confirmed the diagnosis of peripheral T-cell lymphoma, not otherwise specified (PTL-NOS), stage Ia (T1 N0 M0). Imaging techniques excluded metastatic spread. By interdisciplinary tumour board, R-CHOP (rituximab, cyclophosphamide, hydroxyl-doxorubicin, vincristine, and prednisolone) was recommended and initiated by hemato-oncologists.</p>
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<title>CONCLUSIONS</title>
<p>PLT-NOS confirmed in the present patient has a poor prognosis with a 5-year survival rate of less than 20%.</p>
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<pmc-dir>properties open_access</pmc-dir>
<front>
<journal-meta>
<journal-id journal-id-type="nlm-ta">Open Access Maced J Med Sci</journal-id>
<journal-id journal-id-type="iso-abbrev">Open Access Maced J Med Sci</journal-id>
<journal-title-group>
<journal-title>Open Access Macedonian Journal of Medical Sciences</journal-title>
</journal-title-group>
<issn pub-type="epub">1857-9655</issn>
<publisher>
<publisher-name>ID Design 2012/DOOEL Skopje</publisher-name>
<publisher-loc>Republic of Macedonia</publisher-loc>
</publisher>
</journal-meta>
<article-meta>
<article-id pub-id-type="pmid">28785337</article-id>
<article-id pub-id-type="pmc">5535662</article-id>
<article-id pub-id-type="publisher-id">OAMJMS-5-476</article-id>
<article-id pub-id-type="doi">10.3889/oamjms.2017.085</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Case Report</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>Rapid Evolving Unilateral Indurated Oozing Facial Plaques in a Patient with Head-and-Neck Cancer: Peripheral T-Cell Lymphoma Not Otherwise Specified (NOS)</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname>Wollina</surname>
<given-names>Uwe</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
<xref ref-type="corresp" rid="cor1">*</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Hansel</surname>
<given-names>Gesina</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Langner</surname>
<given-names>Dana</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Koch</surname>
<given-names>André</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Schönlebe</surname>
<given-names>Jacqueline</given-names>
</name>
<xref ref-type="aff" rid="aff2">2</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Tchernev</surname>
<given-names>Georgi</given-names>
</name>
<xref ref-type="aff" rid="aff3">3</xref>
</contrib>
</contrib-group>
<aff id="aff1">
<label>1</label>
<italic>Department of Dermatology and Allergology, “Georg Schmorl”, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067 Dresden, Germany</italic>
</aff>
<aff id="aff2">
<label>2</label>
<italic>Institute of Pathology, “Georg Schmorl”, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067 Dresden, Germany</italic>
</aff>
<aff id="aff3">
<label>3</label>
<italic>Department of Dermatology, Venereology and Dermatologic Surgery, Medical Institute of Ministry of Interior, and Onkoderma Policlinic for Dermatology and Dermatologic Surgery, Sofia, Bulgaria</italic>
</aff>
<author-notes>
<corresp id="cor1">
<label>*</label>
<bold>Correspondence:</bold>
Uwe Wollina. Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067 Dresden, Germany. E-mail:
<email xlink:href="wollina-uw@khdf.de">wollina-uw@khdf.de</email>
</corresp>
</author-notes>
<pub-date pub-type="collection">
<day>25</day>
<month>7</month>
<year>2017</year>
</pub-date>
<pub-date pub-type="epub">
<day>19</day>
<month>7</month>
<year>2017</year>
</pub-date>
<volume>5</volume>
<issue>4</issue>
<fpage>476</fpage>
<lpage>479</lpage>
<history>
<date date-type="received">
<day>31</day>
<month>3</month>
<year>2017</year>
</date>
<date date-type="rev-recd">
<day>05</day>
<month>5</month>
<year>2017</year>
</date>
<date date-type="accepted">
<day>06</day>
<month>5</month>
<year>2017</year>
</date>
</history>
<permissions>
<copyright-statement>Copyright: © 2017 Uwe Wollina, Gesina Hansel, Dana Langner, André Koch, Jacqueline Schönlebe, Georgi Tchernev.</copyright-statement>
<copyright-year>2017</copyright-year>
