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deposits around blood vessels and sweat glands and in the papillary dermis&#46; The second group of authors also found IgA deposits in elastic fibers around hair follicles&#46; Histopathology shows disperse fragmented&#44; granular elastic fibres in the reticular dermis&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">The differential diagnosis in blepharochalasis should include inflammatory diseases &#40;contact dermatitis&#44; angioedema&#44; blepharitis&#44; and hereditary angioedema&#41;&#44; tumors &#40;retrobulbar tumor or lacrimal gland tumor&#47;cyst&#41;&#44; systemic diseases &#40;thyroid and kidney disease&#41;&#44; other extracellular matrix diseases &#40;Ehlers-Danlos syndrome&#44; elastic pseudoxanthoma&#44; mid-dermal elastolysis&#44; anetodermia&#44; and postinflammatory elastolysis&#41;&#44; and even physiologic aging&#46; The condition can also form part of syndromes&#44; such as Ascher syndrome&#44; which is characterized by blepharochalasis&#44; double lip&#44; and nontoxic goiter&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">The treatment for blepharochalasis is reconstructive surgery with cosmetic and therapeutic intent&#44; but recurrence is common&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">In this article&#44; we describe the case of a young man with blepharochalasis as a clinical presentation of acquired cutis laxa&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">A 24-year-old man presented with looseness of the upper eyelids that had become progressively worse over 8 years&#46; The sagging was such that it had caused considerable cosmetic damage&#46; The patient reported that he had experienced several episodes of eyelid swelling over a period of more than 2 years before his eyelids began to sag&#46; He had not received any treatment and reported no other problems&#46; Skin examination showed loose skin on both upper eyelids &#40;more pronounced on the right&#41; and partial occlusion of the eyelashes&#46; The skin in the upper eyelid region was atrophic and had a violaceous color &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Vision was not affected by the blepharoptis&#46; The patient confirmed that the condition had not been treated medically&#46; Biopsy of the eyelids &#40;with hematoxylin-eosin staining&#41; showed just slight swelling of the upper dermis&#46; Orcein staining showed absence of elastic fibers &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; IgA was positive in the dermal vessel walls&#46; Computed tomography of the orbits&#44; a chest X-ray&#44; and laboratory tests were all normal&#46; The patient underwent upper blepharoplasty &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41; and the cosmetic and functional results during follow-up were excellent&#46; He reported that his field of vision had improved&#44; particularly the upper field as his eyes were no longer covered by the sagging eyelids&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Blepharochalasis is an uncommon clinical presentation of cutis laxa characterized by painless episodes of bilateral edema&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> The condition is generally self-limiting and does not respond to antihistamines or corticosteroids&#46; During the quiescent stage of disease and following several acute attacks&#44; it progresses to cause bilateral loss of periorbital skin&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> There may be violaceous coloring on the upper eyelids&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> In our patient&#44; this coloring led to consideration of dermatomyositis in the differential diagnosis&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">The pathophysiology of blepharochalasis remains elusive&#46; Inflammatory episodes are believed to give rise to elastic fiber degradation due to elastolysis triggered by increased elastase activity or impaired elastase inhibitor function&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> Systemic manifestations have not been observed in the vast majority of cases&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> In line with previously described findings&#44; IgA deposits&#44; which probably have a pathogenic role&#44; were observed in the blood vessel walls&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Proposed triggers include stress&#44; fever&#44; and upper respiratory tract infections&#44; among others&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> A more specific immune mechanism associated with elastin and collagen degradation by metalloproteases may also be involved &#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The recommended treatment is blepharoplasty with cosmetic or therapeutic intent&#46; Ideally&#44; surgery should be performed during the quiescent disease stage&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> Unlike most connective tissue diseases&#44; cutis laxa does not usually interfere with postoperative recovery&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> Patient follow-up is necessary&#44; however&#44; given the risk of recurrence&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Blepharochalasis must be contemplated in the differential diagnosis of skin disorders that affect the periorbital region&#46; Correct diagnosis is important for ensuring adequate treatment and follow-up&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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Case and Research Letter
Blepharochalasis: A Rare Presentation of Cutis Laxa
Blefarocalasia: una presentación rara de cutis laxo
S.G. Dantas, B.M. Trope, T.C. de Magalhães, D.R. Azulay, D.C. Quintella, M. Ramos-e-Silva
Autor para correspondencia
ramos.e.silva@dermato.med.br

Corresponding author.
