Case for Diagnosis
Periorificial Lesions in a Young Girl
Lesiones periorificiales en una niña
A. Mayor-Ibarguren
, R. Maseda-Pedrero, M. Feito-Rodríguez
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Servicio de Dermatología, Hospital Universitario La Paz, Madrid, Spain
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Mayor-Ibarguren, R. Maseda-Pedrero, M. Feito-Rodríguez" "autores" => array:3 [ 0 => array:4 [ "nombre" => "A." "apellidos" => "Mayor-Ibarguren" "email" => array:1 [ 0 => "andermayor@gmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "R." "apellidos" => "Maseda-Pedrero" ] 2 => array:2 [ "nombre" => "M." "apellidos" => "Feito-Rodríguez" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología, Hospital Universitario La Paz, Madrid, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Lesiones periorificiales en una niña" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 401 "Ancho" => 975 "Tamanyo" => 78944 ] ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">An 8-year-old girl with no relevant personal or family medical history presented with asymptomatic facial lesions that had appeared several months earlier and had not responded to topical corticosteroids. The patient had no history of atopic dermatitis. She was in good general health, with no joint pain, fever, abdominal pain, or weight loss.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">The patient presented numerous firm, skin-colored, monomorphous papules distributed in periorificial areas (perioral, perinasal, periocular), accompanied by erythema and desquamation (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Similar lesions were present in the vulvar area (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). The physical examination was otherwise completely normal.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">A biopsy of a vulvar lesion revealed a dense lymphohistiocytic perivascular infiltrate in the upper dermis as well as the formation of noncaseating epithelioid granulomas (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). The epidermis was spared. Ziehl-Neelsen and periodic acid-Schiff stains were negative. The presence of <span class="elsevierStyleItalic">Demodex folliculorum</span> was not detected in the follicles.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">A complete blood count and biochemistry profile revealed no abnormalities. Levels of angiotensin-converting enzyme (ACE) and immunoglobulins were normal. A Mantoux test was negative and a chest radiograph showed no anomalies.</p><p id="par0025" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What Is Your Diagnosis?</span></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Childhood granulomatous periorificial dermatitis (CGPD).</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Topical corticosteroids were suspended. Treatment with oral erythromycin was started, but little improvement was noted. The patient was switched to oral metronidazole (250<span class="elsevierStyleHsp" style=""></span>mg/d). A striking improvement was noted after 2 weeks and the lesions had resolved completely at 4 weeks.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Childhood granulomatous periorificial dermatitis (CGPD) is a benign, self-limited skin disease that has many characteristics in common with the perioral dermatitis typically seen in middle-aged women. The disease was first described in 1970 by Gianotti,<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> who reported a case series of 5 children with monomorphous papules that resembled perioral dermatitis. Other names have been used to describe this entity, including facial Afro-Caribbean cutaneous eruption (FACE), sarcoid-like granulomatous dermatitis, and Gianotti-type perioral dermatitis.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> The term <span class="elsevierStyleItalic">childhood granulomatous periorificial dermatitis</span>, first used by Urbatsch et al.