<p>Madam,</p> <p>A 39-year-old woman presented to the Dermatology Clinic at Azahra Hospital Center with a 6-month history of intermittently painful, small cluster of subcutaneous nodules and papules of varying sizes ranging from 0.5 cm to 2 cm in diameter on the lateral aspects of the face that were sensitive to cold weather <xref ref-type="fig" rid="F1">Figure 1</xref>. The pseudo-Darier sign was positive. On detailed inquiry, she gave a history of menorrhagia 2 years ago that on sonography uterine leiomyomatosis (UL) has been proposed. After myomectomy, on histopathology UL has been confirmed. Otherwise, her medical, occupational, and family history were normal. <fig id="F1"> <label>Figure 1</label> <caption> <p>Multiple papules and nodules of varying sizes ranging from 0.5 cm to 2 cm in diameter on the lateral aspect of the face</p> </caption> <alt-text>Figure 1</alt-text> <graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="JResMedSci_2013_18_11_1011_124792_u1.tif" /> </fig></p> <p>Laboratory testing revealed no significant finding. Pathology samples with H and E staining showed a circumscribed, non-encapsulated proliferation of eosinophilic spindle cells with uniform pencil-shaped nuclei in the papillary and upper reticular dermis. Rare normal mitosis has been detected <xref ref-type="fig" rid="F2">Figure 2</xref>. The immunohistochemistry staining revealed positive staining with smooth muscle actin antibody, confirming the smooth muscle origin of the lesions. These pathology findings were compatible with cutaneous leiomyomatosis (CL). Therapy with nifedipine markedly results in controlling pain. <fig id="F2"> <label>Figure 2</label> <caption> <p>Circumscribed, non-encapsulated proliferation of eosinophilic spindle cells with uniform pencil-shaped nuclei</p> </caption> <alt-text>Figure 2</alt-text> <graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="JResMedSci_2013_18_11_1011_124792_u2.tif" /> </fig></p> <p>Owing to the possibility of occurrence of renal cancer (RC) with the simultaneous multiple CL and UL, <sup> <xref ref-type="bibr" rid="ref1">1</xref> </sup>, <sup> <xref ref-type="bibr" rid="ref6">6</xref> </sup>we considered the evaluation of genitourinary apparatus. However, on abdominopelvic sonography, and urine biochemistry no finding in favor of RC was detected.</p> <p>CL is a benign neoplasm originating from smooth muscle that presents by painful nodules. Despite the existence of three variants, the most common presentation is the multiple piloleiomyomatosis that the lesions commonly distributed in a grouped or dermatomal pattern. Even though, CL do not progress to malignant transformation into leiomyosarcoma, the main threat is the occurrence of RC especially when there is simultaneous multiple CL and UL. <sup> <xref ref-type="bibr" rid="ref1">1</xref> </sup>, <sup> <xref ref-type="bibr" rid="ref2">2</xref> </sup></p> <p>In about 75% of patients with multiple leiomyomas, a mutation in the fumarate hydratase on chromosome 1q42.3-43 has been reported. This mutation is strongly persistent in cases in which RC occur. <sup> <xref ref-type="bibr" rid="ref3">3</xref> </sup>, <sup> <xref ref-type="bibr" rid="ref4">4</xref> </sup>, <sup> <xref ref-type="bibr" rid="ref5">5</xref> </sup></p> <p>In our patient, despite the coexistence of CL and UL, work-up for detecting RC, was negative. This finding was predictable due to the fact that RC has association more dominantly in cases in which there is a positive family history of leiomyoma. <sup> <xref ref-type="bibr" rid="ref6">6</xref> </sup>However, one should always look for the rare internal associations of cutaneous lesions even when the patient does not complain of a significant problem.