Case and Research Letter
Congenital Fascial Dystrophy or Stiff Skin Syndrome: A Case Report
Distrofia fascial congénita o síndrome de la piel rígida: presentación de un caso
A. Plana Pla
, I. Bielsa Marsol, M. Fernández-Figueras, C. Ferrándiz Foraster
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Departamento de Dermatología, Hospital Universitari Germans Trias i Pujol, Badalona, Barcelona, Spain
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Plana Pla, I. Bielsa Marsol, M. Fernández-Figueras, C. Ferrándiz Foraster" "autores" => array:4 [ 0 => array:4 [ "nombre" => "A." "apellidos" => "Plana Pla" "email" => array:1 [ 0 => "adriplanapla@yahoo.es" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "I." "apellidos" => "Bielsa Marsol" ] 2 => array:2 [ "nombre" => "M." "apellidos" => "Fernández-Figueras" ] 3 => array:2 [ "nombre" => "C." "apellidos" => "Ferrándiz Foraster" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Departamento de Dermatología, Hospital Universitari Germans Trias i Pujol, Badalona, Barcelona, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Distrofia fascial congénita o síndrome de la piel rígida: presentación de un caso" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 716 "Ancho" => 951 "Tamanyo" => 364847 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Bundles of thickened collagen in the subcutaneous cellular tissue, forming a latticework. There is a total absence of inflammatory infiltrate. Hematoxylin-eosin, original magnification ×<span class="elsevierStyleHsp" style=""></span>20.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Congenital fascial dystrophy (CFD) or stiff skin syndrome is a rare skin disease that was described in 1971 by Esterly and McKusick.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The condition is characterized by noninflammatory fibrosis of the subcutaneous cellular tissue and of the muscle fascia, leading to hardening of the skin and interference with movement of the underlying joints. It can be hereditary and show a very variable degree of severity, sometimes causing minimal symptoms, as in the case we describe below, in which the diagnosis was made in adulthood.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The patient was a 46-year-old man with no past medical history of interest. He had been referred from another hospital with a clinical suspicion of morphea profunda that had not responded to treatment with oral corticosteroids. The patient stated that since childhood he had had difficulty performing certain movements, such as flexing the trunk, and that it had been impossible to administer intramuscular injections into the right buttock. He also said that his daughter had similar symptoms. Examination revealed difficulty raising a skin fold in the lumbar and right gluteal regions (<a class="elsevierStyleCrossRef" href="#fig0005">Figs. 1 and 2</a>), where the skin was hard to the touch, and a limitation of movement of the right hip, particularly flexion. Investigations performed at the previous hospital included an autoimmunity study that was normal, magnetic resonance imaging, which excluded bone or muscle involvement, and skin biopsy, which was reported to be consistent with morphea profunda. The biopsy was reviewed: thickened collagen was observed in a horizontally oriented lattice pattern in the deepest layers of the dermis, and there was no inflammatory infiltrate (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 3</a>). We made a diagnosis of CFD based on the clinical and pathological findings, and physiotherapy was recommended.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">CFD is a very rare disorder and descriptions are scarce in the literature.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> It affects men and women equally, and a family history is found in 30% of patients. In the case described, the targeted history enabled us to identify a daughter with similar symptoms, though, at the time of writing, no additional tests had been performed to confirm the diagnosis.