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> in 1989, is currently the most widely accepted term because it most accurately encompasses the characteristics of the entity. CGPD usually affects prepubertal children, and the incidence of the disease may be higher in black patients, although publication bias may account for the difference.</p><p id="par0045" class="elsevierStylePara elsevierViewall">CGPD is characterized by the appearance of lesions in the form of monomorphous skin-colored papules distributed across the periorificial regions of the face, which may be accompanied by erythema and desquamation. Unlike perioral dermatitis, CGPD generally does not manifest with pustules.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Extrafacial involvement can include the perivulvar region, the neck, the upper trunk, and the limbs. The lesions are usually asymptomatic and tend to resolve without scarring. The etiology of CGPD is unknown, but it appears that topical corticosteroids, especially fluorinated corticosteroids, can trigger or exacerbate the process.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Histologic findings show a perivascular and perifollicular lymphohistiocytic infiltrate and the formation of epithelioid granulomas that can be either caseating or noncaseating.<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2,3</span></a> The presence of granulomas is not a necessary criterion for diagnosis. The differential diagnosis should include rosacea, acne vulgaris, sarcoidosis, Blau syndrome, lupus miliaris disseminatus faciei, candidiasis, angular cheilitis, atopic dermatitis, demodicidosis, and benign cephalic histiocytosis.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> It is important to ask about constitutional symptoms, joint pain, and respiratory involvement. A laboratory workup including angiotensin-converting enzyme levels and a chest radiograph should be ordered in order to rule out sarcoidosis. In the case of our patient, it was possible to rule out these possible diagnoses on the basis of the medical history and the additional test results.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Treatment options include the suspension of topical corticosteroids and the application of various topical antibiotics, including erythromycin and 0.75% metronidazole, with variable results. In patients with extensive or refractory lesions, systemic treatment with erythromycin or doxycycline is recommended (the latter in children older than 8 years of age).<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> A recent case report described the successful treatment of CGPD with oral metronidazole, as in our patient.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></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" => "Additional Tests" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Diagnosis" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Clinical Course and Treatment" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Comment" ] 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: Mayor-Ibarguren A. Lesiones periorificiales en una niña. Actas Dermosifiliogr. 2016;107:773–774.</p>" ] ] "multimedia" => array:3 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 401 "Ancho" => 975 "Tamanyo" => 78944 ] ] ] 1 => array:6 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 336 "Ancho" => 400 "Tamanyo" => 40278 ] ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 302 "Ancho" => 400 "Tamanyo" => 86864 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin-eosin, original magnification ×100.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Particuliére dermatite periorale infantile. 