</p> </sec> </body> <back> <ref-list> <ref id="ref1"> <label>1</label> <nlm-citation citation-type="book"> <person-group person-group-type="author"> <name> <surname>James</surname> <given-names>WD</given-names> </name> <name> <surname>Berger</surname> <given-names>TG</given-names> </name> <name> <surname>Elston</surname> <given-names>DM</given-names> </name> </person-group> <article-title>China: Mosby Publishing; 2011</article-title> <source></source> <publisher-loc>Mosby Publishing</publisher-loc> <publisher-name>China</publisher-name> <year>2011</year> <volume></volume> <fpage>614</fpage> <lpage>6</lpage> </nlm-citation> </ref> <ref id="ref2"> <label>2</label> <nlm-citation citation-type="book"> <person-group person-group-type="author"> <name> <surname>Bolognia</surname> <given-names>J</given-names> </name> <name> <surname>Jorizzo</surname> <given-names>J</given-names> </name> <name> <surname>Rapini</surname> <given-names>R</given-names> </name> </person-group> <article-title>Spain: Mosby Publishing; 2008</article-title> <source></source> <publisher-loc>Mosby Publishing</publisher-loc> <publisher-name>Spain</publisher-name> <year>2008</year> <volume></volume> <fpage>1831</fpage> <lpage>44</lpage> </nlm-citation> </ref> <ref id="ref3"> <label>3</label> <nlm-citation citation-type="journal"> <person-group person-group-type="author"> <name> <surname>Martinez-Mir</surname> <given-names>A</given-names> </name> <name> <surname>Gordon</surname> <given-names>D</given-names> </name> <name> <surname>Horev</surname> <given-names>L</given-names> </name> <name> <surname>Klapholz</surname> <given-names>L</given-names> </name> <name> <surname>Ott</surname> <given-names>J</given-names> </name> <name> <surname>Christiano</surname> <given-names>AM</given-names> </name> <etal /> </person-group> <article-title>Multiple cutaneous and uterine leiomyomas: Refinement of the genetic locus for multiple cutaneous and uterine leiomyomas on chromosome 1q42.3-43</article-title> <source>J Invest Dermatol</source> <year>2002</year> <volume>118</volume> <fpage>876</fpage> <lpage>80</lpage> </nlm-citation> </ref> <ref id="ref4"> <label>4</label> <nlm-citation citation-type="journal"> <person-group person-group-type="author"> <name> <surname>Badeloe</surname> <given-names>S</given-names> </name> <name> <surname>Frank</surname> <given-names>J</given-names> </name> </person-group> <article-title>Clinical and molecular genetic aspects of hereditary multiple cutaneous leiomyomatosis</article-title> <source>Eur J Dermatol</source> <year>2009</year> <volume>19</volume> <fpage>545</fpage> <lpage>51</lpage> </nlm-citation> </ref> <ref id="ref5"> <label>5</label> <nlm-citation citation-type="journal"> <person-group person-group-type="author"> <name> <surname>Alam</surname> <given-names>NA</given-names> </name> <name> <surname>Olpin</surname> <given-names>S</given-names> </name> <name> <surname>Leigh</surname> <given-names>IM</given-names> </name> </person-group> <article-title>Fumarate hydratase mutations and predisposition to cutaneous leiomyomas, uterine leiomyomas and renal cancer</article-title> <source>Br J Dermatol</source> <year>2005</year> <volume>153</volume> <fpage>11</fpage> <lpage>7</lpage> </nlm-citation> </ref> <ref id="ref6"> <label>6</label> <nlm-citation citation-type="book"> <person-group person-group-type="author"> <name> <surname>White</surname> <given-names>LE</given-names> </name> <name> <surname>Levy</surname> <given-names>RM</given-names> </name> <name> <surname>Alam</surname> <given-names>M</given-names> </name> </person-group> <article-title>Fitzpatricks Dermatology in General Medicine</article-title> <source></source> <publisher-loc>McGraw Hill</publisher-loc> <publisher-name>US</publisher-name> <year>2008</year> <volume></volume> <fpage>1172</fpage> <lpage>3</lpage> </nlm-citation> </ref> <ref id="ref7"> <label>7</label> <nlm-citation citation-type="journal"> <person-group person-group-type="author"></person-group> <article-title></article-title> <source></source> <year></year> <volume></volume> <fpage></fpage> </nlm-citation> </ref> </ref-list> </back> </article>

