Case for diagnosis
Nodular-Cystic Lesions on a Congenital Alopecic Plaque
Lesiones nódulo-quísticas sobre placa de alopecia congénita
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Fuentelsaz-del Barrio, V. Parra Blanco, D.E. Cieza Díaz" "autores" => array:3 [ 0 => array:4 [ "nombre" => "V." "apellidos" => "Fuentelsaz-del Barrio" "email" => array:1 [ 0 => "victoriafuentelsaz@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "V." "apellidos" => "Parra Blanco" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "D.E." "apellidos" => "Cieza Díaz" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Gregorio Marañón, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Gregorio Marañón, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Lesiones nódulo-quísticas sobre placa de alopecia congénita" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 601 "Ancho" => 995 "Tamanyo" => 187762 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Hematoxylin-eosin, original magnification ×<span class="elsevierStyleHsp" style=""></span>40. B, Hematoxylin-eosin, original magnification ×<span class="elsevierStyleHsp" style=""></span>40.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Case Description</span><p id="par0005" class="elsevierStylePara elsevierViewall">We present the case of a 40-year-old woman with no relevant medical history who presented with asymptomatic nodular-cystic lesions on a congenital alopecic plaque on the scalp. The lesions had developed slowly and progressively over the previous 5 years. The patient reported that her family physician had occasionally incised and drained some of the lesions. No cases of similar lesions were reported in her family history.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">We observed yellowish papules and skin-colored nodular-cystic lesions measuring 0.3 to 1<span class="elsevierStyleHsp" style=""></span>cm in diameter that were soft on palpation and located on an alopecic plaque on the left frontotemporal region. The lesions extended to the left malar region (<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">Histology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Several biopsies were taken from the area. Histologic findings revealed multilocular cystic epithelial tumors surrounded by a well-differentiated, flat epithelium with a crenulated lining that had an eosinophilic cuticle (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>, A and B). In addition, mature sebaceous glands were observed to be in close contact with the epithelium. Some of these glands opened into the lumen of the cyst.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par1020" 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">Steatocystoma multiplex (SM) on a congenital alopecic plaque on the scalp</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course and Treatment</span><p id="par0030" class="elsevierStylePara elsevierViewall">Simple excision was performed on several of the larger lesions, and although the esthetic outcome was favorable, the patient rejected further treatment.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0035" class="elsevierStylePara elsevierViewall">SM is considered to result from hamartomatous malformations of the pilosebaceous unit. It is a rare condition that typically affects adolescents or young adults. Although it follows an autosomal dominant inheritance pattern, many sporadic cases have been described. Clinically, it consists of yellowish or skin-colored papules and nodules measuring 0.2 to 2<span class="elsevierStyleHsp" style=""></span>cm in diameter that typically appear on the anterior aspect of the trunk and on the neck, axillas, thighs, and scrotum. Presentation on the scalp is rare and, unlike SM in more typical areas, it is not associated with an autosomal dominant inheritance pattern and appears in older individuals,<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> as in our patient.