Servicio de Dermatología, Hospital Universitario, Escuela de Medicina, Universidad Federal de Río de Janeiro, Río de Janeiro, Brasil
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Cutis laxa is a congenital or acquired connective tissue disorder characterized by extracellular matrix and elastic fiber defects&#46; It manifests clinically as sagging skin&#44; giving an appearance of premature aging&#46; Blepharochalasis is an acquired form of cutis laxa that affects the periorbital region&#46; It normally starts in childhood or adolescence and is characterized by recurrent episodes of painless eyelid swelling&#46; Repeated acute attacks lead to elastin fiber degradation and sagging skin in the periorbital region that can interfere with vision&#46; The pathogenesis of blepharochalasis or blepharoptosis is still unclear&#44; although a role has been proposed for immunopathogenic mechanisms that result in elastin fiber degradation&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> Grassegger et al<span class="elsevierStyleItalic">&#46;</span><a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> and Schaeppi et al<span class="elsevierStyleItalic">&#46;</span><a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> reported immunoglobulin A &#40;IgA&#41; deposits around blood vessels and sweat glands and in the papillary dermis&#46; The second group of authors also found IgA deposits in elastic fibers around hair follicles&#46; Histopathology shows disperse fragmented&#44; granular elastic fibres in the reticular dermis&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">The differential diagnosis in blepharochalasis should include inflammatory diseases &#40;contact dermatitis&#44; angioedema&#44; blepharitis&#44; and hereditary angioedema&#41;&#44; tumors &#40;retrobulbar tumor or lacrimal gland tumor&#47;cyst&#41;&#44; systemic diseases &#40;thyroid and kidney disease&#41;&#44; other extracellular matrix diseases &#40;Ehlers-Danlos syndrome&#44; elastic pseudoxanthoma&#44; mid-dermal elastolysis&#44; anetodermia&#44; and postinflammatory elastolysis&#41;&#44; and even physiologic aging&#46; The condition can also form part of syndromes&#44; such as Ascher syndrome&#44; which is characterized by blepharochalasis&#44; double lip&#44; and nontoxic goiter&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">The treatment for blepharochalasis is reconstructive surgery with cosmetic and therapeutic intent&#44; but recurrence is common&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">In this article&#44; we describe the case of a young man with blepharochalasis as a clinical presentation of acquired cutis laxa&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">A 24-year-old man presented with looseness of the upper eyelids that had become progressively worse over 8 years&#46; The sagging was such that it had caused considerable cosmetic damage&#46; The patient reported that he had experienced several episodes of eyelid swelling over a period of more than 2 years before his eyelids began to sag&#46; He had not received any treatment and reported no other problems&#46; Skin examination showed loose skin on both upper eyelids &#40;more pronounced on the right&#41; and partial occlusion of the eyelashes&#46; The skin in the upper eyelid region was atrophic and had a violaceous color &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Vision was not affected by the blepharoptis&#46; The patient confirmed that the condition had not been treated medically&#46; Biopsy of the eyelids &#40;with hematoxylin-eosin staining&#41; showed just slight swelling of the upper dermis&#46; Orcein staining showed absence of elastic fibers &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; IgA was positive in the dermal vessel walls&#46; Computed tomography of the orbits&#44; a chest X-ray&#44; and laboratory tests were all normal&#46; The patient underwent upper blepharoplasty &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41; and the cosmetic and functional results during follow-up were excellent&#46; He reported that his field of vision had improved&#44; particularly the upper field as his eyes were no longer covered by the sagging eyelids&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Blepharochalasis is an uncommon clinical presentation of cutis laxa characterized by painless episodes of bilateral edema&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> The condition is generally self-limiting and does not respond to antihistamines or corticosteroids&#46; During the quiescent stage of disease and following several acute attacks&#44; it progresses to cause bilateral loss of periorbital skin&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> There may be violaceous coloring on the upper eyelids&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> In our patient&#44; this coloring led to consideration of dermatomyositis in the differential diagnosis&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">The pathophysiology of blepharochalasis remains elusive&#46; Inflammatory episodes are believed to give rise to elastic fiber degradation due to elastolysis triggered by increased elastase activity or impaired elastase inhibitor function&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> Systemic manifestations have not been observed in the vast majority of cases&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> In line with previously described findings&#44; IgA deposits&#44; which probably have a pathogenic role&#44; were observed in the blood vessel walls&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Proposed triggers include stress&#44; fever&#44; and upper respiratory tract infections&#44; among others&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> A more specific immune mechanism associated with elastin and collagen degradation by metalloproteases may also be involved &#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The recommended treatment is blepharoplasty with cosmetic or therapeutic intent&#46; Ideally&#44; surgery should be performed during the quiescent disease stage&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> Unlike most connective tissue diseases&#44; cutis laxa does not usually interfere with postoperative recovery&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> Patient follow-up is necessary&#44; however&#44; given the risk of recurrence&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Blepharochalasis must be contemplated in the differential diagnosis of skin disorders that affect the periorbital region&#46; Correct diagnosis is important for ensuring adequate treatment and follow-up&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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