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1</a>&#41;&#44; with occasional papules in the right axilla&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">No other areas of the body were affected&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Dermoscopy revealed well-delimited&#44; homogenous&#44; whitish papules with some dotted vessels at the periphery&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">A 4-mm punch biopsy taken from a lesion on the neck showed slightly thickened collagen fibers in the mid dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; No calcium deposits were observed and staining of the elastic fibers revealed no abnormality&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis&#63;</p><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Diagnosis</span><p id="par0035" class="elsevierStylePara elsevierViewall">Fibroelastolytic papulosis of the neck&#46;</p></span></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Treatment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Given the benign nature of the lesions and the fact that they were asymptomatic&#44; 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which they named pseudoxanthoma elasticum-like papillary dermal elastolysis&#44; in which they observed yellowish papules resembling those of white fibrous papulosis&#46; In this case&#44; however&#44; the papules showed a tendency to coalesce&#44; giving rise to cobblestone-like plaques suggestive of pseudoxanthoma elasticum &#40;PXE&#41;&#46; Histologically the lesions were mainly characterized by elastolysis&#44; with no calcium deposits or fibrosis&#44; and were categorized as elastic fiber abnormalities&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Balus et al&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> subsequently discovered common clinical and histologic findings when reviewing cases reported for both entities alongside 20 of their own&#46; They concluded that there were 2 variants of a single condition that they named fibroelastolytic papulosis of the neck&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The exact cause of fibroelastolytic papulosis of the neck is unknown&#44; although most authors have related it to the skin aging process&#46; Recently cases have also been reported in younger patients with a history of inflammatory diseases in the same area&#46; This suggests that factors other than aging&#44; such as the inflammation of skin adnexa&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> can exert an influence on the subsequent appearance of fibrosis and elastolysis&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">Diagnosis is based on clinical presentation&#44; histologic findings&#44; and&#44; more recently&#44; dermoscopy&#44; which shows well-delimited&#44; whitish papules with some dotted vessels at the periphery&#44; as well as pigment that fails to form a true reticulated area&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">The case reported here is more compatible with what was originally known as white fibrous papulosis of the neck&#46; This definitive diagnosis was established from the clinical presentation and dermoscopy&#44; but particularly after the exclusion of other entities&#44; such as PXE&#44; on pathology study of the biopsy&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">In conclusion&#44; knowledge of fibroelastolytic papulosis of the neck is important as it bears a strong clinical resemblance to PXE&#46; Unlike PXE&#44; however&#44; prognosis is good and not associated with heart or ophthalmological disease&#46;</p></span></span>"
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Case for Diagnosis
Cobblestone Papules on the Neck
Pápulas en empedrado en el cuello
B. Echeverría-Garcíaa,
Corresponding author
Begoecheverria2@gmail.com

Corresponding author.
, V. Parrab, J.A. Avilésa, R. Suárez-Fernándeza
a Servicio de Dermatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain
b Servicio de Anatomía Patológica, Hospital General Universitario Gregorio Marañón, Madrid, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 65-year-old woman with a 13-year-history of primary biliary cirrhosis&#44; systemic hypertension&#44; dyslipidemia&#44; and osteoporosis came to our clinic for evaluation of lesions that had developed 2 years earlier&#46; The lesions had gradually appeared on the back of the neck and were asymptomatic&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed multiple whitish papules measuring 3&#160;mm in diameter and with a rubbery consistency&#59; the follicular openings were spared and showed no mucinous material when pressed&#46; The papules appeared in clusters on the back of the neck &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#44; with occasional papules in the right axilla&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">No other areas of the body were affected&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Dermoscopy revealed well-delimited&#44; homogenous&#44; whitish papules with some dotted vessels at the periphery&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">A 4-mm punch biopsy taken from a lesion on the neck showed slightly thickened collagen fibers in the mid dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; No calcium deposits were observed and staining of the elastic fibers revealed no abnormality&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis&#63;</p><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Diagnosis</span><p id="par0035" class="elsevierStylePara elsevierViewall">Fibroelastolytic papulosis of the neck&#46;</p></span></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Treatment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Given the benign nature of the lesions and the fact that they were asymptomatic&#44; it was decided to adopt a conservative approach and no treatment was given&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Comment</span><p id="par0045" class="elsevierStylePara elsevierViewall">In 1989 Shimizu et al&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> described white fibrous papulosis of the neck&#46; Clinically this entity was characterized by isolated&#44; asymptomatic whitish papules&#44; mainly located on the back and side of the neck&#44; although they were occasionally found on the shoulders and axillae&#46; Histologically&#44; white fibrous papulosis was classified under collagen disorders&#44; given that the lesions were composed of circumscribed collagen bundles in the papillary and upper reticular dermis&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Rongioletti and Rebora<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> later reported another very similar entity&#44; which they named pseudoxanthoma elasticum-like papillary dermal elastolysis&#44; in which they observed yellowish papules resembling those of white fibrous papulosis&#46; In this case&#44; however&#44; the papules showed a tendency to coalesce&#44; giving rise to cobblestone-like plaques suggestive of pseudoxanthoma elasticum &#40;PXE&#41;&#46; Histologically the lesions were mainly characterized by elastolysis&#44; with no calcium deposits or fibrosis&#44; and were categorized as elastic fiber abnormalities&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Balus et al&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> subsequently discovered common clinical and histologic findings when reviewing cases reported for both entities alongside 20 of their own&#46; They concluded that there were 2 variants of a single condition that they named fibroelastolytic papulosis of the neck&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The exact cause of fibroelastolytic papulosis of the neck is unknown&#44; although most authors have related it to the skin aging process&#46; Recently cases have also been reported in younger patients with a history of inflammatory diseases in the same area&#46; This suggests that factors other than aging&#44; such as the inflammation of skin adnexa&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> can exert an influence on the subsequent appearance of fibrosis and elastolysis&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">Diagnosis is based on clinical presentation&#44; histologic findings&#44; and&#44; more recently&#44; dermoscopy&#44; which shows well-delimited&#44; whitish papules with some dotted vessels at the periphery&#44; as well as pigment that fails to form a true reticulated area&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">The case reported here is more compatible with what was originally known as white fibrous papulosis of the neck&#46; This definitive diagnosis was established from the clinical presentation and dermoscopy&#44; but particularly after the exclusion of other entities&#44; such as PXE&#44; on pathology study of the biopsy&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">In conclusion&#44; knowledge of fibroelastolytic papulosis of the neck is important as it bears a strong clinical resemblance to PXE&#46; Unlike PXE&#44; however&#44; prognosis is good and not associated with heart or ophthalmological disease&#46;</p></span></span>"
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