Iranzo, P. (Pilar)
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- 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.
- Anti-desmocollin autoantibodies in autoimmune blistering diseases(Frontiers, 2021) Ivars-Lleó, M. (Marta); España, A. (Agustín); Mascaró-Galy, J.M. (José Manuel); Bosch-Amate, X. (Xavier); Iranzo, P. (Pilar)The presence of anti-desmocollin (Dsc) antibodies is rarely described in autoimmune blistering diseases patients. Moreover, several clinical phenotypes of pemphigus may be associated with these antibodies. In this review we analyze clinicopathological, immunologic and outcome features of anti-Dsc autoimmune blistering diseases patients, to improve their diagnosis and management. We conducted a systematic search of PubMed and Embase (1990-present) for studies reporting cases of autoimmune blistering diseases with anti-Dsc antibodies. We classified the selected patients as patients with exclusively anti-Dsc autoantibodies, and patients with anti-Dsc and other autoantibodies. Of 93 cases with anti-Dsc autoantibodies included, 38 (41%) had exclusively these antibodies. Only 18% of patients presented with the typical clinicopathological phenotype of pemphigus vulgaris or pemphigus foliaceous. Mucosal involvement was seen in approximately half of the patients. Up to 18% of cases were associated with neoplasms. Acantholysis was described in 54% of cases with histopathological information. Treatments and outcomes vary in the different clinical phenotypes. The presence of anti-Dsc antibodies must be suspected mainly in those patients with either atypical pemphigus, in special with clinical pustules, or in cases showing intraepithelial or dermal neutrophilic/eosinophilic infiltrate on histological examination and dual pattern by direct immunofluorescence examination.
- Reactive granulomatous dermatitis as a histological pattern including manifestations of interstitial granulomatous dermatitis and palisaded neutrophilic and granulomatous dermatitis: a study of 52 patients(Wiley, 2020) Paricio, J.J. (J. J.); España, A. (Agustín); Mascaró-Galy, J.M. (José Manuel); Bielsa, I. (I.); Iranzo, P. (Pilar); Quer, A. (A.); Rodríguez-Garijo, N. (Nuria); Idoate, M.A. (Miguel Ángel)Background: Confusion exists regarding interstitial granulomatous dermatitis (IGD) and palisaded neutrophilic and granulomatous dermatitis (PNGD). Objective: To determine whether IGD and PNGD are two different entities, or whether they must be considered as two subtypes of the same reactive pattern, and thus whether the unification of the nomenclature is necessary. Methods: Observational retrospective multicentre study of patients with IGD and PNGD evaluated between 1999 and 2019 and review of their clinical and histological features. Results: We identified 52 patients (38 women and 14 men). Clinical and histological findings of IGD were observed in 88.4% of cases. The most common cutaneous lesions were plaques/macules (IGD) or annular plaques and papules/nodules (PNGD), located mostly on the limbs and trunk. The rope sign was developed in two patients with IGD that associated autoimmune disorders. Similar associated comorbidities (75%) were found in both entities, mainly autoimmune diseases (53.8%). In IGD, the infiltrate was predominantly lympho-histiocytic. Neutrophilic infiltrates, karyorrhexis and skin lesions with limited clinical course were mainly associated with PNGD biopsies. In biopsies with a limited recurrent course, a predominant lymphocytic inflammatory infiltrate was found. Collagen degeneration was present in 75.9% of cases. The floating sign was observed only in IGD type patients (63%). Overlapping histological findings were found in one fourth of cases, especially between IGD and interstitial granuloma annulare. Interface dermatitis, apparently unrelated to drug intake, was observed in 4 cases of IGD. Conclusion: We support the term reactive granulomatous dermatitis to unify both the clinical and histological findings of IGD and PNGD, and the overlapping between IGD and interstitial granuloma annulare. According to this, a spectrum of histological changes will be found depending on the clinical course of the skin lesions.