Predictors of Tumor Response to Cetuximab and Panitumumab in 116 Patients and a Review of Approaches to Managing Skin Toxicity.

INTRODUCTION AND OBJECTIVES: Cetuximab and panitumumab are monoclonal antibodies that target the epidermal growth factor receptor (EGFR) in the treatment of metastatic colorectal cancer. Most patients develop a papulopustular rash, which may predict tumor response. We studied whether the other adverse cutaneous effects associated with these monoclonal antibodies are also clinical predictors of response. We also reviewed publications describing approaches to treating the papulopustular rash since no evidence-based guidelines have yet been published. MATERIAL AND METHODS: We performed a retrospective study of 116 patients with metastatic colorectal cancer receiving anti-EGRF therapy with cetuximab or panitumumab at Hospital Universitario Donostia. RESULTS: In total, 81.9% of the patients developed a papulopustular rash. Patients who received the most cycles of treatment with the EGFR inhibitor were at the highest risk of developing the rash, and these patients also had the most severe rash reactions (P=.03). All of the patients who exhibited a complete tumor response had the rash, and the incidence of rash was lower in patients with poor tumor response (P=.03). We also observed an association between tumor response and xerosis (53.4% of the patients who developed xerosis also exhibited tumor response, P=.002). The papulopustular rash was managed according to an algorithm developed by our department. CONCLUSIONS: Severe papulopustular rash and xerosis may be clinical predictors of good response to anti-EGFR therapy. Patients who develop a papulopustular rash should be treated promptly because suboptimal treatment of this and other adverse effects can lead to delays in taking the prescribed anti-EGFR dose or to interruption of therapy.

A descriptive study of transthyretin amyloidosis in a tertiary hospital without a referral unit.

BACKGROUND AND OBJECTIVE: Transthyretin amyloidosis (ATTR) is a rare disease that is part of systemic amyloidosis and is life-threatening. It can affect all organs and systems, the most frequent being neurological and cardiac involvement. This study aims to detect possible ATTR cases and carry out a descriptive study of them. MATERIAL AND METHODS: Descriptive single-centre study carried out in a tertiary hospital, which included patients with suspected ATTR between September 2016 and January 2020. RESULTS: A total of 190 suspected ATTR patients were detected. The study includes 100 of these patients, as well as 10 relatives of patients in whom ATTR was detected in its genetic variant (ATTRv). In total, ATTRv was detected in 7 individuals (3 with a presymptomatic mutation of the disease), 16 patients with age-related ATTR and 31 individuals with unknown cardiac amyloidosis with the tests performed, which confirms the presence of this disease in non-endemic areas. CONCLUSIONS: ATTR is a disease that must be taken into account in the differential diagnosis of patients with heart failure with preserved LVEF, especially if associated with neurological symptoms.

Atypical presentation of transthyretin amyloidosis in a non-endemic area. / Presentación atípica de amiloidosis por transtiretina en un área no endémica.

BACKGROUND AND OBJECTIVE: There are 2 types of amyloidosis caused by transthyretin deposits: the wild type (wt-ATTR) and the mutant type (m-ATTR), transmitted by autosomal dominant inheritance with variable penetrance, manifesting with neurological and/or cardiac symptoms. We report on 3 families affected by m-ATTR diagnosed in a nonendemic area. MATERIAL AND METHODS: We studied 63 patients with a high suspicion of ATTR. The diagnosis was subsequently performed by magnification through polymerase chain reaction of DNA. For the positive cases, we studied the first-degree relatives. RESULTS: We detected 7 positive cases of m-ATTR, distributed among 3 families (Glu74Gln, Val142Ile in heterozygosity and Val142Ile in homozygosity), and 3 cases of nonpathogenic variants. CONCLUSIONS: Hereditary ATTR is a rare disease but is present in nonendemic areas and should therefore be considered in the differential diagnosis of patients with polyneuropathy and/or heart failure with preserved ejection fraction.

[Lipoid pneumonia related to an accidental aspiration of gas-oil]. / Neumonía lipoidea en relación con una aspiración accidental de gasóleo.

A 35-year-old non smoker man with no known history of chronic pulmonary disease, was treated at our hospital after accidental aspiration of gas-oil. He had developed an acute lipoid pneumonia in a few hours. Computed tomography of the chest showed a ground-glass pattern in middle lobe; given the immediate epidemiological precedent, it was possible to confirm a definitive diagnosis. One year later the patient is asymptomatic although small signs of acute process remain in the computed tomography.

[Relationship between objective data and health-related quality of life in COPD patients]. / Relación entre datos objetivos y calidad de vida percibida por el paciente con EPOC.

INTRODUCTION: In chronic diseases take special importance their influence in patient s daily life, in other words, on its quality of life. The Chronic Obstructive Pulmonary Disease (COPD) is one of the most important chronic diseases in our country, as much by its prevalence as by its economic impact. For the assessment of the life quality in these patients, we have specific questionnaires like the SGRQ, which can help us to better know the disease and its influence on patient s life. In our work the relationship of SGRQ with age, tobacco consumption, gasometrical data and severity of the disease are studied. METHODS: 51 patients with COPD and without significant comorbidities were selected and the SGRQ was administered to them by a trained interviewer; for every patient we also recovered the gasometrical values, the spirometric data, and their tobacco consumption background. RESULTS: The average score in each one of SGRQ scales were: global scale 40.49 +/- 18.98, symptoms scale 34.76 +/- 16.31, activity scale 56.68 +/- 23.72 and in the impact scale 32.86 +/- 19.96.We have found weak but significant correlations between the SGRQ results and the patient s age and the FEV1, but not with the gasometrical data or with the accumulated tobacco consumption. CONCLUSION: We can conclude that, in our work, the parameters that better correlates objective data with health-related quality of life of COPD patients are the age and the severity of the obstruction.

