Case for Diagnosis
Papular Eruption on Sun-Exposed Skin
Erupción papulosa fotodistribuida
L. Schneller-Pavelescu
, E. Vergara-de Caso, M.M. Blanes
Autor para correspondencia
Servicio de Dermatología, Hospital General Universitario de Alicante, Alicante, Spain
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Schneller-Pavelescu, E. Vergara-de Caso, M.M. Blanes" "autores" => array:3 [ 0 => array:4 [ "nombre" => "L." "apellidos" => "Schneller-Pavelescu" "email" => array:1 [ 0 => "lucapavelescu@gmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "E." "apellidos" => "Vergara-de Caso" ] 2 => array:2 [ "nombre" => "M.M." "apellidos" => "Blanes" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología, Hospital General Universitario de Alicante, Alicante, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Erupción papulosa fotodistribuida" ] ] "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" => 1191 "Ancho" => 1667 "Tamanyo" => 468440 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Hematoxylin–eosin ×4. B and C), Hematoxylin–eosin ×10.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 52-year-old woman presented with pruritic dermatitis that had been treated in different hospitals for more than 40 years. Atopic dermatitis was suspected during childhood and the patient was treated with cyclosporin A with some improvement. In 2002, she presented with facial nodules that were histologically compatible with B-cell lymphoma (CD20<span class="elsevierStyleSup">+</span>, BCL2<span class="elsevierStyleSup">+</span>, IgH rearrangement) and treatment with cyclosporin was suspended. After ruling out extracutaneous involvement, she was given intravenous rituximab at a dosage of 375<span class="elsevierStyleHsp" style=""></span>mg/m<span class="elsevierStyleSup">2</span> in 4 weekly cycles. Symptoms improved but reappeared when treatment was suspended. In 2013, the patient presented again with facial nodules that were histologically compatible with B-cell lymphoid hyperplasia. the patient was treated with rituximab and showed an identical response.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Due to a change of address, she was sent to our department in 2016 with no definitive diagnosis. On questioning, she confirmed the recurrence of the lesions after long periods of exposure to sunlight.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0015" class="elsevierStylePara elsevierViewall">The patient presented erythematous papules, many of which showed central excoriation, that were confluent in some locations (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A), predominantly in the areas exposed to sunlight (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). Some residual hyperpigmented macules were visible in these areas.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0020" class="elsevierStylePara elsevierViewall">Biopsies were taken of a crusted papular lesion and an erythematous plaque on the temple, which the patient had stated were similar to those diagnosed as cutaneous lymphoma/lymphoid hyperplasia; both lesions were histologically similar (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>A): superficial perivascular dermal infiltrate (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>B) and deep T lymphocyte infiltrate with abundant eosinophils (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>C).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Other Tests</span><p id="par0025" class="elsevierStylePara elsevierViewall">HLA determination revealed the HLA-DR4 variant (DRB1*04:08). The autoimmune study was negative and IgE was normal.</p><p id="par0030" class="elsevierStylePara elsevierViewall">What is your Diagnosis?</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0035" class="elsevierStylePara elsevierViewall">Actinic prurigo (AP)</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Treatment and Course</span><p id="par0040" class="elsevierStylePara elsevierViewall">Narrow-band UVB phototherapy was instated to stimulate habituation, with irregular compliance by the patient, who finally abandoned this treatment despite some improvement of the condition. Thalidomide does not currently appear to be an option because the patient suffers from bilateral carpal tunnel syndrome, which makes monitoring potential neuropathy difficult. The patient is currently undergoing treatment with topical corticosteroids, oral antihistamines, and photoprotection, and maintains mild symptoms.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0045" class="elsevierStylePara elsevierViewall">AP is a frequent photodermatosis in Native Americans and few cases have been reported in the European population.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> The forms that present in childhood, generally before the age of 10 years, may improve during adolescence. Later onset, though less frequent, tends to be associated with persistence into adulthood.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1–3</span></a> Half of patients present a family history of the disease and nearly 90% have HLA-DR4, and HLA-DRB1*04:07 is present in between 60% and 70% of patients.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">3,4</span></a> Our patient reported that her father had presented a similar dermatosis.