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in which nonconfluent skin lesions called dermatofibrosis lenticularis disseminata appear&#46; The histology of Buschke-Ollendorff syndrome&#44; however&#44; is usually different&#44; as there is an increase in the number of elastic fibers rather than a decrease&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Even so&#44; some authors argue that papular elastorrhexis is one of these incomplete forms with no bone involvement &#40;osteopoikilosis&#41; or other findings&#46; For this reason&#44; dermatofibrosis lenticularis disseminata&#44; together with nevus anelasticus&#44; is the main differential diagnosis of papular elastorrhexis&#46; Nevus anelasticus is characterized by multiple reddish perifollicular papules that appear asymmetrically on the trunk and arms and may become confluent&#44; forming plaques&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> As occurs in papular elastorrhexis&#44; the main histologic finding is a reduction and degeneration of elastic fibers&#46; However&#44; some authors state that there is a greater loss of fibers than in papular elastorrhexis but with less fragmentation&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The differential diagnoses that must be borne in mind&#44; and their clinical and pathological signs&#44; include the following<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7&#8211;9</span></a>&#58;<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">1&#46;</span><p id="par0030" class="elsevierStylePara elsevierViewall">Eruptive collagenoma&#46; This presents as multiple whitish papules measuring 2 to 5<span class="elsevierStyleHsp" style=""></span>mm that appear on the trunk during adolescence&#46; There is a reduction of elastic fibers and homogenization and thickening of collagen&#46;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">2&#46;</span><p id="par0035" class="elsevierStylePara elsevierViewall">Perifollicular elastolysis&#46; This occurs in elderly women&#44; with the appearance of whitish-gray perifollicular papules measuring 1 to 4<span class="elsevierStyleHsp" style=""></span>mm in diameter on the neck&#44; ear lobes&#44; arms&#44; and trunk&#46; The etiology appears to be due to the epidermolytic toxin of <span class="elsevierStyleItalic">Staphylococcus epidermidis</span>&#46;</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">3&#46;</span><p id="par0040" class="elsevierStylePara elsevierViewall">Papular scarring of acne&#46; In this condition&#44; hypopigmented follicular papules develop on the upper trunk in patients with a history of acne&#46; There is a loss of elastic fibers around the follicles&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a></p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">4&#46;</span><p id="par0045" class="elsevierStylePara elsevierViewall">Secondary anetoderma&#46; This is formed of atrophic plaques or macules measuring 5 to 25<span class="elsevierStyleHsp" style=""></span>mm in diameter&#44; with fine wrinkles and baglike herniation of the underlying tissue&#46; There is a loss of elastic fibers&#44; and elastorrhexis may occasionally affect the entire dermis&#46;</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">5&#46;</span><p id="par0050" class="elsevierStylePara elsevierViewall">Mid dermal elastolysis&#46; This disorder&#44; most common in women aged 30 to 50 years&#44; presents as erythematous plaques&#44; telangiectasias&#44; and perifollicular papules on the neck and trunk&#46; The lesions resolve leaving an asymptomatic area with fine wrinkles&#46; There is a bandlike loss of elastic fibers in the mid dermis&#46;</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">6&#46;</span><p id="par0055" class="elsevierStylePara elsevierViewall">Pseudoxanthoma elasticum&#46; Yellow macules and papules coalesce to form plaques&#44; giving rise to a characteristic cobblestone pattern&#44; associated with flaccidity and laxity&#46; There is fragmentation of thickened elastic fibers and basophils with calcium deposits&#46;</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">7&#46;</span><p id="par0060" class="elsevierStylePara elsevierViewall">Fibroelastolytic papulosis of the neck&#46; A condition that is related to photoaging and that appears in the elderly&#46; Clinical features are similar to pseudoxanthoma elasticum with phenomena of elastolysis&#44; disappearance of elastic fibers&#44; and increased collagen fibers&#44; with reduced melanin content in the epidermis&#46;</p></li></ul></p><p id="par0065" class="elsevierStylePara elsevierViewall">Several of these disorders have been reported recently and are relatively unknown due to the limited number of cases published&#46; Moreover&#44; almost all share similar nonspecific clinical and histological features&#46; A series of data such as age&#44; sex&#44; clinical presentation &#40;nonfollicular papules&#44; no previous history of acne&#44; nonconfluent lesions&#41;&#44; and the histology are key to the diagnosis of papular elastorrhexis&#46; The case we report reflects the difficulty of diagnosing this group of diseases&#44; and our aim has been to provide a series of fundamental data to help differentiate each entity&#44; thereby aiding diagnosis&#46;</p></span>"
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Case and Research Letters
Papular Elastorrhexis: A Case Report and Principal Differential Diagnoses
Elastorrexis papulosa. Presentación de un caso y claves para el diagnóstico diferencial
F.M. Almazán-Fernándeza,
Corresponding author
almazanweb@hotmail.com

Corresponding author.