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Lesher" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Pediatr Dermatol" "fecha" => "1996" "volumen" => "13" "paginaInicial" => "131" "paginaFinal" => "134" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/9122070" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0055" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Childhood granulomatous periorificial dermatitis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "H.C. Williams" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:4 [ "tituloSerie" => "Pediatr Dermatol" "fecha" => "1996" "volumen" => "6" "paginaInicial" => "515" ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0060" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Childhood granulomatous periorificial dermatitis with a good response to oral metronidazole" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "C. Rodríguez-Caruncho" 1 => "I. Bielsa" 2 => "M.T. Fernández-Figueras" 3 => "C. Ferrándiz" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/pde.12110" "Revista" => array:6 [ "tituloSerie" => "Pediatr Dermatol" "fecha" => "2013" "volumen" => "30" "paginaInicial" => "e98" "paginaFinal" => "e99" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23437890" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000010700000009/v1_201611010039/S1578219016301883/v1_201611010039/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/15782190/0000010700000009/v1_201611010039/S1578219016301883/v1_201611010039/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219016301883?idApp=UINPBA000044" ]
| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 15 | 14 | 29 |
| 2024 Octubre | 69 | 49 | 118 |
| 2024 Septiembre | 89 | 32 | 121 |
| 2024 Agosto | 110 | 51 | 161 |
| 2024 Julio | 87 | 42 | 129 |
| 2024 Junio | 73 | 52 | 125 |
| 2024 Mayo | 88 | 44 | 132 |
| 2024 Abril | 67 | 26 | 93 |
| 2024 Marzo | 73 | 32 | 105 |
| 2024 Febrero | 77 | 40 | 117 |
| 2024 Enero | 48 | 25 | 73 |
| 2023 Diciembre | 55 | 29 | 84 |
| 2023 Noviembre | 63 | 24 | 87 |
| 2023 Octubre | 55 | 27 | 82 |
| 2023 Septiembre | 64 | 32 | 96 |
| 2023 Agosto | 57 | 15 | 72 |
| 2023 Julio | 46 | 24 | 70 |
| 2023 Junio | 34 | 19 | 53 |
| 2023 Mayo | 37 | 23 | 60 |
| 2023 Abril | 45 | 25 | 70 |
| 2023 Marzo | 33 | 32 | 65 |
| 2023 Febrero | 43 | 30 | 73 |
| 2023 Enero | 43 | 20 | 63 |
| 2022 Diciembre | 67 | 34 | 101 |
| 2022 Noviembre | 42 | 32 | 74 |
| 2022 Octubre | 72 | 23 | 95 |
| 2022 Septiembre | 23 | 23 | 46 |
| 2022 Agosto | 75 | 27 | 102 |
| 2022 Julio | 65 | 34 | 99 |
| 2022 Junio | 28 | 26 | 54 |
| 2022 Mayo | 35 | 28 | 63 |
| 2022 Abril | 32 | 26 | 58 |
| 2022 Marzo | 52 | 36 | 88 |
| 2022 Febrero | 31 | 22 | 53 |
| 2022 Enero | 43 | 36 | 79 |
| 2021 Diciembre | 34 | 33 | 67 |
| 2021 Noviembre | 31 | 35 | 66 |
| 2021 Octubre | 43 | 46 | 89 |
| 2021 Septiembre | 21 | 43 | 64 |
| 2021 Agosto | 22 | 25 | 47 |
| 2021 Julio | 31 | 26 | 57 |
| 2021 Junio | 26 | 21 | 47 |
| 2021 Mayo | 33 | 32 | 65 |
| 2021 Abril | 69 | 46 | 115 |
| 2021 Marzo | 68 | 26 | 94 |
| 2021 Febrero | 44 | 25 | 69 |
| 2021 Enero | 24 | 21 | 45 |
| 2020 Diciembre | 24 | 12 | 36 |
| 2020 Noviembre | 21 | 15 | 36 |
| 2020 Octubre | 17 | 13 | 30 |
| 2020 Septiembre | 37 | 12 | 49 |
| 2020 Agosto | 23 | 18 | 41 |
| 2020 Julio | 18 | 13 | 31 |
| 2020 Junio | 38 | 24 | 62 |
| 2020 Mayo | 21 | 10 | 31 |
| 2020 Abril | 27 | 12 | 39 |
| 2020 Marzo | 33 | 16 | 49 |
| 2020 Febrero | 6 | 2 | 8 |
| 2019 Diciembre | 4 | 0 | 4 |
| 2019 Septiembre | 4 | 0 | 4 |
| 2019 Junio | 2 | 0 | 2 |
| 2019 Mayo | 1 | 0 | 1 |
| 2019 Abril | 0 | 4 | 4 |
| 2019 Marzo | 2 | 0 | 2 |
| 2019 Febrero | 3 | 0 | 3 |
| 2019 Enero | 3 | 0 | 3 |
| 2018 Diciembre | 17 | 0 | 17 |
| 2018 Noviembre | 13 | 0 | 13 |
| 2018 Octubre | 3 | 0 | 3 |
| 2018 Septiembre | 3 | 0 | 3 |
| 2018 Febrero | 29 | 6 | 35 |
| 2018 Enero | 107 | 14 | 121 |
| 2017 Diciembre | 102 | 7 | 109 |
| 2017 Noviembre | 49 | 7 | 56 |
| 2017 Octubre | 39 | 13 | 52 |
| 2017 Septiembre | 37 | 12 | 49 |
| 2017 Agosto | 31 | 6 | 37 |
| 2017 Julio | 42 | 9 | 51 |
| 2017 Junio | 35 | 8 | 43 |
| 2017 Mayo | 32 | 8 | 40 |
| 2017 Abril | 32 | 13 | 45 |
| 2017 Marzo | 23 | 8 | 31 |
| 2017 Febrero | 20 | 9 | 29 |
| 2017 Enero | 20 | 8 | 28 |
| 2016 Diciembre | 37 | 23 | 60 |
| 2016 Noviembre | 53 | 38 | 91 |
| 2016 Octubre | 12 | 10 | 22 |
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