Case for Diagnosis
Umbilicated Papules on the Elbows of a Woman With Systemic Vasculitis
Pápulas umbilicadas en los codos en una paciente con una vasculitis sistémica
C. Lloret-Ruiz
, N. Barrado-Solís, E. Quecedo-Estébanez
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Servicio de Dermatología, Hospital Arnau de Vilanova, Valencia, Spain
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Lloret-Ruiz, N. Barrado-Solís, E. Quecedo-Estébanez" "autores" => array:3 [ 0 => array:4 [ "nombre" => "C." "apellidos" => "Lloret-Ruiz" "email" => array:1 [ 0 => "clloretruiz@gmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "N." "apellidos" => "Barrado-Solís" ] 2 => array:2 [ "nombre" => "E." 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The patient came to our department for assessment of asymptomatic skin lesions that had appeared exclusively on her elbows in the previous 6 months.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed multiple mildly erythematous papular lesions measuring 2<span class="elsevierStyleHsp" style=""></span>mm in diameter, each with central umbilication, distributed symmetrically on both elbows (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">A punch biopsy of one lesion showed an inflammatory infiltrate in the superficial dermis containing histiocytes, several multinucleated giant cells, and lymphocytes expressing T and B immunohistochemical markers, without significant atypia or the presence of lymphocytes in the epidermis. Collagen degeneration was present, with a discrete mucin deposit. Elimination of a material consistent with degenerated collagen through epidermal interruptions was observed in certain areas of the lesion (hematoxylin-eosin, original magnification ×<span class="elsevierStyleHsp" style=""></span>100) (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What Is Your Diagnosis?</span></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0025" class="elsevierStylePara elsevierViewall">Perforating granuloma annulare.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course and Treatment</span><p id="par0030" class="elsevierStylePara elsevierViewall">The patient was informed that the process was benign and treatment was started with topical methylprednisolone. A slight improvement in the lesions was achieved after several weeks.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Perforating granuloma annulare (GA) is a rare variant of GA (occurring in 3%-5% of cases) that was first described by Calnan<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> in 1951 and later by Owens and Freeman<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> in 1971. The most common site is the limbs in children and young adults. Perforating GA has been reported in association with diabetes mellitus (especially in generalized cases), tuberculosis, rheumatoid arthritis, human immunodeficiency virus infection, tattoo application, and as Wolf's isotopic response after a herpes zoster infection.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The etiology and pathogenesis of perforating GA are not fully understood, although it is believed that this entity could be related to a delayed hypersensitivity reaction mediated by helper T cells in the presence of an exogenous antigen. Unlike other forms of GA, perforating GA lesions are located in the most superficial portion of the dermis due to the transepidermal elimination of degenerated collagen.</p><p id="par0045" class="elsevierStylePara elsevierViewall">The clinical presentation of perforating GA consists of skin-colored or slightly erythematous papules measuring 1-5<span class="elsevierStyleHsp" style=""></span>mm, with central umbilication. The papules can be scattered or grouped forming annular lesions, as in classic granuloma annulare.</p><p id="par0050" class="elsevierStylePara elsevierViewall">The differential diagnosis should include various entities. Acquired perforating dermatosis is the most common type of perforating disease, occurring in up to 10% of patients on hemodialysis; lesions typically appear on the extensor surfaces of the limbs and the clinical course is marked by self-resolving flares.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> In contrast, perforating GA follows a more chronic clinical course. Reactive perforating collagenosis is a rare familial disorder that first appears during childhood. As in perforating GA, the perforating material in reactive perforating collagenosis is collagen. In elastosis perforans serpiginosa, however, the perforating material consists of elastic fibers. The lesions consist of keratotic papules with a serpiginous distribution, affecting the neck, face, and flexural areas.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Follicular mycosis fungoides can manifest as papules centered on a follicle, which can be mistaken for the central umbilication seen in perforating GA, although histology shows a dense perifollicular infiltrate of atypical T cells, occasionally with involvement of the follicular epithelium, in addition to specific immunohistochemical characteristics. Follicular mycosis fungoides most frequently affects the head and neck, unlike perforating GA, which usually affects the limbs. In follicular mucinosis, the lesions tend to group in inflammatory plaques, sometimes causing alopecia, and can be either primary or secondary to a hematologic neoplasm, generally mycosis fungoides.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Given the autoimmune context of our patient, erythema elevatum diutinum was also considered in the differential diagnosis. This entity presents as erythematous brownish or purpuric papules or plaques on the extensor surfaces of the limbs and is characterized histologically by vasculitis with progressive concentric perivascular fibrosis.