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1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Additional tests</span><p id="par0015" class="elsevierStylePara elsevierViewall">Surgical resection of the lesion was performed by shaving of the lesion&#46; Histopathology revealed a well demarcated cystic structure covered with a single layer of columnar cells with a ciliated surface &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Immune staining was positive for epithelial membrane antigen &#40;EMA&#41;&#44; estrogen receptor&#44; and cytokeratin 7 &#40;CK7&#41;&#44; and negative for progesterone receptor&#44; cytokeratin 20 &#40;CK20&#41;&#44; and carcinoembryonic antigen &#40;CEA&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0020" class="elsevierStylePara elsevierViewall">Ciliated vulvar cyst&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course</span><p id="par0025" class="elsevierStylePara elsevierViewall">The patient has remained asymptomatic and has presented no recurrence of the lesion after 6 months of follow-up&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Discussion</span><p id="par0030" class="elsevierStylePara elsevierViewall">Ciliated vulvar cysts &#40;CVC&#41; are a rarely reported entity that normally appears in young women&#44; although it has been reported in women of all ages&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;6</span></a> It has been linked to pregnancy and progesterone use&#44; and some authors consider it a variant of the ciliated cutaneous cyst&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> The etiology is unknown&#44; although different origins have been suggested&#58; remains of Mullerian ducts &#40;paramesonephric origin&#41;&#44; vestiges of the terminal portion of the Wolffian ducts &#40;mesonephric origin&#41;&#44; the urogenital sinus&#44; or the sweat glands&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> The most widely accepted theory&#44; however&#44; is Mullerian heterotopia&#44; due to the sequestration and migration of these tissues during embryo development&#46; This theory is based on the histologic and immunohistochemical similarities with the Mullerian epithelium&#44; and other lesions derived from it&#44; such as retroperitoneal Mullerian cysts or transitional cell carcinoma of the fallopian tube&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">CVC presents as a solitary lesion of variable size that typically affects the upper portion of the labia minora&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;6</span></a> Histopathology reveals a cystic structure demarcated by a single layer of columnar or cuboid ciliated cells&#46; Immune staining is normally positive for EMA&#44; ER&#44; progesterone receptor&#44; and CK7&#44; and negative for S-100&#44; CEA&#44; CK20&#44; and smooth-muscle actin&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2&#44;5&#44;6</span></a> The differential diagnosis includes other vulvar cystic lesions&#44; such as Bartholin&#8217;s cyst&#44; papilliferum hidradenoma&#44; cyst of Nuck&#44; inclusion cyst&#44; and vestibular mucinous cyst&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a> The differential diagnosis with Bartholin&#8217;s cysts is of note due to their frequency&#46; These cysts are normally found in the middle-lower portion of the labia minora and may have an acinar origin &#40;nonciliated columnar epithelium&#41; or ductal origin &#40;transitional or squamous epithelium&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The treatment of choice is surgical&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;6</span></a> The lesions may grow and become disfiguring&#44; especially in young girls due to the smaller size of the anatomical structures&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3&#44;4</span></a> No cases of local recurrence after surgery or of malignant transformation have been reported&#44; 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Case for Diagnosis
Cystic Vulvar Lesion
Lesión quística vulvar
S. Poncea,
Corresponding author
sauloponce90@gmail.com

Corresponding author.
