Case and Research Letters
Scarring Alopecia in Classic Adult Type I Pityriasis Rubra Pilaris
Alopecia cicatricial en pitiriasis rubra pilaris tipo I clásica del adulto
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Grandes placas de alopecia cicatricial con descamación folicular y eritema perifolicular parcheado que evolucionan a placas nacaradas atróficas con ausencia de eritema y descamación.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "C. Martín Callizo, J. Molinero Caturla, J. Sánchez Sánchez, R.M. Penín Mosquera" "autores" => array:4 [ 0 => array:2 [ "nombre" => "C." "apellidos" => "Martín Callizo" ] 1 => array:2 [ "nombre" => "J." "apellidos" => "Molinero Caturla" ] 2 => array:2 [ "nombre" => "J." "apellidos" => "Sánchez Sánchez" ] 3 => array:2 [ "nombre" => "R.M." 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(A) January 2011. (B) March 2012. The inflammatory margins progressed centrifugally upwards to the medial aspect of the thigh. (C) September 2012. The margins reached the left hip and gluteus.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "J.M. Mir-Bonafé, J.C. Santos-Durán, A. Santos-Briz, E. Fernández-López" "autores" => array:4 [ 0 => array:2 [ "nombre" => "J.M." "apellidos" => "Mir-Bonafé" ] 1 => array:2 [ "nombre" => "J.C." "apellidos" => "Santos-Durán" ] 2 => array:2 [ "nombre" => "A." "apellidos" => "Santos-Briz" ] 3 => array:2 [ "nombre" => "E." 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Martín Callizo, J. Molinero Caturla, J. Sánchez Sánchez, R.M. Penín Mosquera" "autores" => array:4 [ 0 => array:4 [ "nombre" => "C." "apellidos" => "Martín Callizo" "email" => array:1 [ 0 => "claramartincallizo@gmail.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" => "J." "apellidos" => "Molinero Caturla" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "J." "apellidos" => "Sánchez Sánchez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "R.M." "apellidos" => "Penín Mosquera" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitari de Bellvitge, Barcelona, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitari de Bellvitge, Barcelona, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Alopecia cicatricial en pitiriasis rubra pilaris tipo I clásica del adulto" ] ] "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" => 1168 "Ancho" => 1600 "Tamanyo" => 616465 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A and B, Skin biopsy of a pityriasis rubra pilaris lesion showing hyperkeratosis alternating with parakeratosis. A, Hematoxylin-eosin, original magnification ×4. B, Hematoxylin-eosin, original magnification ×10. C and D, Scalp biopsy with stellate perifollicular dermal fibrosis. No perifollicular inflammatory infiltrate is seen. C, Hematoxylin-eosin, original magnification ×10. D, Hematoxylin-eosin, original magnification ×20.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Pityriasis rubra pilaris (PRP) is an inflammatory disease of unknown etiology<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> with an estimated incidence ranging from 1 in 5000 to 1 in 50 000 dermatology patients. The established Griffiths classification recognizes 5 groups (types I-V) according to clinical features and patient age.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> More recently, Miralles et al.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> added a sixth group after observing clinical differences in cases associated with human immunodeficiency virus (HIV) infection. Type I (classic adult PRP) is the most common subtype. Complete remission is achieved within 3 years of onset in 80% of cases,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> whereas recurrence occurs in 20% of cases. Systemic retinoids, specifically acitretin, are the current first-line treatment.<span class="elsevierStyleVsp" style="height:0.5px"></span></p><p id="par0010" class="elsevierStylePara elsevierViewall">We report for the first time a case of classic adult type I PRP associated at first recurrence with rapidly developing disseminated scarring alopecia of the scalp. The patient was a 41-year-old woman with no relevant past history who presented with a pruritic rash that had appeared 3 weeks earlier at the same time as an orange-hued waxy palmoplantar keratoderma (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>, A). The trunk and the proximal extremities had orange-red lesions with some apparently healthy areas of skin (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>, B-D). Skin biopsy confirmed the suspected diagnosis of classic adult type I PRP. Findings included alternating hyperkeratosis and parakeratosis as well as psoriasiform acanthosis (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>, A