Scientific Letter
Recurrent Spontaneous Pneumothorax as a Manifestation of Birt-Hogg-Dube Syndrome
Neumotórax espontáneos de repetición como presentación del síndrome de Birt-Hogg-Dubé
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Fibla Alfara, Laureano Molins López-Rodó, Jorge Hernández Ferrández, Angela Guirao Montes" "autores" => array:4 [ 0 => array:4 [ "nombre" => "Juan J." "apellidos" => "Fibla Alfara" "email" => array:1 [ 0 => "juanjofibla@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" => "Laureano" "apellidos" => "Molins López-Rodó" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "Jorge" "apellidos" => "Hernández Ferrández" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "Angela" "apellidos" => "Guirao Montes" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Cirugía Torácica, Hospital Universitari Sagrat Cor, Barcelona, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Cirugía Torácica, Hospital Clínic, 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" => "Neumotórax espontáneos de repetición como presentación del síndrome de Birt-Hogg-Dubé" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1694 "Ancho" => 1583 "Tamanyo" => 259377 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Bilateral lung cysts.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Birt-Hogg-Dubé syndrome (BHDS) is a rare clinico-pathological entity, named after the 3 Canadian doctors who first described it in 1977.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">1</span></a> Prevalence is estimated at 1/200<span class="elsevierStyleHsp" style=""></span>000 births. The underlying cause is a mutation located on chromosome 17p11.2 of the FCLN gene that encodes folliculin.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">2</span></a> It is characterized clinically by the presence of skin lesions, lung cysts that may be associated with recurrent pneumothorax, and kidney tumors.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">3</span></a> We report the case of a patient who was diagnosed with BHDS after presenting multiple recurrent episodes of pneumothorax.</p><p id="par0010" class="elsevierStylePara elsevierViewall">This was a 35-year-old man, non-smoker, with a clinical history of arterial hypertension and bronchial asthma, who was admitted to the emergency department with a 1-week history of right scapular pain. Chest X-ray revealed complete right pneumothorax with no other lung abnormalities, so a chest tube was placed and progress was favorable. Thirty days later, he presented a new episode of complete right pneumothorax, so a video-assisted thoracoscopy was performed in the right side, revealing the presence of small apical bullae. These were resected, and mechanical pleural abrasion of the upper third of the hemithorax was performed. The patient progressed favorably and was discharged from hospital three days after the intervention. The histological study results were consistent with emphysematous bullae. Four years later, the patient presented in the emergency department again with left pleuritic pain, and a complete left pneumothorax was diagnosed. Chest CT was performed, revealing pneumothorax and multiple, large bilateral cystic cavities, predominantly in the lung bases (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Given the patient's history of previously treated contralateral pneumothorax, left video-assisted thoracoscopy was performed, with resection of the apex and mechanical pleural abrasion of the upper third of the hemithorax. Histological study of the resected pulmonary apex found emphysematous bullae, with no other changes. The patient progressed favorably and was discharged from hospital three days later. Two years later he was readmitted with recurrence of complete right pneumothorax, so video-assisted thoracoscopy was performed again, and chemical pleurodesis was applied with 8<span class="elsevierStyleHsp" style=""></span>g of talc. Given the recurrent, bilateral nature of the pneumothorax episodes and the CT image of bilateral cysts, a detailed clinical exploration was performed. Notably, discrete papular skin lesions of microcystic appearance were found on the patient's forehead. These were biopsied and determined on histology to be fibrofolliculomas. The recurrent bilateral pneumothoraxes, bilateral pulmonary cystic cavities, and fibrofolliculomas of the skin suggested the possibility of BHDS, so a study of peripheral blood for the FLCN gene was performed, as mutations in this gene are associated with the appearance of this syndrome. A change in exon 11 of the FLCN gene in position 1285, consistent with a cytosine deletion, was detected. This alteration involves the introduction of a premature stop codon, resulting in a truncated protein. Because this syndrome is associated with kidney tumors, an abdominal CT was performed, which was normal. The patient has had no recurrence of pneumothorax to date. A more comprehensive histological analysis of the lung tissue obtained during the first two surgical interventions was requested. The new report confirmed that the pulmonary bullae in both the right and apices were surrounded by normal alveolar walls, protruding into the interlobular septa. These histologic features have been described (along with the presence of intracystic septa and profusion of venules in the cystic space) as characteristic of BHDS.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">4</span></a></p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">BHDS is a rare autosomal dominant genodermatosis characterized mainly by cutaneous fibrofolliculomas and/or trichodiscomas, pulmonary cysts, spontaneous pneumothorax, and kidney tumors. The gene involved in this syndrome, FLCN, encodes folliculin, which is expressed mainly in the skin, kidneys, and lung.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">5</span></a> The main criteria for the diagnosis of BHDS are FLCN mutations on the genetic study, and the presence of skin lesions (fibrofolliculomas or trichodiscomas).