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1</a>&#41;&#46; These round&#44; skin-colored&#44; and in some cases umbilicated papules measured from 2 to 5 <span class="elsevierStyleHsp" style=""></span>mm in diameter and contained a small central keratotic plug &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The older lesions were macular&#44; light brown&#44; and infiltrated&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Thickened collagen bundles were observed in the superficial dermis&#46; These bundles were surrounded by areas of epidermal proliferation where transepidermal elimination of collagen took place through a small central crater containing keratotic material&#44; mixed inflammatory infiltrate&#44; and cellular detritus &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">The results of a complete blood biochemistry ruled out abnormalities in glucose metabolism and renal insufficiency&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis&#63;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Familial reactive perforating collagenosis&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Topical treatment was started with tretinoin 0&#46;1&#37;&#44; in combination with emollients&#44; on the more recent lesions&#46; The patient was strongly advised to avoid injury and scratching&#46; Improvement was seen in 4 weeks&#44; with a reduction in the number of lesions and symptom intensity&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Familial reactive perforating collagenosis is one of the primary perforating diseases&#44; a group of disorders characterized by keratotic plugs through which dermal connective tissue is eliminated through the epidermis&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">These diseases fall into 3 main categories&#58; elastosis perforans serpiginosa involving the elimination of elastic fibers&#44; acquired perforating dermatosis with adult onset often related to diabetes&#44; renal insufficiency&#44; or toxicoderma&#44;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and&#44; least frequently&#44; familial reactive perforating collagenosis&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Mehregan&#44; Schwartz&#44; and Livingood first described this hereditary disorder in 1967&#59; the etiology is still poorly understood and the pattern of transmission varies&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> The condition is rare and only 50 cases have been described&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">From infancy&#44; these patients develop skin-colored papules measuring less than 10<span class="elsevierStyleHsp" style=""></span>mm in diameter in response to superficial trauma&#46; These lesions become umbilicated and eventually form a crater with a small central keratotic plug&#46; They are typically found on the extensor aspects of the arms&#44; the dorsum of the hands&#44; and&#44; less frequently&#44; the elbows&#44; knees&#44; and feet&#46; The palms of the hands and the soles of the feet are not affected&#46; The lesions disappear within 2 months&#44; leaving atrophy and hypopigmentation&#46; The isomorphic response is more typical in this disease than in other perforating disorders&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The basic defect is thought to be due to a genetic abnormality that affects the collagen&#44; leading to greater lability after injury&#46; The same abnormality is also associated with an increase in serum and tissue concentrations of fibronectin and favors epithelial migration and proliferation&#44; resulting in the transepidermal elimination of damaged collagen&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Histologic examination reveals deposits of basophilic dermal collagen surrounded by inflammatory cells and areas of epidermal proliferation that eventually give rise to transepidermal elimination through a small central crater containing keratotic material and inflammatory cells&#46; 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Case for Diagnosis
Umbilicated Papules on the Dorsum of Hands
Pápulas umbilicadas en dorso de manos
A.I. Sánchez-Moyaa,
Autor para correspondencia
anai.sanchezmoya@yahoo.es

Corresponding author.
, R. Juarez-Tosinab, D. García Almagroa
a Servicio de Dermatología, Complejo Hospitalario, Toledo, Spain
b Servicio de Anatomía Patológica, Complejo Hospitalario, Toledo, 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">We report the case of a 35-year-old woman who presented with pruritic lesions on the dorsum of the hands that had appeared 6 months earlier&#46; After she started working as a baker and received small burns&#44; the patient experienced more intense symptoms and the lesions worsened&#46; She reported a lifelong history of anomalous scarring from minor injuries&#44; taking the form of papular and erosive lesions that regressed after 6 weeks&#46; The patient has 2 brothers who have similar symptoms&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Lesions at various stages of development were observed on the dorsum of the hands and the arms &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; These round&#44; skin-colored&#44; and in some cases umbilicated papules measured from 2 to 5 <span class="elsevierStyleHsp" style=""></span>mm in diameter and contained a small central keratotic plug &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The older lesions were macular&#44; light brown&#44; and infiltrated&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Thickened collagen bundles were observed in the superficial dermis&#46; These bundles were surrounded by areas of epidermal proliferation where transepidermal elimination of collagen took place through a small central crater containing keratotic material&#44; mixed inflammatory infiltrate&#44; and cellular detritus &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">The results of a complete blood biochemistry ruled