España, A. (Agustín)
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- Familial primary cutaneous amyloidosis: Caspase activation may be involved in amyloid formation(Wiley, 2022) España, A. (Agustín); Alkorta-Aranburu, G. (Gorka); Pelacho-Samper, B. (Beatriz); Antoñanzas-Perez, J. (Javier); Echeveste, J.I. (José I.)Primary localized cutaneous amyloidosis (PLCA) is a rare form of cutaneous amyloidosis, characterized by the presence of flat-topped papules and macules with amyloid deposits in the superficial dermis. It is a purely cutaneous disease with no association with systemic forms of amyloidosis.1 Although most cases are sporadic, familial cases (FPLCA) represent about 10% of total reports and show an autosomal dominant inheritance, with mutations described in the genes for the oncostatin M receptor (OSMR) and the interleukin-31 receptor A (IL31RA).2 Herein, we present a family affected by FPLCA and underline the role of caspase activation in amyloid formation.
- Generalized morphea following the COVID vaccine: A series of two patients and a bibliographic review(Wiley, 2022) Estenaga, Á. (Ángela); Morelló-Vicente, A. (Ana); España, A. (Agustín); Rodríguez-Garijo, N. (Nuria); Antoñanzas-Perez, J. (Javier); Aguado, L. (Leyre)The appearance of morphea after vaccination has been reported to date as single and deep lesions that appear exactly at the site of the skin puncture. It was therefore postulated that the origin could be the trauma related to the injection. The aim of this article is to review the various hypotheses offered in the published literature about generalized morphea following vaccination. We present two cases of generalized morphea after COVID-19 vaccination and review the published literature on immune-related cutaneous reactions. As previously reported, antigenic crossreactivity between vaccine spike proteins and human tissues could cause certain immune-mediated diseases, including generalized morphea. Herein we report two cases of generalized morphea probably induced by the COVID-19 vaccine, given the temporal relationship with its administration. In summary, environmental factors such as vaccination against SARS-COV-2 could induce an immune system dysregulation, which would have an important role in the pathogenesis of morphea. We present two cases of generalized morphea probably induced by the COVID-19 vaccine, given the time elapsed between vaccination and the onset of the skin lesions.
- Rituximab treatment of pemphigus foliaceus: A retrospective study of 12 patients(Elsevier BV, 2018) Palacios-Alvarez, I. (Irene); Riquelme, C. (Constanza); España, A. (Agustín); Garcia-Diez, I. (Irene); Iranzo, P. (Pilar); Curto-Barredo, L. (Laia)Pemphigus foliaceus (PF) is a chronic autoimmune blistering disease caused by pathogenic serum autoantibodies against desmoglein 1. Initial treatments for PF include systemic corticosteroids, immunosuppressants, and dapsone.1 Rituximab, a chimeric monoclonal antibody specific to the CD20 molecule on B cells, was shown to be effective for severe and refractory cases of pemphigus in a meta-analysis.2 The researchers also tried to analyze the efficacy of this treatment for PF, but the results were based on heterogeneous case series and reports. Our aim was to evaluate the clinical response to rituximab in a series of patients with PF. This study has been approved by our Institutional Review Board.
- Refractory facial Darier's disease treated with daylight photodynamic therapy(Wiley, 2022) Estenaga, Á. (Ángela); Morelló-Vicente, A. (Ana); España, A. (Agustín); Salido-Vallejo, R. (Rafael); Antoñanzas-Perez, J. (Javier); Oteiza-Rius, I. (Inés)Darier’s disease (DD) is an infrequent autosomal dominant skin disorder caused by a mutation of the ATP2A2 gene on chromosome 12 [1]. Mutations in this gene result in abnorma-lities in keratinocyte cell-cell adhesion producing an alterati-on of the keratinization of the skin, which clinically presents with dyskeratotic papules mostly affecting seborrheic and in-tertriginous areas. Palmoplantar and nail involvement is often present [1–3]. As a wide range of treatments have been propo-sed for this skin disorder with different results, the manage-ment of this disease is still a challenge for the dermatologist.