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Various treatments have been used in perforating GA, including topical corticosteroids or calcineurin inhibitors, imiquimod, intralesional corticosteroids, cryotherapy, and surgical excision, all with unsatisfactory results.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of Interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:8 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Medical History" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Physical Examination" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Histopathology" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Diagnosis" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Clinical Course and Treatment" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Comment" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflicts of Interest" ] 7 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Lloret-Ruiz C, Barrado-Solís N, Quecedo-Estébanez E. Pápulas umbilicadas en los codos en una paciente con una vasculitis sistémica. Actas Dermosifiliogr. 2018;109:353–354.</p>" ] ] "multimedia" => array:2 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 889 "Ancho" => 995 "Tamanyo" => 161045 ] ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 746 "Ancho" => 995 "Tamanyo" => 235356 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin-eosin, original magnification<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>100.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Granuloma annulare. Case history (summary)" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "C.D. Calnan" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:4 [ "tituloSerie" => "Br J Dermatol" "fecha" => "1954" "volumen" => "66" "paginaInicial" => "254" ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0035" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Perforating granuloma annulare" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "D.W. Owens" 1 => "R.G. 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Singh" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.4103/2229-5178.160286" "Revista" => array:6 [ "tituloSerie" => "Indian Dermatol Online J" "fecha" => "2015" "volumen" => "6" "paginaInicial" => "296" "paginaFinal" => "298" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26225344" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0045" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Skin manifestations of chronic kidney disease" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "J.C. Robles-Mendez" 1 => "O. Vazquez-Martinez" 2 => "J. 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Dou" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/jdv.13174" "Revista" => array:6 [ "tituloSerie" => "J Eur Acad Dermatol Venereol" "fecha" => "2016" "volumen" => "30" "paginaInicial" => "1246" "paginaFinal" => "1247" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25924054" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000010900000004/v1_201805040442/S157821901830043X/v1_201805040442/en/main.assets" "Apartado" => array:4 [ "identificador" => "6154" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Case for Diagnosis" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15782190/0000010900000004/v1_201805040442/S157821901830043X/v1_201805040442/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S157821901830043X?idApp=UINPBA000044" ]
| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 23 | 10 | 33 |
| 2024 Octubre | 120 | 48 | 168 |
| 2024 Septiembre | 125 | 35 | 160 |
| 2024 Agosto | 128 | 83 | 211 |
| 2024 Julio | 106 | 51 | 157 |
| 2024 Junio | 101 | 29 | 130 |
| 2024 Mayo | 101 | 38 | 139 |
| 2024 Abril | 85 | 34 | 119 |
| 2024 Marzo | 121 | 28 | 149 |
| 2024 Febrero | 88 | 41 | 129 |
| 2024 Enero | 83 | 27 | 110 |
| 2023 Diciembre | 98 | 15 | 113 |
| 2023 Noviembre | 142 | 25 | 167 |
| 2023 Octubre | 126 | 28 | 154 |
| 2023 Septiembre | 150 | 33 | 183 |
| 2023 Agosto | 129 | 25 | 154 |
| 2023 Julio | 138 | 32 | 170 |
| 2023 Junio | 92 | 27 | 119 |
| 2023 Mayo | 115 | 29 | 144 |
| 2023 Abril | 113 | 24 | 137 |
| 2023 Marzo | 95 | 31 | 126 |
| 2023 Febrero | 97 | 21 | 118 |
| 2023 Enero | 122 | 43 | 165 |
| 2022 Diciembre | 110 | 45 | 155 |
| 2022 Noviembre | 61 | 16 | 77 |
| 2022 Octubre | 85 | 32 | 117 |
| 2022 Septiembre | 92 | 33 | 125 |
| 2022 Agosto | 74 | 35 | 109 |
| 2022 Julio | 37 | 35 | 72 |
| 2022 Junio | 22 | 27 | 49 |
| 2022 Mayo | 81 | 39 | 120 |
| 2022 Abril | 108 | 32 | 140 |
| 2022 Marzo | 98 | 50 | 148 |
| 2022 Febrero | 103 | 35 | 138 |
| 2022 Enero | 116 | 47 | 163 |
| 2021 Diciembre | 88 | 52 | 140 |
| 2021 Noviembre | 100 | 57 | 157 |
| 2021 Octubre | 72 | 53 | 125 |
| 2021 Septiembre | 78 | 42 | 120 |
| 2021 Agosto | 69 | 40 | 109 |
| 2021 Julio | 70 | 35 | 105 |
| 2021 Junio | 65 | 36 | 101 |
| 2021 Mayo | 78 | 52 | 130 |
| 2021 Abril | 248 | 84 | 332 |
| 2021 Marzo | 140 | 37 | 177 |
| 2021 Febrero | 109 | 55 | 164 |
| 2021 Enero | 62 | 57 | 119 |
| 2020 Diciembre | 86 | 47 | 133 |
| 2020 Noviembre | 54 | 47 | 101 |
| 2020 Octubre | 41 | 21 | 62 |
| 2020 Septiembre | 65 | 26 | 91 |
| 2020 Agosto | 43 | 32 | 75 |
| 2020 Julio | 39 | 25 | 64 |
| 2020 Junio | 55 | 38 | 93 |
| 2020 Mayo | 25 | 19 | 44 |
| 2020 Abril | 27 | 15 | 42 |
| 2020 Marzo | 34 | 18 | 52 |
| 2020 Febrero | 4 | 0 | 4 |
| 2020 Enero | 4 | 0 | 4 |
| 2019 Diciembre | 8 | 0 | 8 |
| 2019 Noviembre | 4 | 0 | 4 |
| 2019 Septiembre | 11 | 0 | 11 |
| 2019 Agosto | 4 | 0 | 4 |
| 2019 Julio | 4 | 0 | 4 |
| 2019 Junio | 4 | 0 | 4 |
| 2019 Mayo | 4 | 0 | 4 |
| 2019 Abril | 2 | 0 | 2 |
| 2019 Marzo | 2 | 0 | 2 |
| 2019 Febrero | 2 | 0 | 2 |
| 2018 Diciembre | 4 | 0 | 4 |
| 2018 Noviembre | 1 | 0 | 1 |
| 2018 Septiembre | 2 | 0 | 2 |
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