<license license-type="open-access" xlink:href="http://creativecommons.org/licenses/CC BY-NC/4.0">
<license-p>This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).</license-p>
</license>
</permissions>
<abstract>
<sec id="st1">
<title>BACKGROUND</title>
<p>The sudden development of facial plaques and nodules may be an alarming clinical sign for underlying malignancies. Nevertheless, a broad range of inflammatory and infectious diseases must be considered as well in the differential diagnosis.</p>
</sec>
<sec id="st2">
<title>CASE REPORT</title>
<p>We report on a 53-year-old male patient with a left-sided cheek infiltration with oozing but no lymphadenopathy. He had a medical history of head-and-neck cancer. The primary differential diagnosis was herpes zoster with secondary impetiginization or pyoderma facial. About eight weeks later, the patient presented with progressive formation of nodules and plaques on the face and isotretinoin was stopped. Skin biopsy suggested mycosis fungoid and an oral treatment with bexarotene was started. After limited response for another eight weeks, he returned later with massive facial swelling, nodules and impetiginization. Another skin biopsy was performed to exclude diagnostic error or investigate possible disease progression. Microscopic evaluation and multiplex-polymerase chain reaction confirmed the diagnosis of peripheral T-cell lymphoma, not otherwise specified (PTL-NOS), stage Ia (T1 N0 M0). Imaging techniques excluded metastatic spread. By interdisciplinary tumour board, R-CHOP (rituximab, cyclophosphamide, hydroxyl-doxorubicin, vincristine, and prednisolone) was recommended and initiated by hemato-oncologists.</p>
</sec>
<sec id="st3">
<title>CONCLUSIONS</title>
<p>PLT-NOS confirmed in the present patient has a poor prognosis with a 5-year survival rate of less than 20%.</p>
</sec>
</abstract>
<kwd-group>
<kwd>Peripheral T-cell lymphoma – not otherwise specified</kwd>
<kwd>Chemotherapy</kwd>
<kwd>R-CHOP</kwd>
<kwd>Facial plaques and nodules</kwd>
<kwd>Herpes</kwd>
<kwd>Bexarotene</kwd>
<kwd>Differential diagnosis</kwd>
</kwd-group>
</article-meta>
</front>
<body>
<sec sec-type="intro" id="sec1-1">
<title>Introduction</title>
<p>Sudden development of indurated facial plaques refers to a broader range of benign but also serious life threatening diseases. Various inflammatory disorders are warranting consideration in the differential diagnosis.</p>
<p>Pyoderma facial or rosacea fulminant is characterised by erythematous indurated and painful plaques, sometimes oozing. It can develop unilaterally but is a rare diagnosis in males in contrast to females [
<xref rid="ref1" ref-type="bibr">1</xref>
]. Sweet syndrome is a rare neutrophilic disease with rapid onset, fever, neutrophilic leukocytosis and painful cutaneous plaques, which may involve the face [
<xref rid="ref2" ref-type="bibr">2</xref>
].</p>
<p>Well’s syndrome or eosinophilic cellulitis is a rare disorder characterised by flame figures of eosinophils in dermal tissue. Pruritic cellulitis-like plaques may masquerade as bacterial facial infection [
<xref rid="ref3" ref-type="bibr">3</xref>
]. Granuloma faciale is an uncommon, chronic inflammatory disorder initially described as eosinophilic granuloma, responding to corticosteroids, and shares the apple jelly phenomenon on diascopy with sarcoidosis [
<xref rid="ref4" ref-type="bibr">4</xref>
].</p>
<p>Histiocytic diseases may affect the facial skin. Benign cutaneous Rosai-Dorfman disease - a rare, non-Langerhans cell histiocytosis, affects the facial skin in about 11% of cases with erythematous plaques [
<xref rid="ref5" ref-type="bibr">5</xref>
]. Disseminated xanthogranuloma typically involves periorbital skin [
<xref rid="ref6" ref-type="bibr">6</xref>
].</p>
<p>Among infectious disorders, cutaneous leishmaniasis is emerging in non-epidemic areas. The disease is caused by vector-borne protozoal cutaneous infection by several species of
<italic>Leishmania</italic>
transmitted by sandflies [
<xref rid="ref7" ref-type="bibr">7</xref>
,
<xref rid="ref8" ref-type="bibr">8</xref>