- Systemic lupus erythematosus-associated anetoderma and anti-phospholipid antibodies(Wiley-Blackwell, 2003) Lloret, P. (Pedro); España, A. (Agustín); Fernadez-Galar, M. (Marta)Anetoderma is characterized by a loss of normal elastic tissue that presents clinically as localized areas of wrinkled or flaccid skin. We describe the case of a 30-year-old woman with systemic lupus erythematosus-associated anetoderma and positive anti-phospholipid antibodies. We discuss the possible role of these antibodies in the pathogenesis of anetoderma, and, when detected, the need to check for an associated anti-phospholipid syndrome in such patients.
- Acneiform lesions secondary to ZD1839, an inhibitor of the epidermal growth factor receptor(Wiley-Blackwell, 2003) España, A. (Agustín); Lopez-Picazo, J.M. (José M.); Fernadez-Galar, M. (Marta)Drugs that inhibit the epidermal growth factor receptor, such as ZD1839 or C225, are being used increasingly in the treatment of solid tumours. This has led to the appearance of new secondary effects. We describe the case of a patient who presented with an acneiform eruption secondary to the administration of ZD1839. These lesions healed in a few days after stopping the drug
- Cutaneous polyarteritis nodosa(Wiley-Blackwell, 2002) España, A. (Agustín); Idoate, M.A. (Miguel Ángel); Bauza, A. (Ana)Classic polyarteritis nodosa (PAN) is a segmentary leucocytoclastic vasculitis that affects small- and medium-sized arteries. In 1931, Lindberg (Acta Med Scand 1931; 76: 183-225) described the existence of a cutaneous variant of PAN, without visceral involvement and with a more favourable prognosis. We present four patients diagnosed with cutaneous PAN in our hospital between 1987 and 1998. The study group was composed of three women and one child, whose ages ranged from 11 to 70 years old. The follow-up period was between 2 and 13 years. Each patient was submitted for an initial clinical, histological and laboratory evaluation and subsequent follow-up. The presence of nodules was the most frequent cutaneous lesion, preferentially located in the lower limbs. The erythrocyte sedimentation rate was the only parameter that was altered in all patients. Cutaneous biopsies from all patients showed a segmentary leucocytoclastic vasculitis in the arteries of the deep dermis and/or hypodermis. Direct immunofluorescence was positive in just one patient. No visceral involvement was found in any patient. There is confusion about the correct definition of cutaneous PAN. Some clinical findings, such as nodules or livedo reticularis, typical of cutaneous PAN suggest a good prognosis; however, we consider that it is necessary to evaluate these patients for systemic involvement for the possibility of arteritis in other organs as the term polyarteritis suggests.
- Extensive mucinous eccrine naevus following the lines of Blaschko: a new type of eccrine naevus(Wiley-Blackwell, 2006) España, A. (Agustín); Idoate, M.A. (Miguel Ángel); Marquina, M. (Miren)
- Erythema gyratumrepens-like eruption in a patient with epidermolysisbullosaacquisita associated with ulcerative colitis(Wiley-Blackwell, 2007) Sitaru, C. (C.); España, A. (Agustín); Pretel, M. (Maider); Aguado, L. (Leyre); Jimenez, J. (J.)
- Disseminated epidermolytic acanthoma probably related to trauma(Wiley-Blackwell, 1999) Sanchez-Carpintero-Abad, I. (Ignacio); España, A. (Agustín); Idoate, M.A. (Miguel Ángel)Epidermolytic acanthoma is a rare benign tumour, which may occur in both isolated and disseminated forms. Only seven cases of disseminated epidermolytic acanthoma (DEA) have been described. This entity should be distinguished from other hereditary or acquired conditions which involve epidermolytic hyperkeratosis and other benign acanthomas. On the basis of the clinical history and the histological findings, we diagnosed a case of DEA which was probably secondary to repeated trauma.