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
Corresponding author
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" ]
| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 12 | 8 | 20 |
| 2024 October | 128 | 48 | 176 |
| 2024 September | 130 | 46 | 176 |
| 2024 August | 160 | 93 | 253 |
| 2024 July | 148 | 42 | 190 |
| 2024 June | 126 | 44 | 170 |
| 2024 May | 129 | 49 | 178 |
| 2024 April | 123 | 38 | 161 |
| 2024 March | 115 | 48 | 163 |
| 2024 February | 122 | 50 | 172 |
| 2024 January | 129 | 41 | 170 |
| 2023 December | 110 | 31 | 141 |
| 2023 November | 96 | 28 | 124 |
| 2023 October | 93 | 35 | 128 |
| 2023 September | 101 | 28 | 129 |
| 2023 August | 95 | 25 | 120 |
| 2023 July | 82 | 33 | 115 |
| 2023 June | 132 | 35 | 167 |
| 2023 May | 105 | 30 | 135 |
| 2023 April | 74 | 25 | 99 |
| 2023 March | 80 | 29 | 109 |
| 2023 February | 66 | 26 | 92 |
| 2023 January | 64 | 45 | 109 |
| 2022 December | 86 | 54 | 140 |
| 2022 November | 42 | 23 | 65 |
| 2022 October | 38 | 27 | 65 |
| 2022 September | 35 | 51 | 86 |
| 2022 August | 51 | 38 | 89 |
| 2022 July | 32 | 41 | 73 |
| 2022 June | 25 | 20 | 45 |
| 2022 May | 86 | 44 | 130 |
| 2022 April | 90 | 48 | 138 |
| 2022 March | 84 | 45 | 129 |
| 2022 February | 96 | 47 | 143 |
| 2022 January | 119 | 38 | 157 |
| 2021 December | 65 | 34 | 99 |
| 2021 November | 74 | 49 | 123 |
| 2021 October | 72 | 45 | 117 |
| 2021 September | 65 | 35 | 100 |
| 2021 August | 73 | 33 | 106 |
| 2021 July | 57 | 27 | 84 |
| 2021 June | 49 | 20 | 69 |
| 2021 May | 53 | 29 | 82 |
| 2021 April | 150 | 47 | 197 |
| 2021 March | 93 | 20 | 113 |
| 2021 February | 97 | 29 | 126 |
| 2021 January | 52 | 13 | 65 |
| 2020 December | 52 | 14 | 66 |
| 2020 November | 18 | 7 | 25 |
| 2020 October | 31 | 14 | 45 |
| 2020 September | 37 | 6 | 43 |
| 2020 August | 23 | 15 | 38 |
| 2020 July | 25 | 16 | 41 |
| 2020 June | 41 | 23 | 64 |
| 2020 May | 34 | 15 | 49 |
| 2020 April | 29 | 18 | 47 |
| 2020 March | 40 | 17 | 57 |
| 2020 February | 3 | 6 | 9 |
| 2020 January | 4 | 14 | 18 |
| 2019 December | 4 | 13 | 17 |
| 2019 November | 4 | 12 | 16 |
| 2019 October | 0 | 2 | 2 |
| 2019 September | 1 | 3 | 4 |
| 2019 August | 4 | 2 | 6 |
| 2019 July | 6 | 10 | 16 |
| 2019 June | 4 | 7 | 11 |
| 2019 May | 5 | 20 | 25 |
| 2019 April | 8 | 8 | 16 |
| 2019 March | 4 | 4 | 8 |
| 2019 February | 4 | 2 | 6 |
| 2019 January | 5 | 0 | 5 |
| 2018 December | 2 | 1 | 3 |
| 2018 November | 3 | 0 | 3 |
| 2018 October | 3 | 0 | 3 |
| 2018 September | 4 | 2 | 6 |
| 2018 August | 0 | 4 | 4 |
| 2018 July | 0 | 7 | 7 |
| 2018 June | 0 | 5 | 5 |
| 2018 May | 0 | 16 | 16 |
| 2018 April | 0 | 1 | 1 |
| 2018 March | 2 | 10 | 12 |
| 2018 February | 35 | 6 | 41 |
| 2018 January | 48 | 6 | 54 |
| 2017 December | 43 | 11 | 54 |
| 2017 November | 31 | 11 | 42 |
| 2017 October | 23 | 8 | 31 |
| 2017 September | 30 | 8 | 38 |
| 2017 August | 27 | 14 | 41 |
| 2017 July | 26 | 11 | 37 |
| 2017 June | 37 | 15 | 52 |
| 2017 May | 45 | 17 | 62 |
| 2017 April | 34 | 10 | 44 |
| 2017 March | 24 | 33 | 57 |
| 2017 February | 23 | 9 | 32 |
| 2017 January | 24 | 9 | 33 |
| 2016 December | 24 | 15 | 39 |
| 2016 November | 44 | 16 | 60 |
| 2016 October | 33 | 21 | 54 |
| 2016 September | 0 | 3 | 3 |
| 2016 August | 0 | 3 | 3 |
| 2016 July | 6 | 1 | 7 |
| 2016 June | 9 | 7 | 16 |
| 2016 May | 9 | 1 | 10 |
| 2016 April | 11 | 4 | 15 |
| 2016 March | 7 | 10 | 17 |
| 2016 February | 6 | 4 | 10 |
| 2016 January | 13 | 1 | 14 |
| 2015 December | 18 | 3 | 21 |
| 2015 November | 11 | 7 | 18 |
| 2015 October | 10 | 3 | 13 |
| 2015 September | 6 | 5 | 11 |
| 2015 August | 7 | 2 | 9 |
| 2015 July | 32 | 5 | 37 |
| 2015 June | 18 | 8 | 26 |
| 2015 May | 29 | 8 | 37 |
| 2015 April | 11 | 7 | 18 |
| 2015 March | 6 | 5 | 11 |
| 2015 February | 7 | 5 | 12 |
| 2015 January | 7 | 1 | 8 |
| 2014 December | 7 | 3 | 10 |
| 2014 November | 8 | 7 | 15 |
| 2014 October | 18 | 2 | 20 |
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