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Clinical presentation consists of erythematous pruritic papulonodular lesions with excoriation due to scratching, predominantly in the areas exposed to sunlight, although they may also appear in unexposed areas. Associated clinical findings such as cheilitis (46% of cases), conjunctivitis (21% of cases), eczematous lesions, and lichenification have been reported.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Histology may reveal nonspecific findings that are occasionally similar to the histology of polymorphic light eruptions: a superficial and deep perivascular T lymphocyte infiltrate with presence of eosinophils under a dermis with few abnormalities. Chronic lesions may reveal acanthosis and excoriation in the epidermis, fibrosis, and a larger infiltrate in the dermis. Labial lesions may present infiltrates with follicular hyperplasia as a reactive phenomenon.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Association with B cell lymphoma occurred in only 2 patients.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> In our patient, doubt remained regarding whether the nodular lesions corresponded to lymphoma or to florid follicular lymphoid hyperplasia.</p><p id="par0060" class="elsevierStylePara elsevierViewall">The differential diagnosis of PA includes bites and stings, scabies, porphyria, adult atopic dermatitis, and other photodermatoses, which have different courses and histology and should be ruled out to reach diagnosis of this entity.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Different therapeutic approaches to AP have been reported, including topical treatments (photoprotection, corticosteroids, calcinuria inhibitors) and phototherapy (PUVA or narrowband UV-B). Thalidomide, cyclosporin, azathioprine and pentoxifylline have been used in severe cases, with varying results.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">In conclusion, AP is a rare entity in our setting but it cannot be ruled out. Lymphoid hyperplasia may develop during the course of the disease and this should be remembered in order to avoid an erroneous diagnosis that may condition future treatment. Diagnosis of this entity is based on clinical presentation and patient history, and the presence of HLA-DR4 is a finding that supports the suspected diagnosis.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">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:9 [ 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" => "Other Tests" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Diagnosis" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Treatment and Course" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Comment" ] 7 => array:2 [ "identificador" => "sec0040" "titulo" => "Conflicts of Interest" ] 8 => 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: Schneller-Pavelescu L, Caso EV-d, Blanes MM. Erupción papulosa fotodistribuida. Actas Dermosifiliogr. 2019;110:309–310.</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" => 783 "Ancho" => 1667 "Tamanyo" => 124966 ] ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1191 "Ancho" => 1667 "Tamanyo" => 468440 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Hematoxylin–eosin ×4. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 15 | 7 | 22 |
| 2024 Octubre | 151 | 54 | 205 |
| 2024 Septiembre | 206 | 28 | 234 |
| 2024 Agosto | 212 | 57 | 269 |
| 2024 Julio | 183 | 34 | 217 |
| 2024 Junio | 161 | 33 | 194 |
| 2024 Mayo | 170 | 40 | 210 |
| 2024 Abril | 122 | 15 | 137 |
| 2024 Marzo | 104 | 34 | 138 |
| 2024 Febrero | 92 | 35 | 127 |
| 2024 Enero | 86 | 37 | 123 |
| 2023 Diciembre | 93 | 18 | 111 |
| 2023 Noviembre | 128 | 40 | 168 |
| 2023 Octubre | 105 | 19 | 124 |
| 2023 Septiembre | 110 | 35 | 145 |
| 2023 Agosto | 105 | 17 | 122 |
| 2023 Julio | 114 | 30 | 144 |
| 2023 Junio | 97 | 23 | 120 |
| 2023 Mayo | 159 | 23 | 182 |
| 2023 Abril | 136 | 19 | 155 |
| 2023 Marzo | 118 | 30 | 148 |
| 2023 Febrero | 133 | 26 | 159 |
| 2023 Enero | 116 | 30 | 146 |
| 2022 Diciembre | 82 | 34 | 116 |
| 2022 Noviembre | 29 | 24 | 53 |
| 2022 Octubre | 33 | 23 | 56 |
| 2022 Septiembre | 44 | 38 | 82 |
| 2022 Agosto | 59 | 42 | 101 |
| 2022 Julio | 59 | 33 | 92 |
| 2022 Junio | 41 | 33 | 74 |
| 2022 Mayo | 95 | 42 | 137 |
| 2022 Abril | 101 | 32 | 133 |
| 2022 Marzo | 71 | 42 | 113 |
| 2022 Febrero | 63 | 23 | 86 |
| 2022 Enero | 60 | 36 | 96 |
| 2021 Diciembre | 63 | 31 | 94 |
| 2021 Noviembre | 71 | 45 | 116 |
| 2021 Octubre | 130 | 60 | 190 |
| 2021 Septiembre | 55 | 32 | 87 |
| 2021 Agosto | 41 | 29 | 70 |
| 2021 Julio | 34 | 25 | 59 |
| 2021 Junio | 40 | 21 | 61 |
| 2021 Mayo | 31 | 42 | 73 |
| 2021 Abril | 74 | 47 | 121 |
| 2021 Marzo | 55 | 17 | 72 |
| 2021 Febrero | 31 | 19 | 50 |
| 2021 Enero | 21 | 11 | 32 |
| 2020 Diciembre | 23 | 12 | 35 |
| 2020 Noviembre | 28 | 24 | 52 |
| 2020 Octubre | 21 | 18 | 39 |
| 2020 Septiembre | 30 | 9 | 39 |
| 2020 Agosto | 22 | 13 | 35 |
| 2020 Julio | 21 | 15 | 36 |
| 2020 Junio | 25 | 24 | 49 |
| 2020 Mayo | 18 | 15 | 33 |
| 2020 Abril | 10 | 14 | 24 |
| 2020 Marzo | 13 | 7 | 20 |
| 2020 Febrero | 2 | 0 | 2 |
| 2020 Enero | 1 | 0 | 1 |
| 2019 Mayo | 7 | 0 | 7 |
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