</p><p id="par0040" class="elsevierStylePara elsevierViewall">SM affecting the face and scalp has been classified into 3 types: a) a facial papular variant,<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,4</span></a> b) sebocystomatosis,<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> and c) lesions confined to the scalp.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2,6,7</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Only 12 cases of SM on the scalp have been described in the literature to date, and just 2 of these cases involved concurrent alopecic plaques. In 1996 Lee et al.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> reported a case of SM on the face and on a congenital alopecic plaque in the frontal region that they considered to be a clinical variant of this condition. The variant, which they called <span class="elsevierStyleItalic">sebocystomatosis</span>, had very similar clinical characteristics to those seen in our patient. The second published case of SM with concurrent alopecia on the scalp was described by Lee et al.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> in 2011. In that case the lesions appeared on an alopecic plaque caused by trichotillomania.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Some authors have described cases of SM associated with other disorders such as ichthyosis, koilonychia, pachyonychia congenita, acrokeratosis verruciformis of Hopf, hypertrophic lichen planus, hypohidrosis, hypothyroidism, hidradenitis suppurativa, and hypotrichosis.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> No associated conditions, however, have yet been described in patients with SM affecting the scalp only. Our patient had no other cutaneous manifestations or disorders.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Histologically, SM consists of cystic lesions that are empty, partially collapsed, folded, and covered with a wall formed by a stratified squamous epithelium lacking a granular layer and lined with a crenulated cuticle. Sebaceous gland lobules embedded in the cyst wall are also a very characteristic finding.</p><p id="par0060" class="elsevierStylePara elsevierViewall">The clinical differential diagnosis typically includes eruptive vellus hair cysts, the histology of which shows cysts containing keratin and vellus hair surrounded by a thin wall comprised of several layers of squamous cells. As in SM, the sebaceous glands may form part of the cyst wall.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Other diagnoses to consider are epidermal cysts, milia cysts, neurofibromas, and xanthomas.</p><p id="par0070" class="elsevierStylePara elsevierViewall">Although most SM lesions are asymptomatic, they may cause serious esthetic concern. Treatment with simple excision or drainage of cyst content may produce satisfactory results. In cases of inflamed lesions, treatment with locally injected corticosteroids, laser treatment, oral retinoids, and cryotherapy may be effective.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of Interest</span><p id="par0075" 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" => "Case Description" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Physical Examination" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Histology" ] 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 "fechaRecibido" => "2013-04-02" "fechaAceptado" => "2013-08-09" "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Fuentelsaz-del Barrio V, Parra Blanco V, Cieza Díaz D. Lesiones nódulo-quísticas sobre placa de alopecia congénita. Actas Dermosifiliogr. 2014;105:85–86.</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" => 651 "Ancho" => 739 "Tamanyo" => 126826 ] ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 601 "Ancho" => 995 "Tamanyo" => 187762 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Hematoxylin-eosin, original magnification ×<span class="elsevierStyleHsp" style=""></span>40. B, Hematoxylin-eosin, original magnification ×<span class="elsevierStyleHsp" style=""></span>40.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:8 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A case of steatocystoma multiplex limited to the scalp" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "S.J. Kim" 1 => "H.J. Park" 2 => "S.T. Oh" 3 => "J.Y. Lee" 4 => "B.K. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 9 | 10 | 19 |