JRMS

J Res Med Sci

Journal of Research in Medical Sciences

1735-1995 1735-7136

Medknow Publications Pvt Ltd

India

JRMS-18-1011

24520236

Letter to Editor

Multiple cutaneous and uterine leiomyomas

Asilian

Ali

Momeni

Iman

Tajmirriahi

Nabet

Farhadi

Sadaf

Department of Dermatology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran Department of Dermatology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran Department of Dermatology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran Department of Dermatology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran

Address for correspondence:Nabet Tajmirriahi, Department of Dermatology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran nabettajmeerriahi@yahoo.com

November 2013

18 11 1011 1012

2013

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

<p>Madam,</p> <p>A 39-year-old woman presented to the Dermatology Clinic at Azahra Hospital Center with a 6-month history of intermittently painful, small cluster of subcutaneous nodules and papules of varying sizes ranging from 0.5 cm to 2 cm in diameter on the lateral aspects of the face that were sensitive to cold weather <xref ref-type="fig" rid="F1">Figure 1</xref>. The pseudo-Darier sign was positive. On detailed inquiry, she gave a history of menorrhagia 2 years ago that on sonography uterine leiomyomatosis (UL) has been proposed. After myomectomy, on histopathology UL has been confirmed. Otherwise, her medical, occupational, and family history were normal. <fig id="F1"> <label>Figure 1</label> <caption> <p>Multiple papules and nodules of varying sizes ranging from 0.5 cm to 2 cm in diameter on the lateral aspect of the face</p> </caption> <alt-text>Figure 1</alt-text> <graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="JResMedSci_2013_18_11_1011_124792_u1.tif" /> </fig></p> <p>Laboratory testing revealed no significant finding. Pathology samples with H and E staining showed a circumscribed, non-encapsulated proliferation of eosinophilic spindle cells with uniform pencil-shaped nuclei in the papillary and upper reticular dermis. Rare normal mitosis has been detected <xref ref-type="fig" rid="F2">Figure 2</xref>. The immunohistochemistry staining revealed positive staining with smooth muscle actin antibody, confirming the smooth muscle origin of the lesions. These pathology findings were compatible with cutaneous leiomyomatosis (CL). Therapy with nifedipine markedly results in controlling pain. <fig id="F2"> <label>Figure 2</label> <caption> <p>Circumscribed, non-encapsulated proliferation of eosinophilic spindle cells with uniform pencil-shaped nuclei</p> </caption> <alt-text>Figure 2</alt-text> <graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="JResMedSci_2013_18_11_1011_124792_u2.tif" /> </fig></p> <p>Owing to the possibility of occurrence of renal cancer (RC) with the simultaneous multiple CL and UL, <sup> <xref ref-type="bibr" rid="ref1">1</xref> </sup>, <sup> <xref ref-type="bibr" rid="ref6">6</xref> </sup>we considered the evaluation of genitourinary apparatus. However, on abdominopelvic sonography, and urine biochemistry no finding in favor of RC was detected.</p> <p>CL is a benign neoplasm originating from smooth muscle that presents by painful nodules. Despite the existence of three variants, the most common presentation is the multiple piloleiomyomatosis that the lesions commonly distributed in a grouped or dermatomal pattern. Even though, CL do not progress to malignant transformation into leiomyosarcoma, the main threat is the occurrence of RC especially when there is simultaneous multiple CL and UL. <sup> <xref ref-type="bibr" rid="ref1">1</xref> </sup>, <sup> <xref ref-type="bibr" rid="ref2">2</xref> </sup></p> <p>In about 75% of patients with multiple leiomyomas, a mutation in the fumarate hydratase on chromosome 1q42.3-43 has been reported. This mutation is strongly persistent in cases in which RC occur. <sup> <xref ref-type="bibr" rid="ref3">3</xref> </sup>, <sup> <xref ref-type="bibr" rid="ref4">4</xref> </sup>, <sup> <xref ref-type="bibr" rid="ref5">5</xref> </sup></p> <p>In our patient, despite the coexistence of CL and UL, work-up for detecting RC, was negative. This finding was predictable due to the fact that RC has association more dominantly in cases in which there is a positive family history of leiomyoma. <sup> <xref ref-type="bibr" rid="ref6">6</xref> </sup>However, one should always look for the rare internal associations of cutaneous lesions even when the patient does not complain of a significant problem.