].</p>
<p>In the case of immunosuppression, bacterial papillomatosis and severe facial soft tissue infections may develop [
<xref rid="ref9" ref-type="bibr">9</xref>
]. Invasive zygomycosis is another potentially fatal infection [
<xref rid="ref10" ref-type="bibr">10</xref>
]. Malignancies need to be taken into consideration in any atypical case of facial nodular plaque-type affection, as the following case report illustrates.</p>
</sec>
<sec id="sec1-2">
<title>Case presentation</title>
<p>A 53-year-old male patient was admitted to our hospital in July 2016 because of a left sided cheek infiltration with oozing. We observed an oozing indurated nodular plaque of about 6 x 8 cm (
<xref ref-type="fig" rid="F1">Fig. 1A</xref>
) but no lymphadenopathy. The primary differential diagnosis was herpes zoster with secondary impetiginization or pyoderma facial.</p>
<fig id="F1" position="float">
<label>Figure 1</label>
<caption>
<p>Peripheral T-cell lymphoma, not otherwise specified. (A) Initial presentation resembling herpes infection; (B) Worsening with an aspect of mycosis fungoides; (C) Further progression with massive lid oedema and secondary impetiginization</p>
</caption>
<graphic xlink:href="OAMJMS-5-476-g001"></graphic>
</fig>
<p>The patient was treated initially with oral acyclovir 5 x 800 mg /d plus 100 mg prednisolone/d (with tapering the dose) for seven days. Oozing diminished but infiltrates were still present.</p>
<p>After that isotretinoin therapy was initiated. Topical treatment consisted of disinfectant washings and fusidinic acid ointment. The marked lymphedema could be explained by a history of head-and-neck cancer (T2 N2 M0 G2 V1 R0) the year before with surgery, neck dissection and radiotherapy.</p>
<p>The patient returned in September 2016 (
<xref ref-type="fig" rid="F1">Fig. 1B</xref>
). Since there was progress with nodules and lid oedema, isotretinoin was stopped, and a skin biopsy was performed. The working diagnosis was mycosis fungoides. Based on the cutaneous T-cell lymphoma (CTCL) diagnosis the patient was discussed in the interdisciplinary tumour board. Radiotherapy was impossible due to the previous radiotherapy of head-and-neck cancer. Therefore, oral treatment with 525 mg/ d bexarotene was started. There was progress in December leading to a cessation of bexarotene (
<xref ref-type="fig" rid="F1">Fig. 1C</xref>
).</p>
<p>Another skin biopsy was performed. There was a dense inflammatory infiltrate in dermis and subcutis, but no interface dermatitis and no epidermal infiltration. Hair follicles were partly destroyed. The infiltrate consisted mostly of medium-sized polymorphous cells with notched nuclei and numerous mitoses. Some small lymphocytic cells and individual plasma cells and mast cells were intermingled (
<xref ref-type="fig" rid="F2">Fig. 2</xref>
).</p>
<fig id="F2" position="float">
<label>Figure 2</label>
<caption>
<p>Histologic investigations. (a) Dense dermal infiltrate (hematoxylin-eosin x 4); (b) Detail – mononuclear cells with cellular atypia and atypical mitoses (hematoxylin-eosin x 10); (c) Strong expression of CD3 (immunoperoxidase x 10); (d) CD8 expression (immunoperoxidase x 10); (e) Beta-F1 expression (immunoperoxidase x 4; (f) Ki67 for proliferating cells (immunoperoxidase x 4)</p>
</caption>
<graphic xlink:href="OAMJMS-5-476-g002"></graphic>
</fig>
<p>Immunohistological findings are summarised in
<xref ref-type="table" rid="T1">Table 1</xref>
. After DNA extraction multiplex-polymerase chain reaction (PCR) had been performed to investigate clonality of cells (Institute of Pathology, University of Kiel, Germany) (
<xref ref-type="table" rid="T2">Table 2</xref>
). Monoclonality only could be demonstrated for T-cells, not B-cells.</p>
<table-wrap id="T1" position="float">
<label>Table 1</label>
<caption>
<p>Immunohistological findings</p>
</caption>
<table frame="hsides" rules="groups" width="100%">
<thead>
<tr>
<th align="left" rowspan="1" colspan="1">Marker</th>
<th align="center" rowspan="1" colspan="1">Reactivity</th>
</tr>
</thead>
<tbody>