and B). Treatment was started with 25 mg of oral acitretin daily. After 8 months of treatment the rash had cleared completely; we thus decided to discontinue treatment 2 months later. The patient returned after 10 lesion-free months complaining of a new crop of skin lesions similar to the original ones. This new bout occurred together with what appeared to be a severe form of scarring alopecia forming irregular patches on the scalp. The hairless patches were pearly and shiny, with fine scaling and perifollicular erythema. The clinical course was fulminant, and after 2 weeks the only scalp finding was atrophic alopecia patches with no erythema or scaling (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). A biopsy specimen obtained from the periphery of one of the hairless patches and comprising some follicles and perifollicular erythema exhibited significant perifollicular stellate fibrosis replacing the follicles, with no signs of inflammatory infiltrate (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>, C and D). Direct immunofluorescence was negative. Blood tests showed no abnormalities; test results for antinuclear antibodies, cryoglobulins, and antineutrophil cytoplasmic antibodies were all negative, and hepatitis B and C, HIV, and syphilis were ruled out. The rash cleared after 4 months of oral acitretin treatment, but the scarring alopecia persisted without change. The patient has worn a wig since then and has had no further bouts of PRP. Her scarring alopecia could not be reversed and remains stable at the time of writing in 2013, after 3 years of follow-up from initial presentation.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">The onset of scarring alopecia of the scalp in conjunction with a second bout of type I PRP suggested to us that alopecia might have been induced by the follicular involvement characteristic of PRP.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Although fine scaling of the scalp and face is frequent in type I PRP,<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and is sometimes accompanied by nonscarring alopecia,<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> an association of type I PRP with scarring alopecia has not been described before. In 1968, Bergeron and Stone<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> reported the case of a patient with PRP who developed acne conglobata, hidradenitis suppurativa, and dissecting cellulitis of the scalp. Acitretin-induced scarring alopecia has never before been reported,<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> and in any case, our patient was no longer taking acitretin at the onset of the second bout and of scarring alopecia. The follicular hyperkeratosis and perifollicular erythema of the scalp present during both bouts of type I PRP could have disturbed our patient's hair follicles enough to trigger fulminant scarring alopecia.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The clinical appearance of our patient's scarring alopecia is reminiscent of pseudopelade of Brocq in large, scattered patches. It is unclear whether pseudopelade is a disease in itself or the final stage of other, primary forms of scarring alopecia. In the initial stage, our patient's condition resembled lichen planopilaris or cutaneous lupus erythematosus. Nevertheless, the absence of any perifollicular lymphocytic inflammatory infiltrate, the replacement of hair follicles with dermal fibrosis, and the negative direct immunofluorescence test results make lichen, lupus, and pseudopelade unlikely diagnoses.</p><p id="par0030" class="elsevierStylePara elsevierViewall">We therefore consider this to be a case of scarring alopecia of an unusually fulminant course and severity associated with a bout of type I PRP, and with histologic findings that rule out a primary classic form of scarring alopecia. The alopecia underwent rapid clinical transformation into generalized pseudopelade in large patches, but the only histologic finding was fibrosis.</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: Martín Callizo C. Alopecia cicatricial en pitiriasis rubra pilaris tipo I clásica del adulto. Actas Dermosifiliogr. 2014;105:955–957.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 920 "Ancho" => 1500 "Tamanyo" => 267708 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">First bout of type I pityriasis rubra pilaris. A, Waxy palmar keratoderma. B-D, Scaly, orange-red patches on the trunk and limbs with islands of sparing and marked follicular hyperkeratosis.