Cases for Diagnosis
Behind a Leonine Facies
Tras la facies leonina
M. Batista
, J.C. Cardoso, A. Figueiredo
Corresponding author
Departamento de Dermatología, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
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(b, c) Tropismo periecrino y perineural del infiltrado. (d) Conglomerados múltiples de bacilos (globis). (a-c) H&E, (a) x 40, (b) x 100, (c) x 400; (d) tinción de Fite-Faraco, x 200.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M. Batista, J.C. Cardoso, A. Figueiredo" "autores" => array:3 [ 0 => array:2 [ "nombre" => "M." "apellidos" => "Batista" ] 1 => array:2 [ "nombre" => "J.C." "apellidos" => "Cardoso" ] 2 => array:2 [ "nombre" => "A." 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Aparece una cicatriz residual debido a la biopsia cutánea previa. Mujer de 76 años de edad, quien recibió tratamiento con imiquimod 5% en régimen de monoterapia para lesión de lentigo maligno en mejilla derecha con hiperpigmentación postinflamatoria autolimitada (paciente 2, <a class="elsevierStyleCrossRef" href="#tbl0005">tabla 1</a>): <span class="elsevierStyleBold">E)</span> Ligera pigmentación gris, flecha negra; <span class="elsevierStyleBold">F)</span> En esta fotografía dermatoscópica aparecen estructuras anulares-granulares grisáceas alrededor de los folículos (salpicado); <span class="elsevierStyleBold">G)</span> Desaparición espontánea de la pigmentación tras diversas semanas, flecha negra. Varón de 76 años de edad con recidiva de lentigo maligno transcurridos dos meses de la finalización del tratamiento con imiquimod 5% en régimen de monoterapia (paciente 7, <a class="elsevierStyleCrossRef" href="#tbl0005">tabla 1</a>): <span class="elsevierStyleBold">H)</span> Pigmentación moteada marrón de distribución irregular en la región paranasal izquierda; <span class="elsevierStyleBold">I<span class="elsevierStyleSmallCaps">)</span></span> Fotografía dermatoscópica que muestra pigmentación marrón perifolicular asimétrica.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "I. Poveda-Montoyo, P. Álvarez-Chinchilla, L. Schneller-Pavelescu, P. Hispán-Ocete, J. Bañuls-Roca" "autores" => array:5 [ 0 => array:2 [ "nombre" => "I." "apellidos" => "Poveda-Montoyo" ] 1 => array:2 [ "nombre" => "P." "apellidos" => "Álvarez-Chinchilla" ] 2 => array:2 [ "nombre" => "L." "apellidos" => "Schneller-Pavelescu" ] 3 => array:2 [ "nombre" => "P." 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(b, c) Tropismo periecrino y perineural del infiltrado. (d) Conglomerados múltiples de bacilos (globis). (a-c) H&E, (a) x 40, (b) x 100, (c) x 400; (d) tinción de Fite-Faraco, x 200.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M. Batista, J.C. Cardoso, A. Figueiredo" "autores" => array:3 [ 0 => array:2 [ "nombre" => "M." "apellidos" => "Batista" ] 1 => array:2 [ "nombre" => "J.C." "apellidos" => "Cardoso" ] 2 => array:2 [ "nombre" => "A." 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"apellidos" => "Cardoso" ] 2 => array:2 [ "nombre" => "A." "apellidos" => "Figueiredo" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Departamento de Dermatología, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Tras la facies leonina" ] ] "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" => 655 "Ancho" => 872 "Tamanyo" => 229983 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">(a) Diffuse, pale, dermal infiltrate that spares the most superficial zone of the dermis (Grenz zone), consisting predominantly of foamy histiocytes. (b), (c) Perieccrine and perineural tropism of the infiltrate. (d) Multiple conglomerates of bacilli (globi). (a)–(c) H&E, (a) ×40, (b) ×100, (c) ×400; (d) Fite-Faraco's stain, ×200.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Patient history</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 36 year old woman presented with skin lesions and loss of sensation on the left foot that had been evolving for 2 years. She had Brazilian nationality but had been living in Portugal for 17 years.</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 erythematous or brown papulonodular lesions with a firm consistency on the upper lip, interciliary region, chin and nasal dorsum, with a fairly symmetrical distribution. Diffuse infiltration of the malar region gave the appearance of a leonine facies (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>a). Multiple subcutaneous nodules with hyperpigmentation of the overlying skin were symmetrically distributed on the shins (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>b). On neurological examination we documented decreased tactile, thermal and pain sensitivity of the left foot with no palpable nerve bundles or bone deformations.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Complementary tests</span><p id="par0015" class="elsevierStylePara elsevierViewall">A skin biopsy showed a diffuse dermal infiltrate with perieccrine and perineural tropism, predominantly of foamy histiocytes. (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>a–c) Fite-Faraco staining revealed numerous intracellular bacilli, forming dispersed clusters (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>d).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">What is the diagnosis?</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0025" class="elsevierStylePara elsevierViewall">Hasen's disease</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Treatment</span><p id="par0030" class="elsevierStylePara elsevierViewall">Multidrug therapy was initiated according to the WHO scheme (rifampicin 600<span class="elsevierStyleHsp" style=""></span>mg monthly, clofazimine 300<span class="elsevierStyleHsp" style=""></span>mg monthly and 50<span class="elsevierStyleHsp" style=""></span>mg daily, dapsone 100<span class="elsevierStyleHsp" style=""></span>mg daily).<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> Patient's close contacts were observed, none showing suspicious changes. A marked improvement in the clinical condition was observed including partial recovery of sensitivity in the first three weeks of therapy. So far, she has completed 7 months of treatment out of a total of 12–18 months.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comments</span><p id="par0035" class="elsevierStylePara elsevierViewall">Hansen's disease, caused by <span class="elsevierStyleItalic">Mycobacterium leprae</span>, typically affects the skin and peripheral nerves but can have a wide range of possible clinical manifestations.