, A. Clemente-Ruiz de Almiróna, A. Sanz-Trellesb, J.C. Ruiz-Carrascosaa, R. Naranjo-Sintesa
a Servicio de Dermatología, Hospital Clínico San Cecilio, Granada, Spain
b Servicio de Anatomía Patológica, Hospital Carlos Haya, Málaga, Spain
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Physical examination revealed multiple&#44; slightly raised nonfollicular papules that were whitish in color and measured 1 to 4<span class="elsevierStyleHsp" style=""></span>mm in diameter &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figs&#46; 1 and 2</a>&#41;&#46; On histology&#44; orcein stain showed a marked reduction&#44; thinning&#44; and fragmentation of the elastic fibers in the dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; The remaining laboratory tests and imaging studies were normal&#46; No similar lesions were detected in other members of the patient&#39;s family&#46; A diagnosis of papular elastorrhexis was made on the basis of the clinical and histopathological findings&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">The first case of papular elastorrhexis was described by Bordas et al<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> in 1987 and was considered a variant of nevus anelasticus&#46; Although familial cases have been reported&#44;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> the condition is usually acquired after adolescence and the second decade of life&#44; and is more common in women&#46; It presents clinically as multiple nonfollicular&#44; nonconfluent whitish papules&#44; measuring 5<span class="elsevierStyleHsp" style=""></span>mm or less&#44; distributed mainly over the upper part of the trunk &#40;shoulders and proximal upper arms&#41;&#46; However&#44; recent reports have described more atypical sites on the head and neck&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Most cases are isolated&#44; with no concomitant disease&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Histologically&#44; there is a reduction and fragmentation of elastic fibers&#44; mainly in the upper dermis&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The clinical characteristics of papular elastorrhexis resemble incomplete forms of the Buschke-Ollendorff syndrome&#44; in which nonconfluent skin lesions called dermatofibrosis lenticularis disseminata appear&#46; The histology of Buschke-Ollendorff syndrome&#44; however&#44; is usually different&#44; as there is an increase in the number of elastic fibers rather than a decrease&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Even so&#44; some authors argue that papular elastorrhexis is one of these incomplete forms with no bone involvement &#40;osteopoikilosis&#41; or other findings&#46; For this reason&#44; dermatofibrosis lenticularis disseminata&#44; together with nevus anelasticus&#44; is the main differential diagnosis of papular elastorrhexis&#46; Nevus anelasticus is characterized by multiple reddish perifollicular papules that appear asymmetrically on the trunk and arms and may become confluent&#44; forming plaques&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> As occurs in papular elastorrhexis&#44; the main histologic finding is a reduction and degeneration of elastic fibers&#46; However&#44; some authors state that there is a greater loss of fibers than in papular elastorrhexis but with less fragmentation&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The differential diagnoses that must be borne in mind&#44; and their clinical and pathological signs&#44; include the following<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7&#8211;9</span></a>&#58;<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">1&#46;</span><p id="par0030" class="elsevierStylePara elsevierViewall">Eruptive collagenoma&#46; This presents as multiple whitish papules measuring 2 to 5<span class="elsevierStyleHsp" style=""></span>mm that appear on the trunk during adolescence&#46; There is a reduction of elastic fibers and homogenization and thickening of collagen&#46;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">2&#46;</span><p id="par0035" class="elsevierStylePara elsevierViewall">Perifollicular elastolysis&#46; This occurs in elderly women&#44; with the appearance of whitish-gray perifollicular papules measuring 1 to 4<span class="elsevierStyleHsp" style=""></span>mm in diameter on the neck&#44; ear lobes&#44; arms&#44; and trunk&#46; The etiology appears to be due to the epidermolytic toxin of <span class="elsevierStyleItalic">Staphylococcus epidermidis</span>&#46;</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">3&#46;</span><p id="par0040" class="elsevierStylePara elsevierViewall">Papular scarring of acne&#46; In this condition&#44; hypopigmented follicular papules develop on the upper trunk in patients with a history of acne&#46; There is a loss of elastic fibers around the follicles&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a></p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">4&#46;</span><p id="par0045" class="elsevierStylePara elsevierViewall">Secondary anetoderma&#46; This is formed of atrophic plaques or macules measuring 5 to 25<span class="elsevierStyleHsp" style=""></span>mm in diameter&#44; with fine wrinkles and baglike herniation of the underlying tissue&#46; There is a loss of elastic fibers&#44; and elastorrhexis may occasionally affect the entire dermis&#46;</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">5&#46;</span><p id="par0050" class="elsevierStylePara elsevierViewall">Mid dermal elastolysis&#46; This disorder&#44; most common in women aged 30 to 50 years&#44; presents as erythematous plaques&#44; telangiectasias&#44; and perifollicular papules on the neck and trunk&#46; The lesions resolve leaving an asymptomatic area with fine wrinkles&#46; There is a bandlike loss of elastic fibers in the mid dermis&#46;</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">6&#46;</span><p id="par0055" class="elsevierStylePara elsevierViewall">Pseudoxanthoma elasticum&#46; Yellow macules and papules coalesce to form plaques&#44; giving rise to a characteristic cobblestone pattern&#44; associated with flaccidity and laxity&#46; There is fragmentation of thickened elastic fibers and basophils with calcium deposits&#46;</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">7&#46;</span><p id="par0060" class="elsevierStylePara elsevierViewall">Fibroelastolytic papulosis of the neck&#46; A condition that is related to photoaging and that appears in the elderly&#46; Clinical features are similar to pseudoxanthoma elasticum with phenomena of elastolysis&#44; disappearance of elastic fibers&#44; and increased collagen fibers&#44; with reduced melanin content in the epidermis&#46;</p></li></ul></p><p id="par0065" class="elsevierStylePara elsevierViewall">Several of these disorders have been reported recently and are relatively unknown due to the limited number of cases published&#46; Moreover&#44; almost all share similar nonspecific clinical and histological features&#46; A series of data such as age&#44; sex&#44; clinical presentation &#40;nonfollicular papules&#44; no previous history of acne&#44; nonconfluent lesions&#41;&#44; and the histology are key to the diagnosis of papular elastorrhexis&#46; The case we report reflects the difficulty of diagnosing this group of diseases&#44; and our aim has been to provide a series of fundamental data to help differentiate each entity&#44; thereby aiding diagnosis&#46;</p></span>"
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ISSN: 15782190
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