Letter to the Editor
HIV infection and porphyria cutanea tarda, report of a case
Infección por el virus de la inmunodeficiencia humana y porfiria cutánea tarda, a propósito de un caso
Juan Carlos Gómez-Polo
, María Álvarez-Carretero, María Megía-Sánchez, María de los Ángeles Lozano Parras
Corresponding author
Servicio de Medicina Interna I, Hospital Clínico San Carlos, Madrid, Spain
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(B–D) CT scan of the chest, showing the presence of an interstitial infiltrate, like an image on frosted glass and bilateral septal thickening suggestive of pneumonia caused by an atypical microorganism.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Porphyria cutanea tarda (PCT) is the most common form of porphyria, due to hepatic uroporphyrinogen decarboxylase deficiency. It occurs clinically as cutaneous erosions in exposed areas, in patients with a certain genetic predisposition exposed to precipitating factors.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a> The role of HIV as possible precipitant has been widely discussed, given the usual coexistence in these patients of other comorbidities such as hepatotropic viral infections or alcoholism, with proven action triggering PCT. Because of the few reports of patients diagnosed with both diseases, and the existence of several pathophysiological hypotheses that attempt to explain a potential association,<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">2</span></a> a direct relationship not having been demonstrated yet, reporting this case is relevant. PubMed (descriptors: hiv, Associated, pct; 1985–2015).</p><p id="par0010" class="elsevierStylePara elsevierViewall">The patient is a 63-year-old man, with a history of chronic alcohol abuse, about 120<span class="elsevierStyleHsp" style=""></span>g alcohol/day and smoking 40 cigarettes, not following any routine treatment. He visits the emergency room for progressive dyspnea of 2 weeks of evolution, poorly productive cough, weight loss of about 10<span class="elsevierStyleHsp" style=""></span>kg in the last 2 months and intense asthenia, with no fever. Upon arrival he reports hemodynamic instability with hypotension (95/64<span class="elsevierStyleHsp" style=""></span>mmHg), and 126<span class="elsevierStyleHsp" style=""></span>lpm heart rate, fever 38.0<span class="elsevierStyleHsp" style=""></span>°C and tachypnea with 93% baseline oxygen saturation. On physical examination, extreme thinness is striking, with facial ocher hyperpigmentation and hypertrichosis for 6 months, oral candidiasis, and systemic hypoventilation under pulmonary auscultation, with no other abnormalities.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Blood analysis shows 6000<span class="elsevierStyleHsp" style=""></span>leuc/mcl (84% neutrophils and 700 lymphocytes, 12% of the formula), iron 38<span class="elsevierStyleHsp" style=""></span>μg/dl; ferritin 1928<span class="elsevierStyleHsp" style=""></span>ng/ml; IST 20%; LDH 893<span class="elsevierStyleHsp" style=""></span>UI/l; GOT 70<span class="elsevierStyleHsp" style=""></span>UI/l; GGT 148<span class="elsevierStyleHsp" style=""></span>UI/l; GPT 83<span class="elsevierStyleHsp" style=""></span>UI/l; FAL 174; as well as negative serology for hepatotropic virus and tumor markers. The baseline arterial blood gas value shows pH 7.45; pO<span class="elsevierStyleInf">2</span> 57.0; pCO<span class="elsevierStyleInf">2</span> 25.0; bicarbonate 17.4<span class="elsevierStyleHsp" style=""></span>mmol/l and lactate 4<span class="elsevierStyleHsp" style=""></span>mmol/l. The chest X-ray (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A) shows a slight increase of bronchoalveolar region with no infiltrates. On admission, chest CT is performed (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B–D), where a diffuse pattern is observed with thickened septa in both hemithorax, to rule out atypical pneumonia. Therefore, because of the clinical picture, HIV serology is requested, with a positive result, with selection of 28 CD4 T lymphocytes, suspecting <span class="elsevierStyleItalic">Pneumocystis jirovecii</span> and beginning treatment with trimethoprim/sulfamethoxazole and steroids, postponing the initiation of highly active antiretroviral therapy 4 days, with evident clinical improvement.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Furthermore, due to the presence of facial hyperpigmentation and hypertrichosis, plus laboratory findings, determination in urine is requested, resulting in: total porphyrin/excretion 423.3<span class="elsevierStyleHsp" style=""></span>μg/24<span class="elsevierStyleHsp" style=""></span>h (0–150); uroporphyrin 72<span class="elsevierStyleHsp" style=""></span>μg/24<span class="elsevierStyleHsp" style=""></span>h (0–25); heptacarboxylporphyrin 108<span class="elsevierStyleHsp" style=""></span>μg/24<span class="elsevierStyleHsp" style=""></span>h (0–5); coproporphyrin I 72<span class="elsevierStyleHsp" style=""></span>μg/24<span class="elsevierStyleHsp" style=""></span>h (0–25); coproporphyrin III 136.8<span class="elsevierStyleHsp" style=""></span>μg/24<span class="elsevierStyleHsp" style=""></span>h (0–75), consistent with PCT diagnosis.</p><p id="par0025" class="elsevierStylePara elsevierViewall">In this case report, it should be noted that the symptomatology, suggestive of porphyria, appears in the last 6 months. Therefore, we hypothesize that it might be the highest reduction in the number of CD4 T lymphocytes, and emergence of opportunistic infections, a possible PCT trigger, as proposed in other publications<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a> where it is indicated that endotoxins released by these agents might interact with cytochrome P450, and interfere with the synthesis of heme. Other hypotheses, which remain under discussion, are the existence of excess estrogen and impaired levels of cortisol affecting individuals with HIV<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">4–6</span></a>; as well as a chronic stimulation of the immune system that would modify porphyrin metabolism.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">7</span></a> This, coupled with the limited reported cases (only 2 cases diagnosed with both diseases in our center since 2008), and reported in the literature makes it necessary to continue research in this field, in the presence of evidence suggesting a potential association between the two entities, still unconfirmed.</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: Gómez-Polo JC, Álvarez-Carretero M, Megía-Sánchez M, de Lozano Parras MÁ. Infección por el virus de la inmunodeficiencia humana y porfiria cutánea tarda, a propósito de un caso. Med Clin (Barc). 2016;146:e49–e50.</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" => 1036 "Ancho" => 1750 "Tamanyo" => 299635 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Posteroanterior chest X-ray showing increased bilateral bronchoalveolar region with no clear infiltrates. (B–D) CT scan of the chest, showing the presence of an interstitial infiltrate, like an image on frosted glass and bilateral septal thickening suggestive of pneumonia caused by an atypical microorganism.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0040" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Porfiria cutánea tarda: su asociación con HIV. Presentación de 4 pacientes" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "P. Salazar" 1 => "E. Cesaroni" 2 => "M. Allevato" 3 => "M. 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