</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" => 1168 "Ancho" => 1600 "Tamanyo" => 616465 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A and B, Skin biopsy of a pityriasis rubra pilaris lesion showing hyperkeratosis alternating with parakeratosis. A, Hematoxylin-eosin, original magnification ×4. B, Hematoxylin-eosin, original magnification ×10. C and D, Scalp biopsy with stellate perifollicular dermal fibrosis. No perifollicular inflammatory infiltrate is seen. C, Hematoxylin-eosin, original magnification ×10. D, Hematoxylin-eosin, original magnification ×20.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 807 "Ancho" => 900 "Tamanyo" => 191931 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Second bout of type I pityriasis rubra pilaris. Large patches of scarring alopecia with follicular flaking and patchy perifollicular erythema that developed into atrophic pearly patches without erythema or scaling.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pityriasis rubra pilaris: a review of diagnosis and treatment" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "A. Klein" 1 => "M. Landthaler" 2 => "S. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 12 | 7 | 19 |
| 2024 Octubre | 78 | 40 | 118 |
| 2024 Septiembre | 107 | 32 | 139 |
| 2024 Agosto | 130 | 54 | 184 |
| 2024 Julio | 114 | 37 | 151 |
| 2024 Junio | 130 | 26 | 156 |
| 2024 Mayo | 129 | 32 | 161 |
| 2024 Abril | 112 | 38 | 150 |
| 2024 Marzo | 117 | 131 | 248 |
| 2024 Febrero | 110 | 47 | 157 |
| 2024 Enero | 112 | 39 | 151 |
| 2023 Diciembre | 114 | 16 | 130 |
| 2023 Noviembre | 161 | 31 | 192 |
| 2023 Octubre | 148 | 23 | 171 |
| 2023 Septiembre | 195 | 35 | 230 |
| 2023 Agosto | 132 | 25 | 157 |
| 2023 Julio | 121 | 37 | 158 |
| 2023 Junio | 81 | 35 | 116 |
| 2023 Mayo | 133 | 21 | 154 |
| 2023 Abril | 189 | 18 | 207 |
| 2023 Marzo | 162 | 26 | 188 |
| 2023 Febrero | 120 | 31 | 151 |
| 2023 Enero | 100 | 40 | 140 |
| 2022 Diciembre | 99 | 42 | 141 |
| 2022 Noviembre | 57 | 27 | 84 |
| 2022 Octubre | 57 | 17 | 74 |
| 2022 Septiembre | 92 | 46 | 138 |
| 2022 Agosto | 78 | 54 | 132 |
| 2022 Julio | 73 | 39 | 112 |
| 2022 Junio | 61 | 24 | 85 |
| 2022 Mayo | 116 | 45 | 161 |
| 2022 Abril | 140 | 33 | 173 |
| 2022 Marzo | 114 | 46 | 160 |
| 2022 Febrero | 142 | 29 | 171 |
| 2022 Enero | 191 | 35 | 226 |
| 2021 Diciembre | 112 | 28 | 140 |
| 2021 Noviembre | 114 | 43 | 157 |
| 2021 Octubre | 117 | 51 | 168 |
| 2021 Septiembre | 95 | 33 | 128 |
| 2021 Agosto | 81 | 24 | 105 |
| 2021 Julio | 99 | 22 | 121 |
| 2021 Junio | 124 | 28 | 152 |
| 2021 Mayo | 146 | 24 | 170 |
| 2021 Abril | 429 | 42 | 471 |
| 2021 Marzo | 174 | 14 | 188 |
| 2021 Febrero | 116 | 20 | 136 |
| 2021 Enero | 72 | 22 | 94 |
| 2020 Diciembre | 117 | 14 | 131 |
| 2020 Noviembre | 69 | 22 | 91 |
| 2020 Octubre | 77 | 15 | 92 |
| 2020 Septiembre | 61 | 17 | 78 |
| 2020 Agosto | 56 | 15 | 71 |
| 2020 Julio | 60 | 21 | 81 |
| 2020 Junio | 36 | 22 | 58 |
| 2020 Mayo | 40 | 21 | 61 |
| 2020 Abril | 36 | 18 | 54 |
| 2020 Marzo | 23 | 21 | 44 |
| 2020 Febrero | 8 | 9 | 17 |
| 2020 Enero | 0 | 3 | 3 |
| 2019 Diciembre | 4 | 3 | 7 |
| 2019 Noviembre | 0 | 12 | 12 |
| 2019 Octubre | 0 | 3 | 3 |
| 2019 Septiembre | 3 | 6 | 9 |
| 2019 Agosto | 0 | 14 | 14 |
| 2019 Julio | 4 | 13 | 17 |
| 2019 Junio | 0 | 12 | 12 |
| 2019 Mayo | 2 | 8 | 10 |
| 2019 Abril | 0 | 9 | 9 |
| 2019 Marzo | 0 | 6 | 6 |
| 2019 Febrero | 2 | 6 | 8 |
| 2019 Enero | 0 | 6 | 6 |
| 2018 Diciembre | 1 | 9 | 10 |
| 2018 Noviembre | 1 | 3 | 4 |
| 2018 Octubre | 1 | 1 | 2 |
| 2018 Septiembre | 3 | 2 | 5 |
| 2018 Julio | 0 | 1 | 1 |
| 2018 Mayo | 0 | 5 | 5 |
| 2018 Abril | 0 | 13 | 13 |
| 2018 Marzo | 1 | 0 | 1 |
| 2018 Febrero | 69 | 6 | 75 |
| 2018 Enero | 105 | 11 | 116 |
| 2017 Diciembre | 51 | 9 | 60 |
| 2017 Noviembre | 43 | 6 | 49 |
| 2017 Octubre | 42 | 4 | 46 |
| 2017 Septiembre | 27 | 8 | 35 |
| 2017 Agosto | 38 | 10 | 48 |
| 2017 Julio | 30 | 5 | 35 |
| 2017 Junio | 55 | 19 | 74 |
| 2017 Mayo | 22 | 21 | 43 |
| 2017 Abril | 33 | 15 | 48 |
| 2017 Marzo | 13 | 7 | 20 |
| 2017 Febrero | 24 | 8 | 32 |
| 2017 Enero | 13 | 19 | 32 |
| 2016 Diciembre | 27 | 8 | 35 |
| 2016 Noviembre | 36 | 10 | 46 |
| 2016 Octubre | 25 | 20 | 45 |
| 2016 Septiembre | 0 | 8 | 8 |
| 2016 Agosto | 0 | 2 | 2 |
| 2016 Julio | 4 | 1 | 5 |
| 2016 Junio | 11 | 6 | 17 |
| 2016 Mayo | 16 | 1 | 17 |
| 2016 Abril | 19 | 1 | 20 |
| 2016 Marzo | 19 | 1 | 20 |
| 2016 Febrero | 6 | 13 | 19 |
| 2016 Enero | 12 | 2 | 14 |
| 2015 Diciembre | 13 | 4 | 17 |
| 2015 Noviembre | 9 | 2 | 11 |
| 2015 Octubre | 17 | 3 | 20 |
| 2015 Septiembre | 15 | 1 | 16 |
| 2015 Agosto | 15 | 2 | 17 |
| 2015 Julio | 26 | 3 | 29 |
| 2015 Junio | 21 | 3 | 24 |
| 2015 Mayo | 6 | 8 | 14 |
| 2015 Abril | 5 | 9 | 14 |
| 2015 Marzo | 15 | 4 | 19 |
| 2015 Febrero | 7 | 2 | 9 |
| 2015 Enero | 3 | 5 | 8 |
| 2014 Diciembre | 4 | 2 | 6 |
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