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The pathogenesis of this disease was unknown until recently.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> However, Loeys et al.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> discovered that all affected individuals had a mutation in the <span class="elsevierStyleItalic">fibrillin-1</span> gene. This protein, which is also mutated in patients with Marfan syndrome, is mainly involved in the formation of microfibrils (formed of fibrillin polymers), which, together with elastin, form the elastic fibers. This mutation leads to a disorganized accumulation of microfibrils in the dermis; this can be observed on confocal or electron microscopy. These accumulations produce abnormal activation of another molecule, transforming growth factor (TGF-β), which has the ability to promote collagen deposition in the dermis. The increased TGF-β levels in patients with CFD leads to greater collagen deposition in the deeper regions of the dermis, the subcutaneous cellular tissue, and the muscle fascia.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Clinical manifestations are usually present at birth, but they can also appear during the first 6 years of life.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Patients with CFD have clearly circumscribed areas of hardened skin with no visible changes to the skin surface; these areas arise particularly around the pelvic or shoulder girdles and on the proximal areas of the thighs.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Less common findings include hypertrichosis, hyperpigmentation in the affected areas,<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> and the presence of subcutaneous nodules on the distal phalanges of the fingers.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The main problem caused by this condition is limitation of joint movement. In the majority of patients this limitation is mild and does not interfere excessively with daily life; however, changes can sometimes be very widespread and can even limit lung capacity.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> If the symptoms are mild, the diagnosis may not be made until adult life.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Not only the clinical manifestations but also the microscopic findings are very important. Microscopy reveals a proliferation of collagen tissue, particularly in the muscle fascia and in the subcutaneous cellular tissue, although there are reports of cases in which only the reticular dermis is affected.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> It is important to note that involvement of the muscle fascia is not a prerequisite for making the diagnosis. The term <span class="elsevierStyleItalic">congenital fascial dystrophy</span> was proposed in the original description of the disorder because the fascia was found to be affected in all the patients with this diagnosis. However, not all cases diagnosed since that time have had fascial involvement. The most characteristic microscopy finding suggestive of CFD is not so much the site of the excess collagen, but its arrangement in a lattice-like array.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> Other typical features are the absence of an inflammatory infiltrate and the presence of mucin.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Finally, to confirm the diagnosis it is important to exclude the presence of specific autoantibodies and of structural bone or muscle lesions.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The main condition to be taken into account in the differential diagnosis is morphea profunda.