| 2024 Octubre | 64 | 35 | 99 |
| 2024 Septiembre | 77 | 19 | 96 |
| 2024 Agosto | 117 | 52 | 169 |
| 2024 Julio | 71 | 36 | 107 |
| 2024 Junio | 89 | 29 | 118 |
| 2024 Mayo | 84 | 26 | 110 |
| 2024 Abril | 90 | 22 | 112 |
| 2024 Marzo | 84 | 29 | 113 |
| 2024 Febrero | 73 | 32 | 105 |
| 2024 Enero | 64 | 33 | 97 |
| 2023 Diciembre | 69 | 15 | 84 |
| 2023 Noviembre | 71 | 32 | 103 |
| 2023 Octubre | 95 | 35 | 130 |
| 2023 Septiembre | 81 | 37 | 118 |
| 2023 Agosto | 54 | 16 | 70 |
| 2023 Julio | 117 | 32 | 149 |
| 2023 Junio | 75 | 19 | 94 |
| 2023 Mayo | 84 | 21 | 105 |
| 2023 Abril | 68 | 14 | 82 |
| 2023 Marzo | 41 | 23 | 64 |
| 2023 Febrero | 69 | 20 | 89 |
| 2023 Enero | 38 | 21 | 59 |
| 2022 Diciembre | 63 | 53 | 116 |
| 2022 Noviembre | 33 | 26 | 59 |
| 2022 Octubre | 30 | 20 | 50 |
| 2022 Septiembre | 27 | 31 | 58 |
| 2022 Agosto | 21 | 35 | 56 |
| 2022 Julio | 30 | 31 | 61 |
| 2022 Junio | 29 | 25 | 54 |
| 2022 Mayo | 54 | 35 | 89 |
| 2022 Abril | 52 | 42 | 94 |
| 2022 Marzo | 50 | 51 | 101 |
| 2022 Febrero | 35 | 29 | 64 |
| 2022 Enero | 45 | 35 | 80 |
| 2021 Diciembre | 32 | 43 | 75 |
| 2021 Noviembre | 52 | 51 | 103 |
| 2021 Octubre | 64 | 53 | 117 |
| 2021 Septiembre | 37 | 44 | 81 |
| 2021 Agosto | 50 | 22 | 72 |
| 2021 Julio | 33 | 35 | 68 |
| 2021 Junio | 35 | 32 | 67 |
| 2021 Mayo | 46 | 30 | 76 |
| 2021 Abril | 59 | 65 | 124 |
| 2021 Marzo | 71 | 21 | 92 |
| 2021 Febrero | 50 | 32 | 82 |
| 2021 Enero | 39 | 11 | 50 |
| 2020 Diciembre | 51 | 20 | 71 |
| 2020 Noviembre | 20 | 13 | 33 |
| 2020 Octubre | 24 | 10 | 34 |
| 2020 Septiembre | 35 | 11 | 46 |
| 2020 Agosto | 26 | 18 | 44 |
| 2020 Julio | 28 | 14 | 42 |
| 2020 Junio | 35 | 19 | 54 |
| 2020 Mayo | 24 | 19 | 43 |
| 2020 Abril | 43 | 14 | 57 |
| 2020 Marzo | 35 | 27 | 62 |
| 2020 Febrero | 3 | 7 | 10 |
| 2020 Enero | 4 | 13 | 17 |
| 2019 Diciembre | 8 | 0 | 8 |
| 2019 Noviembre | 4 | 1 | 5 |
| 2019 Septiembre | 10 | 0 | 10 |
| 2019 Agosto | 4 | 1 | 5 |
| 2019 Julio | 4 | 6 | 10 |
| 2019 Junio | 4 | 1 | 5 |
| 2019 Mayo | 5 | 4 | 9 |
| 2019 Abril | 2 | 12 | 14 |
| 2019 Marzo | 0 | 7 | 7 |
| 2019 Febrero | 5 | 4 | 9 |
| 2019 Enero | 2 | 0 | 2 |
| 2018 Diciembre | 0 | 4 | 4 |
| 2018 Noviembre | 4 | 0 | 4 |
| 2018 Octubre | 1 | 0 | 1 |
| 2018 Septiembre | 3 | 0 | 3 |
| 2018 Mayo | 0 | 1 | 1 |
| 2018 Marzo | 4 | 1 | 5 |
| 2018 Febrero | 73 | 6 | 79 |
| 2018 Enero | 47 | 6 | 53 |
| 2017 Diciembre | 73 | 5 | 78 |
| 2017 Noviembre | 41 | 3 | 44 |
| 2017 Octubre | 49 | 3 | 52 |
| 2017 Septiembre | 47 | 3 | 50 |
| 2017 Agosto | 44 | 5 | 49 |
| 2017 Julio | 36 | 4 | 40 |
| 2017 Junio | 64 | 14 | 78 |
| 2017 Mayo | 85 | 2 | 87 |
| 2017 Abril | 46 | 5 | 51 |
| 2017 Marzo | 59 | 13 | 72 |
| 2017 Febrero | 138 | 6 | 144 |
| 2017 Enero | 36 | 9 | 45 |
| 2016 Diciembre | 49 | 13 | 62 |
| 2016 Noviembre | 106 | 10 | 116 |
| 2016 Octubre | 119 | 9 | 128 |
| 2016 Septiembre | 195 | 17 | 212 |
| 2016 Agosto | 183 | 7 | 190 |
| 2016 Julio | 50 | 12 | 62 |
| 2016 Junio | 9 | 13 | 22 |
| 2016 Mayo | 17 | 7 | 24 |
| 2016 Abril | 13 | 11 | 24 |
| 2016 Marzo | 12 | 1 | 13 |
| 2016 Febrero | 8 | 17 | 25 |
| 2016 Enero | 8 | 20 | 28 |
| 2015 Diciembre | 7 | 11 | 18 |
| 2015 Noviembre | 9 | 16 | 25 |
| 2015 Octubre | 9 | 9 | 18 |
| 2015 Septiembre | 8 | 1 | 9 |
| 2015 Agosto | 5 | 2 | 7 |
| 2015 Julio | 49 | 6 | 55 |
| 2015 Junio | 36 | 3 | 39 |
| 2015 Mayo | 44 | 2 | 46 |
| 2015 Abril | 23 | 11 | 34 |
| 2015 Marzo | 32 | 4 | 36 |
| 2015 Febrero | 24 | 7 | 31 |
| 2015 Enero | 21 | 3 | 24 |
| 2014 Diciembre | 24 | 3 | 27 |
| 2014 Noviembre | 21 | 6 | 27 |
| 2014 Octubre | 26 | 5 | 31 |
| 2014 Septiembre | 26 | 5 | 31 |
| 2014 Agosto | 15 | 4 | 19 |
| 2014 Julio | 31 | 9 | 40 |
| 2014 Junio | 16 | 4 | 20 |
| 2014 Mayo | 28 | 7 | 35 |
| 2014 Abril | 24 | 3 | 27 |
| 2014 Marzo | 17 | 3 | 20 |
| 2014 Febrero | 4 | 1 | 5 |
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