<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">6</span></a> The most common extracutaneous manifestations are respiratory: up to 80% of BHDS patients have pulmonary cysts which can remain asymptomatic for years.<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">7</span></a> The number and size of the lesions varies from one patient to another, ranging from small cysts to bullae measuring several centimeters, located mainly in the lung bases and in the subpleural region. Larger cyst size and volume have been associated with a greater risk of developing pneumothorax. Approximately 20%–30% of patients with pulmonary cysts have a history of around two episodes of pneumothorax. Moreover, most patients with a history of pneumothorax have been reported to have multiple pulmonary cysts. The right lung is more often affected, although both lungs may be involved in up to 23% of cases.<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">8</span></a> A recent study found that 5%–10% of spontaneous primary pneumothoraxes may be related with BHDS.<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">9</span></a> The pathophysiology of the pulmonary cysts is unknown. The most current theory is the “stretch hypothesis”<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">10</span></a> which suggests that the cysts may originate from cell–cell adhesion defects generated by the mutation. Over time, repeated pulmonary expansion “stretches” the alveolar spaces, particularly in the regions of the lung with larger changes in alveolar volume. Chest CT is the examination of choice for the diagnosis of lung involvement.<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">11,12</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The prevalence of renal tumors in these patients varies from 6.5% to 34%, depending on the study,<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">7</span></a> with a predilection for men and the 20–55 age group.<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">13</span></a> Kidney lesions are usually bilateral and multifocal, with some specific histologic types, five of which are: hybrid forms of oncocytoma and chromophobe renal cell carcinoma (50%), and pure forms of chromophobe carcinoma (34%), oncocytoma (5%), clear cell (3%), or papillary (2%).<a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">7,14</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Although some authors have associated BHDS with colon cancer, no specific indication for colonoscopy has been described in these patients, and recommendations are the same as for the general population.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">15</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">In conclusion, a patient who presents with multiple recurrent pneumothoraxes who shows bilateral pulmonary cysts on CT should undergo a dermatological examination to detect accessible skin lesions for biopsy and genetic study. This diagnosis justifies a study of the abdomen, and patients should be monitored for the early detection and treatment of kidney tumors.</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: Fibla Alfara JJ, Molins López-Rodó L, Hernández Ferrández J, Guirao Montes A. Neumotórax espontáneos de repetición como presentación del síndrome de Birt-Hogg-Dubé. Arch Bronconeumol. 2018;54:396–397.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1694 "Ancho" => 1583 "Tamanyo" => 259377 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Bilateral lung cysts.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:15 [ 0 => array:3 [ "identificador" => "bib0080" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Hereditary multiple fibrofolliculomas with trichodiscomas and acrochordons" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "A.R. Birt" 1 => "G.R. Hogg" 2 => "W.J. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 7 | 3 | 10 |
| 2024 October | 39 | 15 | 54 |
| 2024 September | 49 | 12 | 61 |
| 2024 August | 65 | 39 | 104 |
| 2024 July | 57 | 23 | 80 |
| 2024 June | 66 | 31 | 97 |
| 2024 May | 116 | 26 | 142 |
| 2024 April | 54 | 33 | 87 |
| 2024 March | 40 | 23 | 63 |
| 2024 February | 34 | 23 | 57 |
| 2023 June | 1 | 0 | 1 |
| 2023 March | 15 | 6 | 21 |
| 2023 February | 46 | 30 | 76 |
| 2023 January | 24 | 47 | 71 |
| 2022 December | 54 | 39 | 93 |
| 2022 November | 54 | 21 | 75 |
| 2022 October | 72 | 49 | 121 |
| 2022 September | 46 | 40 | 86 |
| 2022 August | 45 | 71 | 116 |
| 2022 July | 39 | 71 | 110 |
| 2022 June | 35 | 60 | 95 |
| 2022 May | 39 | 45 | 84 |
| 2022 April | 37 | 27 | 64 |
| 2022 March | 35 | 42 | 77 |
| 2022 February | 45 | 39 | 84 |
| 2022 January | 59 | 48 | 107 |
| 2021 December | 47 | 46 | 93 |
| 2021 November | 40 | 44 | 84 |
| 2021 October | 83 | 54 | 137 |
| 2021 September | 54 | 58 | 112 |
| 2021 August | 61 | 37 | 98 |
| 2021 July | 35 | 41 | 76 |
| 2021 June | 45 | 31 | 76 |
| 2021 May | 51 | 32 | 83 |
| 2021 April | 84 | 98 | 182 |
| 2021 March | 69 | 16 | 85 |
| 2021 February | 56 | 38 | 94 |
| 2021 January | 41 | 40 | 81 |
| 2020 December | 46 | 22 | 68 |
| 2020 November | 46 | 22 | 68 |
| 2020 October | 58 | 33 | 91 |
| 2020 September | 55 | 11 | 66 |
| 2020 August | 32 | 11 | 43 |
| 2020 July | 33 | 24 | 57 |
| 2020 May | 1 | 1 | 2 |
| 2020 March | 27 | 9 | 36 |
| 2020 February | 44 | 12 | 56 |
| 2020 January | 66 | 22 | 88 |
| 2019 December | 75 | 18 | 93 |
| 2019 November | 70 | 15 | 85 |
| 2019 October | 42 | 8 | 50 |
| 2019 September | 52 | 6 | 58 |
| 2019 August | 59 | 18 | 77 |
| 2019 July | 95 | 29 | 124 |
| 2019 June | 43 | 8 | 51 |
| 2019 May | 48 | 11 | 59 |
| 2019 April | 47 | 39 | 86 |
| 2019 March | 47 | 13 | 60 |
| 2019 February | 35 | 15 | 50 |
| 2019 January | 27 | 14 | 41 |
| 2018 December | 1 | 2 | 3 |
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