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> In contrast to CFD, morphea profunda usually has an asymmetric distribution, it does not present at an early age, visible skin changes are usually present, and, microscopically, the collagen is compact. In addition, in the initial phases there is usually a lymphoplasmacytic inflammatory infiltrate. In pansclerotic morphea of childhood, although the sclerotic process is also deep, as in CFD, the presence of very evident skin changes, including ulcers and pigment disorders, makes differentiation easy.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">No effective treatment for CFD exists. Numerous drugs, including topical and oral corticosteroids, methotrexate, and psoralen–UV-A, have been tried and the results have been very poor with all of them.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Only physiotherapy appears to be able to improve joint comfort and movement.</p><p id="par0055" class="elsevierStylePara elsevierViewall">The interest in knowing CFD and being able to make a correct diagnosis derives mainly from the need to differentiate it from morphea profunda, a condition with similar clinical and microscopic characteristics. Only in this way is it possible to avoid the administration of potentially harmful drugs that, in the case of CFD, would be totally ineffective.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Plana Pla A, Bielsa Marsol I, Fernández-Figueras M, Ferrándiz Foraster C. Distrofia fascial congénita o síndrome de la piel rígida: presentación de un caso. Actas Dermosifiliogr. 2014;105:805–807.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figures 1 and 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1v2.jpeg" "Alto" => 952 "Ancho" => 1301 "Tamanyo" => 178716 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">1, The skin of the lumbar region showed no visible changes, but it was difficult to pinch it into a fold. 2, The skin of the buttock was similar to that of the lumbar region; it felt hard to the touch but there were no changes visible on the surface.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 716 "Ancho" => 951 "Tamanyo" => 364847 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Bundles of thickened collagen in the subcutaneous cellular tissue, forming a latticework. 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Tuzün" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Pediatr Dermatol" "fecha" => "2003" "volumen" => "20" "paginaInicial" => "339" "paginaFinal" => "341" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12869158" "web" => "Medline" ] ] ] ] ] ] ] ] 6 => array:3 [ "identificador" => "bib0035" "etiqueta" => "7" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A subcutaneous lattice-like array of thick collagen is a clue to the diagnosis of stiff skin syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "T.H. McCallmont" 1 => "A.E. Gilliam" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1600-0560.2011.01845_2.x" "Revista" => array:6 [ "tituloSerie" => "J Cutan Pathol" "fecha" => "2012" "volumen" => "39" "paginaInicial" => "2" "paginaFinal" => "4" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22211327" "web" => "Medline" ] ] ] ] ] ] ] ] 7 => array:3 [ "identificador" => "bib0040" "etiqueta" => "8" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Disabling pansclerotic morphea of children" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "J.M. Diaz-Perez" 1 => "S.M. Connoly" 2 => "S.K. Winkelmann" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Arch Dermatol" "fecha" => "1980" "volumen" => "116" "paginaInicial" => "169" "paginaFinal" => "173" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/7356347" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000010500000008/v1_201409260034/S1578219014002108/v1_201409260034/en/main.assets" "Apartado" => array:4 [ "identificador" => "6157" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Case and Research Letters" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15782190/0000010500000008/v1_201409260034/S1578219014002108/v1_201409260034/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219014002108?idApp=UINPBA000044" ]
| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 5 | 6 | 11 |
| 2024 Octubre | 128 | 48 | 176 |
| 2024 Septiembre | 130 | 46 | 176 |
| 2024 Agosto | 160 | 93 | 253 |
| 2024 Julio | 148 | 42 | 190 |
| 2024 Junio | 126 | 44 | 170 |
| 2024 Mayo | 129 | 49 | 178 |
| 2024 Abril | 123 | 38 | 161 |
| 2024 Marzo | 115 | 48 | 163 |
| 2024 Febrero | 122 | 50 | 172 |
| 2024 Enero | 129 | 41 | 170 |
| 2023 Diciembre | 110 | 31 | 141 |
| 2023 Noviembre | 96 | 28 | 124 |
| 2023 Octubre | 93 | 35 | 128 |
| 2023 Septiembre | 101 | 28 | 129 |
| 2023 Agosto | 95 | 25 | 120 |
| 2023 Julio | 82 | 33 | 115 |
| 2023 Junio | 132 | 35 | 167 |
| 2023 Mayo | 105 | 30 | 135 |
| 2023 Abril | 74 | 25 | 99 |
| 2023 Marzo | 80 | 29 | 109 |
| 2023 Febrero | 66 | 26 | 92 |
| 2023 Enero | 64 | 45 | 109 |
| 2022 Diciembre | 86 | 54 | 140 |
| 2022 Noviembre | 42 | 23 | 65 |
| 2022 Octubre | 38 | 27 | 65 |
| 2022 Septiembre | 35 | 51 | 86 |
| 2022 Agosto | 51 | 38 | 89 |
| 2022 Julio | 32 | 41 | 73 |
| 2022 Junio | 25 | 20 | 45 |
| 2022 Mayo | 86 | 44 | 130 |
| 2022 Abril | 90 | 48 | 138 |
| 2022 Marzo | 84 | 45 | 129 |
| 2022 Febrero | 96 | 47 | 143 |
| 2022 Enero | 119 | 38 | 157 |
| 2021 Diciembre | 65 | 34 | 99 |
| 2021 Noviembre | 74 | 49 | 123 |
| 2021 Octubre | 72 | 45 | 117 |
| 2021 Septiembre | 65 | 35 | 100 |
| 2021 Agosto | 73 | 33 | 106 |
| 2021 Julio | 57 | 27 | 84 |
| 2021 Junio | 49 | 20 | 69 |
| 2021 Mayo | 53 | 29 | 82 |
| 2021 Abril | 150 | 47 | 197 |
| 2021 Marzo | 93 | 20 | 113 |
| 2021 Febrero | 97 | 29 | 126 |
| 2021 Enero | 52 | 13 | 65 |
| 2020 Diciembre | 52 | 14 | 66 |
| 2020 Noviembre | 18 | 7 | 25 |
| 2020 Octubre | 31 | 14 | 45 |
| 2020 Septiembre | 37 | 6 | 43 |
| 2020 Agosto | 23 | 15 | 38 |
| 2020 Julio | 25 | 16 | 41 |
| 2020 Junio | 41 | 23 | 64 |
| 2020 Mayo | 34 | 15 | 49 |
| 2020 Abril | 29 | 18 | 47 |
| 2020 Marzo | 40 | 17 | 57 |
| 2020 Febrero | 3 | 6 | 9 |
| 2020 Enero | 4 | 14 | 18 |
| 2019 Diciembre | 4 | 13 | 17 |
| 2019 Noviembre | 4 | 12 | 16 |
| 2019 Octubre | 0 | 2 | 2 |
| 2019 Septiembre | 1 | 3 | 4 |
| 2019 Agosto | 4 | 2 | 6 |
| 2019 Julio | 6 | 10 | 16 |
| 2019 Junio | 4 | 7 | 11 |
| 2019 Mayo | 5 | 20 | 25 |
| 2019 Abril | 8 | 8 | 16 |
| 2019 Marzo | 4 | 4 | 8 |
| 2019 Febrero | 4 | 2 | 6 |
| 2019 Enero | 5 | 0 | 5 |
| 2018 Diciembre | 2 | 1 | 3 |
| 2018 Noviembre | 3 | 0 | 3 |
| 2018 Octubre | 3 | 0 | 3 |
| 2018 Septiembre | 4 | 2 | 6 |
| 2018 Agosto | 0 | 4 | 4 |
| 2018 Julio | 0 | 7 | 7 |
| 2018 Junio | 0 | 5 | 5 |
| 2018 Mayo | 0 | 16 | 16 |
| 2018 Abril | 0 | 1 | 1 |
| 2018 Marzo | 2 | 10 | 12 |
| 2018 Febrero | 35 | 6 | 41 |
| 2018 Enero | 48 | 6 | 54 |
| 2017 Diciembre | 43 | 11 | 54 |
| 2017 Noviembre | 31 | 11 | 42 |
| 2017 Octubre | 23 | 8 | 31 |
| 2017 Septiembre | 30 | 8 | 38 |
| 2017 Agosto | 27 | 14 | 41 |
| 2017 Julio | 26 | 11 | 37 |
| 2017 Junio | 37 | 15 | 52 |
| 2017 Mayo | 45 | 17 | 62 |
| 2017 Abril | 34 | 10 | 44 |
| 2017 Marzo | 24 | 33 | 57 |
| 2017 Febrero | 23 | 9 | 32 |
| 2017 Enero | 24 | 9 | 33 |
| 2016 Diciembre | 24 | 15 | 39 |
| 2016 Noviembre | 44 | 16 | 60 |
| 2016 Octubre | 33 | 21 | 54 |
| 2016 Septiembre | 0 | 3 | 3 |
| 2016 Agosto | 0 | 3 | 3 |
| 2016 Julio | 6 | 1 | 7 |
| 2016 Junio | 9 | 7 | 16 |
| 2016 Mayo | 9 | 1 | 10 |
| 2016 Abril | 11 | 4 | 15 |
| 2016 Marzo | 7 | 10 | 17 |
| 2016 Febrero | 6 | 4 | 10 |
| 2016 Enero | 13 | 1 | 14 |
| 2015 Diciembre | 18 | 3 | 21 |
| 2015 Noviembre | 11 | 7 | 18 |
| 2015 Octubre | 10 | 3 | 13 |
| 2015 Septiembre | 6 | 5 | 11 |
| 2015 Agosto | 7 | 2 | 9 |
| 2015 Julio | 32 | 5 | 37 |
| 2015 Junio | 18 | 8 | 26 |
| 2015 Mayo | 29 | 8 | 37 |
| 2015 Abril | 11 | 7 | 18 |
| 2015 Marzo | 6 | 5 | 11 |
| 2015 Febrero | 7 | 5 | 12 |
| 2015 Enero | 7 | 1 | 8 |
| 2014 Diciembre | 7 | 3 | 10 |
| 2014 Noviembre | 8 | 7 | 15 |
| 2014 Octubre | 18 | 2 | 20 |
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