</p> </sec> </body> <back> <ref-list> <ref id="ref1"> <label>1</label> <nlm-citation citation-type="book"> <person-group person-group-type="author"> <name> <surname>James</surname> <given-names>WD</given-names> </name> <name> <surname>Berger</surname> <given-names>TG</given-names> </name> <name> <surname>Elston</surname> <given-names>DM</given-names> </name> </person-group> <article-title>China: Mosby Publishing; 2011</article-title> <source></source> <publisher-loc>Mosby Publishing</publisher-loc> <publisher-name>China</publisher-name> <year>2011</year> <volume></volume> <fpage>614</fpage> <lpage>6</lpage> </nlm-citation> </ref> <ref id="ref2"> <label>2</label> <nlm-citation citation-type="book"> <person-group person-group-type="author"> <name> <surname>Bolognia</surname> <given-names>J</given-names> </name> <name> <surname>Jorizzo</surname> <given-names>J</given-names> </name> <name> <surname>Rapini</surname> <given-names>R</given-names> </name> </person-group> <article-title>Spain: Mosby Publishing; 2008</article-title> <source></source> <publisher-loc>Mosby Publishing</publisher-loc> <publisher-name>Spain</publisher-name> <year>2008</year> <volume></volume> <fpage>1831</fpage> <lpage>44</lpage> </nlm-citation> </ref> <ref id="ref3"> <label>3</label> <nlm-citation citation-type="journal"> <person-group person-group-type="author"> <name> <surname>Martinez-Mir</surname> <given-names>A</given-names> </name> <name> <surname>Gordon</surname> <given-names>D</given-names> </name> <name> <surname>Horev</surname> <given-names>L</given-names> </name> <name> <surname>Klapholz</surname> <given-names>L</given-names> </name> <name> <surname>Ott</surname> <given-names>J</given-names> </name> <name> <surname>Christiano</surname> <given-names>AM</given-names> </name> <etal /> </person-group> <article-title>Multiple cutaneous and uterine leiomyomas: Refinement of the genetic locus for multiple cutaneous and uterine leiomyomas on chromosome 1q42.3-43</article-title> <source>J Invest Dermatol</source> <year>2002</year> <volume>118</volume> <fpage>876</fpage> <lpage>80</lpage> </nlm-citation> </ref> <ref id="ref4"> <label>4</label> <nlm-citation citation-type="journal"> <person-group person-group-type="author"> <name> <surname>Badeloe</surname> <given-names>S</given-names> </name> <name> <surname>Frank</surname> <given-names>J</given-names> </name> </person-group> <article-title>Clinical and molecular genetic aspects of hereditary multiple cutaneous leiomyomatosis</article-title> <source>Eur J Dermatol</source> <year>2009</year> <volume>19</volume> <fpage>545</fpage> <lpage>51</lpage> </nlm-citation> </ref> <ref id="ref5"> <label>5</label> <nlm-citation citation-type="journal"> <person-group person-group-type="author"> <name> <surname>Alam</surname> <given-names>NA</given-names> </name> <name> <surname>Olpin</surname> <given-names>S</given-names> </name> <name> <surname>Leigh</surname> <given-names>IM</given-names> </name> </person-group> <article-title>Fumarate hydratase mutations and predisposition to cutaneous leiomyomas, uterine leiomyomas and renal cancer</article-title> <source>Br J Dermatol</source> <year>2005</year> <volume>153</volume> <fpage>11</fpage> <lpage>7</lpage> </nlm-citation> </ref> <ref id="ref6"> <label>6</label> <nlm-citation citation-type="book"> <person-group person-group-type="author"> <name> <surname>White</surname> <given-names>LE</given-names> </name> <name> <surname>Levy</surname> <given-names>RM</given-names> </name> <name> <surname>Alam</surname> <given-names>M</given-names> </name> </person-group> <article-title>Fitzpatricks Dermatology in General Medicine</article-title> <source></source> <publisher-loc>McGraw Hill</publisher-loc> <publisher-name>US</publisher-name> <year>2008</year> <volume></volume> <fpage>1172</fpage> <lpage>3</lpage> </nlm-citation> </ref> <ref id="ref7"> <label>7</label> <nlm-citation citation-type="journal"> <person-group person-group-type="author"></person-group> <article-title></article-title> <source></source> <year></year> <volume></volume> <fpage></fpage> </nlm-citation> </ref> </ref-list> </back> </article>