[Bullous pemphigoid related to PUVA therapy: two further cases]. / Penfigoide ampolloso relacionado con tratamiento con PUVA: aportación de dos nuevos casos.

Bullous pemphigoid is an autoimmune disease that generally affects elderly people and is characterised by the development of subepidermal blistering. Although bullous pemphigoid is potentially photosensitive, its occurrence during the treatment course with PUVA, especially in patients with psoriasis, has exceptionally been described. The association of bullous pemphigoid and psoriasis gives rise to difficulties when initiating treatment and we consider that the use of methotrexate, with or without associated corticoids, is a good alternative in the management of such patients. We report two further cases of bullous pemphigoid related to PUVA therapy in patients with psoriasis.

[Photocontacs dermatitis due to dexketoprofen. Report of 2 cases]. / Fotodermatitis de contacto por dexketoprofeno. Descripción de dos casos.

Dexketoprofen is the active isomer of ketoprofen and likewise belongs to the group of non-steroidal anti-inflammatory drugs (NSAIDs) derived from propionic acid. We have recently studied, using patch and photopatch tests, two women with a characteristic clinical picture of contact photodermatitis who had used topical dexketoprofen (Enangel) in the days before onset of the rash. In both cases we used the standard series of GEIDC, a series for NSAIDs, the product itself (Enalgel) and its excipients. On examination of the photopatches of both patients at 96 hours we found positive reactions to dexketoprofen and Enalgel. Furthermore, in one of the women we observed simultaneous photosensitivity to other NSAIDs and to several excipients of Enangel. Reviewing the literature we have found only three references on contact photodermatitis due to dexketoprofen. We describe two new cases, with multiple photosensitivities in one of them. We consider that such patients should be patch tested with dexketoprofen at 0.1-1 % petrolatum, concentrations that are notably inferior to those used in previous publications.

[Urolithiasis in the area III of Zaragoza: biochemistry and epidemiology]. / Litiasis renal en el área III de Zaragoza: bioquímica y epidemiología.

The urolithiasis is a very common disease that it causes, in addition to the suffering for the patients, an important cost sanitary partner. The composition of the renal stones that can influence in the treatment and later evaluation of the disease, varies in the different zones. In our work the composition of the renal stones received in the Biochemistry department of the H.C.U of Zaragoza studies during year 2002, with a brief approach to the epidemiology of the disease in the Area III of Zaragoza. The rate of incidence was of 0.35% with a clear predominance in men; the most frequent renal stones were those of pure calcium oxalate or calcium oxalate combined with apatite carbonate, although the percentage of samples gathered with respect to the total of diagnoses as soon as it surpassed 10%. Not found significant differences between the composition of the renal stones coming from the different zones from our Area, nor based on the sex of the patients. Given the influence of the composition of the urolithiasis for its treatment, we must insist on the collection of the greater number of samples for its analysis, although the epidemiologic data and the study of the urinary sediment can be used in the practice.

[Hypercoagulability state and endotelial injury in stable chronic obstructive pulmonary disease patients]. / Hipercoagulabilidad y daño endotelial en pacientes con enfermedad pulmonar obstructiva crónica en fase estable.

COPD is currently the fourth cause of death in our country; the main causal agent of the disease is tobacco whose effects on the bronchial tree are not limited to the lung parenchyma. The action of tobacco injures the endothelial wall, which could contribute to lung thrombosis, ischemic events or secondary pulmonary hypertension. Our work investigates the existence of a prothombotic state in these patients, characterized by the activation of clotting and endothelial injury, comparing the values of biochemical markers between COPD patients and healthy volunteers. Fifty-one patients with COPD and a group of 30 healthy volunteers of similar ages were selected and fibrinogen, D-dimer, factor VIII and von Willebrand factor (FvW:Ag and FvW:Rico) levels were compared. We found an increase of all markers in the patient group compared to the healthy control group. The differences found were statistically significant in all cases (p<0.05). COPD is characterized by a state of hypercoagulability and endothelial injury that they could contribute to the development of vascular complications such as lung thrombosis, ischemic events or secondary pulmonary hypertension.

Successful treatment of musk ketone-induced chronic actinic dermatitis with cyclosporine and PUVA.

We describe a patient with chronic actinic dermatitis whose photopatch tests revealed reactions to musk ketone and musk ambrette, both of which were found in his aftershave lotion. Minimal erythema doses of UVA and UVB were decreased. After initial unsuccessful treatment with PUVA therapy the patient was successfully treated with a combination of cyclosporine and PUVA.

Bazex's syndrome (acrokeratosis paraneoplastica)--first case report of association with a bladder carcinoma.

Bazex's acrokeratosis paraneoplastica is a clinically well-defined entity consisting of a distal erythemato-squamous eruption and nail dystrophy. It is always associated with neoplasia, mainly squamous carcinomas of the upper digestive and respiratory tracts; therefore, Bazek's acrokeratosis is considered to be a specific marker of internal malignancy. Two new cases of this syndrome are reported. The first was in a 41-year-old man with a squamous cell carcinoma of the oesophagus, the second in a 73-year-old man who presented with a transitional cell bladder carcinoma, an association not previously reported.