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> Eradicated in Portugal since the 1980s, Hansen's disease remains endemic in certain regions of Asia, Africa and Central and South America. Depending on the type of immunity shown by the host, and the intensity of response against the mycobacteria, the disease can be grouped into two major categories: lepromatous and tuberculoid, with intermediate forms between these two opposite poles.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> The incubation period varies considerably from 4 to 10 years, and it can be as long as 3 decades<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> Regarding treatment, promising drugs in the future include moxifloxacin, clarithromycin and rifapentine. The last has higher peak serum concentrations, a longer serum half-life, and more bactericidal activity against M. leprae than does rifampin. Also, moxifloxacin appears to be more bactericidal than ofloxacin.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4,5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Despite being a curable disease with an excellent prognosis, timely investigation and treatment of Hansen's disease is essential, as this slowly progressive chronic condition can result in stigmatizing major deformation and disability.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Our patient came from a high-risk country, and this information was paramount for our diagnostic suspicion, as other causes of leonine facies include leishmaniasis, cutaneous lymphoma and scleromyxedema, among others.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">This case highlights the need for awareness of the cutaneous manifestations of leprosy and remind us that, although rare in developed countries, in the current context of globalization it can still present in clinic today.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Funding</span><p id="par0060" class="elsevierStylePara elsevierViewall">None.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflict of interests</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflict of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Patient history" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Physical examination" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Complementary tests" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Diagnosis" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Treatment" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Comments" ] 6 => array:2 [ "identificador" => "sec0040" "titulo" => "Funding" ] 7 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflict of interests" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 438 "Ancho" => 656 "Tamanyo" => 52002 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(a) Multiple erythematous and pigmented papular lesions symmetrically distributed and diffuse infiltration of the malar regions. (b) Subcutaneous nodular lesions with overlying hyperpigmentation on the shin.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 655 "Ancho" => 872 "Tamanyo" => 229983 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">(a) Diffuse, pale, dermal infiltrate that spares the most superficial zone of the dermis (Grenz zone), consisting predominantly of foamy histiocytes. (b), (c) Perieccrine and perineural tropism of the infiltrate. (d) Multiple conglomerates of bacilli (globi). (a)–(c) H&E, (a) ×40, (b) ×100, (c) ×400; (d) Fite-Faraco's stain, ×200.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Global leprosy strategy 2016–2020: accelerating towards a leprosy-free world – 2016 operational manual" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "WHO" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Libro" => array:3 [ "fecha" => "2016" "editorial" => "WHO" "editorialLocalizacion" => "India" ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0040" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Leprosy in the 21st century" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "C. White" 1 => "C. Franco-Paredes" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1128/CMR.00079-13" "Revista" => array:6 [ "tituloSerie" => "Clin Microbiol Rev" "fecha" => "2015" "volumen" => "28" "paginaInicial" => "80" "paginaFinal" => "94" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25567223" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0045" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Mycobacterial infections" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "M. Ramos-e-Silva" 1 => "M. Castro" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "LibroEditado" => array:6 [ "paginaInicial" => "1296" "paginaFinal" => "1303" "edicion" => "4th ed." "serieVolumen" => "vol. 2" "serieTitulo" => "Dermatology" "serieFecha" => "2018" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0050" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinical trial of pefloxacin and ofloxacin in the treatment of lepromatous leprosy" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "J.H. Grosset" 1 => "B.H. Ji" 2 => "C.C. Guelpa-Lauras" 3 => "E.G. Perani" 4 => "L.N. N’Deli" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Int J Lepr Other Mycobact Dis" "fecha" => "1990" "volumen" => "58" "paginaInicial" => "281" "paginaFinal" => "295" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/2198315" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0055" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Leprosy therapy, past and present: can we hope to eliminate it?" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "P.V. Prasad" 1 => "P.K. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 20 | 14 | 34 |
| 2024 October | 146 | 80 | 226 |
| 2024 September | 162 | 74 | 236 |
| 2024 August | 191 | 94 | 285 |
| 2024 July | 145 | 67 | 212 |
| 2024 June | 144 | 70 | 214 |
| 2024 May | 148 | 71 | 219 |
| 2024 April | 148 | 46 | 194 |
| 2024 March | 128 | 64 | 192 |
| 2024 February | 119 | 52 | 171 |
| 2024 January | 138 | 57 | 195 |
| 2023 December | 99 | 52 | 151 |
| 2023 November | 146 | 53 | 199 |
| 2023 October | 122 | 79 | 201 |
| 2023 September | 97 | 51 | 148 |
| 2023 August | 79 | 49 | 128 |
| 2023 July | 113 | 66 | 179 |
| 2023 June | 92 | 29 | 121 |
| 2023 May | 116 | 44 | 160 |
| 2023 April | 74 | 27 | 101 |
| 2023 March | 88 | 36 | 124 |
| 2023 February | 97 | 29 | 126 |
| 2023 January | 95 | 46 | 141 |
| 2022 December | 99 | 48 | 147 |
| 2022 November | 91 | 51 | 142 |
| 2022 October | 96 | 52 | 148 |
| 2022 September | 70 | 53 | 123 |
| 2022 August | 77 | 53 | 130 |
| 2022 July | 111 | 39 | 150 |
| 2022 June | 89 | 61 | 150 |
| 2022 May | 145 | 63 | 208 |
| 2022 April | 42 | 34 | 76 |
| 2022 March | 24 | 63 | 87 |
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