<tr>
<td align="left" rowspan="1" colspan="1">CD1a</td>
<td align="center" rowspan="1" colspan="1">+ (for single cells only)</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">CD3</td>
<td align="center" rowspan="1" colspan="1">+++</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">CD4</td>
<td align="center" rowspan="1" colspan="1">(+)</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">CD5</td>
<td align="center" rowspan="1" colspan="1">+</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">CD7</td>
<td align="center" rowspan="1" colspan="1">+++</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">CD8</td>
<td align="center" rowspan="1" colspan="1">+++</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">CD10</td>
<td align="center" rowspan="1" colspan="1">-</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">CD20</td>
<td align="center" rowspan="1" colspan="1">+ (focally in the surrounding tissue by small lymphocytes)</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">CD30</td>
<td align="center" rowspan="1" colspan="1">-</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">CD56</td>
<td align="center" rowspan="1" colspan="1">-</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">CD68</td>
<td align="center" rowspan="1" colspan="1">+ (for single histiocytes only)</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">Bcl-6</td>
<td align="center" rowspan="1" colspan="1">-</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">Beta-F1 (T-cell receptor beta chain)</td>
<td align="center" rowspan="1" colspan="1">+++</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">Cyclin-D1</td>
<td align="center" rowspan="1" colspan="1">-</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">Ki67</td>
<td align="center" rowspan="1" colspan="1">++ (up to 60% of medium-sized cells)</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">PD1</td>
<td align="center" rowspan="1" colspan="1">+</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">Perforin</td>
<td align="center" rowspan="1" colspan="1">-</td>
</tr>
</tbody>
</table>
</table-wrap>
<table-wrap id="T2" position="float">
<label>Table 2</label>
<caption>
<p>Multiplex-PCR (bp – base pair; negative means polyclonality instead of monoclonality)</p>
</caption>
<table frame="hsides" rules="groups" width="100%">
<thead>
<tr>
<th align="left" rowspan="1" colspan="1">Beta-chain T-cell receptor gene</th>
<th align="center" rowspan="1" colspan="1"></th>
<th align="center" rowspan="1" colspan="1">monoclonality</th>
</tr>
</thead>
<tbody>
<tr>
<td align="left" rowspan="1" colspan="1"></td>
<td align="center" rowspan="1" colspan="1">A-multiplex PCR</td>
<td align="center" rowspan="1" colspan="1">247 & 248 bp</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1"></td>
<td align="center" rowspan="1" colspan="1">B-multiplex PCR</td>
<td align="center" rowspan="1" colspan="1">253 & 261 bp</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1"></td>
<td align="center" rowspan="1" colspan="1">C-multiplex PCR</td>
<td align="center" rowspan="1" colspan="1">193 & 303 bp</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">Gamma-chain T-cell receptor gene</td>
<td align="center" rowspan="1" colspan="1"></td>
<td align="center" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1"></td>
<td align="center" rowspan="1" colspan="1">va-multiplex PCR</td>
<td align="center" rowspan="1" colspan="1">negative</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1"></td>
<td align="center" rowspan="1" colspan="1">vb-multiplex PCR</td>
<td align="center" rowspan="1" colspan="1">negative</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1">Immunoglobulin heavy chain gene</td>
<td align="center" rowspan="1" colspan="1"></td>
<td align="center" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1"></td>
<td align="center" rowspan="1" colspan="1">F1-multiplex PCR</td>
<td align="center" rowspan="1" colspan="1">negative</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1"></td>
<td align="center" rowspan="1" colspan="1">F2-multiplex PCR</td>