out abnormalities in glucose metabolism and renal insufficiency&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis&#63;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Familial reactive perforating collagenosis&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Topical treatment was started with tretinoin 0&#46;1&#37;&#44; in combination with emollients&#44; on the more recent lesions&#46; The patient was strongly advised to avoid injury and scratching&#46; Improvement was seen in 4 weeks&#44; with a reduction in the number of lesions and symptom intensity&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Familial reactive perforating collagenosis is one of the primary perforating diseases&#44; a group of disorders characterized by keratotic plugs through which dermal connective tissue is eliminated through the epidermis&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">These diseases fall into 3 main categories&#58; elastosis perforans serpiginosa involving the elimination of elastic fibers&#44; acquired perforating dermatosis with adult onset often related to diabetes&#44; renal insufficiency&#44; or toxicoderma&#44;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and&#44; least frequently&#44; familial reactive perforating collagenosis&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Mehregan&#44; Schwartz&#44; and Livingood first described this hereditary disorder in 1967&#59; the etiology is still poorly understood and the pattern of transmission varies&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> The condition is rare and only 50 cases have been described&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">From infancy&#44; these patients develop skin-colored papules measuring less than 10<span class="elsevierStyleHsp" style=""></span>mm in diameter in response to superficial trauma&#46; These lesions become umbilicated and eventually form a crater with a small central keratotic plug&#46; They are typically found on the extensor aspects of the arms&#44; the dorsum of the hands&#44; and&#44; less frequently&#44; the elbows&#44; knees&#44; and feet&#46; The palms of the hands and the soles of the feet are not affected&#46; The lesions disappear within 2 months&#44; leaving atrophy and hypopigmentation&#46; The isomorphic response is more typical in this disease than in other perforating disorders&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The basic defect is thought to be due to a genetic abnormality that affects the collagen&#44; leading to greater lability after injury&#46; The same abnormality is also associated with an increase in serum and tissue concentrations of fibronectin and favors epithelial migration and proliferation&#44; resulting in the transepidermal elimination of damaged collagen&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Histologic examination reveals deposits of basophilic dermal collagen surrounded by inflammatory cells and areas of epidermal proliferation that eventually give rise to transepidermal elimination through a small central crater containing keratotic material and inflammatory cells&#46; Elastic fibres are not affected&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">The differential diagnosis must include inflammatory pathologies &#40;folliculitis&#44; prurigo nodularis&#44; arthropod bites&#41;&#44; mucoid material deposits &#40;acral persistent papular mucinosis&#41;&#44; and tumors &#40;multiple keratoacanthomas&#44; syringomas&#44; and benign fibrous histiocytomas&#41;&#44; in addition to all other perforating diseases&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">Several treatments have been described&#44; including emollients&#44; topical corticosteroids&#44; topical retinoic acid&#44; oral isotretinoin&#44; and vitamin A supplements&#44; but the results are varied&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> The hereditary forms of reactive perforating collagenosis are usually mild and localized&#44; with no alarming symptoms except in rare circumstances&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflict of Interest</span><p id="par0080" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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2019 Febrero 2 17 19
2019 Enero 2 9 11
2018 Diciembre 2 0 2
2018 Noviembre 3 0 3
2018 Octubre 6 0 6
2018 Septiembre 4 12 16
2018 Agosto 0 41 41
2018 Julio 0 22 22
2018 Junio 0 18 18
2018 Mayo 0 29 29
2018 Abril 0 15 15
2018 Marzo 14 30 44
2018 Febrero 100 29 129
2018 Enero 194 29 223
2017 Diciembre 179 31 210
2017 Noviembre 108 28 136
2017 Octubre 111 25 136
2017 Septiembre 90 47 137
2017 Agosto 126 57 183
2017 Julio 96 46 142
2017 Junio 115 51 166
2017 Mayo 107 26 133
2017 Abril 94 51 145
2017 Marzo 104 52 156
2017 Febrero 79 43 122
2017 Enero 64 21 85
2016 Diciembre 104 31 135
2016 Noviembre 144 57 201
2016 Octubre 150 40 190
2016 Septiembre 216 48 264
2016 Agosto 135 30 165
2016 Julio 101 26 127
2016 Junio 17 15 32
2016 Mayo 9 12 21
2016 Abril 4 1 5
2016 Marzo 7 28 35
2016 Febrero 10 31 41
2016 Enero 6 42 48
2015 Diciembre 10 24 34
2015 Noviembre 35 25 60
2015 Octubre 26 28 54
2015 Septiembre 18 10 28
2015 Agosto 11 10 21
2015 Julio 142 16 158
2015 Junio 102 9 111
2015 Mayo 125 17 142
2015 Abril 117 21 138
2015 Marzo 89 15 104
2015 Febrero 88 17 105
2015 Enero 104 18 122
2014 Diciembre 85 10 95
2014 Noviembre 94 11 105
2014 Octubre 145 24 169
2014 Septiembre 103 15 118
2014 Agosto 133 18 151
2014 Julio 130 19 149
2014 Junio 138 20 158
2014 Mayo 81 9 90
2014 Abril 48 4 52
2014 Marzo 47 9 56
2014 Febrero 60 3 63
2014 Enero 47 7 54
2013 Diciembre 46 8 54
2013 Noviembre 39 7 46
2013 Octubre 39 8 47
2013 Septiembre 33 7 40
2013 Agosto 31 14 45
2013 Julio 27 15 42
2013 Junio 10 8 18
2013 Mayo 14 13 27
2013 Abril 17 10 27
2013 Marzo 17 5 22
2013 Febrero 36 9 45
2013 Enero 61 4 65
2012 Diciembre 16 4 20
2012 Noviembre 1 1 2
2012 Octubre 0 1 1
2012 Agosto 3 2 5
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