, T. Montenegrob, D. Camesellea
a Servicio de Dermatología, Complejo Hospitalario Universitario Insular – Materno Infantil, Las Palmas de Gran Canaria, Spain
b Servicio de Anatomía Patológica, Complejo HospitalarioUniversitario Insular – Materno Infantil, Las Palmas deGran Canaria, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 21-year-old woman with no relevant past history and who was only undergoing treatment with oral contraceptives visited our department with a vulvar lesion that had appeared 2 months earlier&#59; the lesion had gradually grown and was causing discomfort on contact with clothing&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed a pediculated papule in the transition between the clitoris and labia minora&#59; the lesion had a maximum diameter of 0&#46;6<span class="elsevierStyleHsp" style=""></span>cm and was brownish in color with a firm consistency &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Additional tests</span><p id="par0015" class="elsevierStylePara elsevierViewall">Surgical resection of the lesion was performed by shaving of the lesion&#46; Histopathology revealed a well demarcated cystic structure covered with a single layer of columnar cells with a ciliated surface &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Immune staining was positive for epithelial membrane antigen &#40;EMA&#41;&#44; estrogen receptor&#44; and cytokeratin 7 &#40;CK7&#41;&#44; and negative for progesterone receptor&#44; cytokeratin 20 &#40;CK20&#41;&#44; and carcinoembryonic antigen &#40;CEA&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0020" class="elsevierStylePara elsevierViewall">Ciliated vulvar cyst&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course</span><p id="par0025" class="elsevierStylePara elsevierViewall">The patient has remained asymptomatic and has presented no recurrence of the lesion after 6 months of follow-up&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Discussion</span><p id="par0030" class="elsevierStylePara elsevierViewall">Ciliated vulvar cysts &#40;CVC&#41; are a rarely reported entity that normally appears in young women&#44; although it has been reported in women of all ages&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;6</span></a> It has been linked to pregnancy and progesterone use&#44; and some authors consider it a variant of the ciliated cutaneous cyst&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> The etiology is unknown&#44; although different origins have been suggested&#58; remains of Mullerian ducts &#40;paramesonephric origin&#41;&#44; vestiges of the terminal portion of the Wolffian ducts &#40;mesonephric origin&#41;&#44; the urogenital sinus&#44; or the sweat glands&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> The most widely accepted theory&#44; however&#44; is Mullerian heterotopia&#44; due to the sequestration and migration of these tissues during embryo development&#46; This theory is based on the histologic and immunohistochemical similarities with the Mullerian epithelium&#44; and other lesions derived from it&#44; such as retroperitoneal Mullerian cysts or transitional cell carcinoma of the fallopian tube&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">CVC presents as a solitary lesion of variable size that typically affects the upper portion of the labia minora&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;6</span></a> Histopathology reveals a cystic structure demarcated by a single layer of columnar or cuboid ciliated cells&#46; Immune staining is normally positive for EMA&#44; ER&#44; progesterone receptor&#44; and CK7&#44; and negative for S-100&#44; CEA&#44; CK20&#44; and smooth-muscle actin&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2&#44;5&#44;6</span></a> The differential diagnosis includes other vulvar cystic lesions&#44; such as Bartholin&#8217;s cyst&#44; papilliferum hidradenoma&#44; cyst of Nuck&#44; inclusion cyst&#44; and vestibular mucinous cyst&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a> The differential diagnosis with Bartholin&#8217;s cysts is of note due to their frequency&#46; These cysts are normally found in the middle-lower portion of the labia minora and may have an acinar origin &#40;nonciliated columnar epithelium&#41; or ductal origin &#40;transitional or squamous epithelium&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The treatment of choice is surgical&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;6</span></a> The lesions may grow and become disfiguring&#44; especially in young girls due to the smaller size of the anatomical structures&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3&#44;4</span></a> No cases of local recurrence after surgery or of malignant transformation have been reported&#44; although follow-up is very limited in the published cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;6</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In conclusion&#44; we present a new case of CVC&#44; a rare entity with only 20 cases described in the literature to date&#46; We also report the 5th case with an immunohistochemical study&#44; with findings similar to those of the other 4 previously published cases&#44; which support the theory of paramesonephric origin due to remains of Mullerian epithelium&#46; It is important for dermatologists and gynecologists to be aware of this entity&#44; with emphasis on the importance of histopathology and immune staining in vulvar cystic lesions&#46; A greater number of cases and a more extensive study of these lesions are required to determine their true etiology&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of interest</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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Article information
ISSN: 15782190
Original language: English
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Idiomas
Actas Dermo-Sifiliográficas
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