<td align="center" rowspan="1" colspan="1">negative</td>
</tr>
<tr>
<td align="left" rowspan="1" colspan="1"></td>
<td align="center" rowspan="1" colspan="1">F3-multiplex PCR</td>
<td align="center" rowspan="1" colspan="1">negative</td>
</tr>
</tbody>
</table>
</table-wrap>
<p>Imaging with diagnostic ultrasound and thoracic X-ray excluded metastatic spread. However, a parotic adenoma was identified. Laboratory investigations revealed an increased ratio of CD3+CD4+/ CD3+CD8+ of 3.56 (normal range: 1.0-2.3).</p>
<p>The diagnosis of peripheral T-cell lymphoma, not otherwise specified (PTL-NOS), stage Ia (T1 N0 M0), was confirmed.</p>
<p>By interdisciplinary tumour board, R-CHOP (rituximab, cyclophosphamide, hydroxyl-doxorubicin, vincristine, and prednisolone) was recommended and initiated in January by hemato-oncologists. The treatment is continued.</p>
</sec>
<sec sec-type="discussion" id="sec1-3">
<title>Discussion</title>
<p>Peripheral T-cell lymphoma (PTL) NOS is a rare but aggressive malignancy. The initial stages often resemble non-malignant plaque-type or nodular dermatoses as in the present case. These primary lesions may become infected and imitate an infectious disease [
<xref rid="ref11" ref-type="bibr">11</xref>
].</p>
<p>In the differential diagnosis, other lymphomas need to be considered. The most common CTCL – mycosis fungoides - was the first suspicion. The aggressive course and the missing epidermotropism of atypical cells argued against. Because of the dominance of CD8+ lymphocytes in the second biopsy, primary cutaneous CD8-positive aggressive epidermotropic T-cell lymphoma (CD8+ AECTCL) had to be considered. AECTL is characterised by rapidly evolving erosive or necrotic plaques and nodules. Epidermotropic infiltrates of CD8+ atypical lymphocytes are the hallmark of CD8+ AECTCL, which exhibits a poor prognosis [
<xref rid="ref12" ref-type="bibr">12</xref>
]. Again, in the present case, there was no epidermotropism at all, confirming the diagnosis of PTL-NOS.</p>
<p>PTL-NOS may present with single nodules or nodular plaques but tends to disseminate rapidly. Responses to radio- and chemotherapy a short-lived. Our patient has achieved a partial remission during R-CHOP therapy [Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Vincristine (Oncovin®), and Prednisolone].</p>
<p>Negative prognostic factors for PTL-NOS are age > 60, Eastern Cooperative Oncology Group (ECOG) performance status of ≥ 2, lactate dehydrogenase levels at normal values or above, and involvement of the bone marrow are independent predictors of decreased survival [
<xref rid="ref13" ref-type="bibr">13</xref>
]. Prognosis is poor even with stem cell transplantation and systemic chemotherapy and radiotherapy with a 5-year survival rate of less than 20% [
<xref rid="ref14" ref-type="bibr">14</xref>
].</p>
<p>In conclusion, sudden development of facial plaques and nodules can be a red flag of unknown malignancy. Even if routine laboratory tests are unremarkable and lymphadenopathy is absent, an aggressive lymphoma-like PTL-NOS in this case, may be present. Repeated skin biopsies are needed to confirm or exclude diagnosis. PTL-NOS, however, has a poor prognosis not improved substantially by systemic treatment.</p>
</sec>
</body>
<back>
<fn-group>
<fn fn-type="supported-by">
<p>
<bold>Funding:</bold>
This research did not receive any financial support.</p>
</fn>
<fn fn-type="other">
<p>
<bold>Competing Interests:</bold>
The authors have declared that no competing interests exist.</p>
</fn>
</fn-group>
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<given-names>E</given-names>
</name>
</person-group>
<article-title>Peripheral T-cell lymphomas, unspecified (or not otherwise specified):a review</article-title>
<source>Hematol Oncol</source>
<year>2008</year>
<volume>26</volume>
<fpage>8</fpage>
<lpage>20</lpage>
<comment>https://doi.org/10.1002/hon.836 PMid:18050364</comment>
<pub-id pub-id-type="pmid">18050364</pub-id>
</element-citation>
</ref>
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