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Sweet syndrome is a neutrophilic dermatosis associated with multiple disorders. This retrospective case-series study of patients with Sweet syndrome in a tertiary hospital in Spain from 2001 to 2021, explores clinicopathological characteristics of Sweet syndrome and variables associated with malignancy, presence of autoinflammatory disorders and differences between histological subtypes. A total of 93 patients were identified: 30% idiopathic, 34% malignancy-associated, 29% reactive to infections or drug-associated, and 6% with an autoimmune/inflammatory condition. Acute myeloid leukaemia was the most common malignancy (16/93) followed by myelodysplastic syndrome (7/93). Patients with acute myeloid leukaemia presented isolated flares, marked cytopaenia and rapid response to treatment, whereas myelodysplastic syndrome followed a chronic-recurrent course. The most frequent associated medications and inflammatory disorders were filgrastim and hydroxyurea (n=2); and inflammatory bowel disease (n=4). In addition, 3 patients were diagnosed with VEXAS syndrome. Male sex (p=0.006), fever (p=0.034), increased erythrocyte sedimentation rate (p<0.001), anaemia (p<0.001), and thrombocytopaenia (p<0.001) were associated with malignancy. Histologically, patients were classified as classic (60%), histiocytoid (22.5%) or subcutaneous (15%), with pain (p=0.011) and nodules (p<0.001) being associated with subcutaneous-Sweet syndrome. Sweet syndrome in the context of cytopaenia should alert the presence of malignancy. An acquired autoinflammatory condition should be explored in relapsing Sweet syndrome with myelodysplastic syndrome. A minimum follow-up of 6 months is recommended. [ABSTRACT FROM AUTHOR]

Facial discoid dermatosis (FDD) is a condition characterized by papules-plaques primarily affecting the face. FDD has been associated with psoriasis, seborrheic dermatitis, and pityriasis rubra pilaris. We conducted a retrospective multicenter study with 13 patients diagnosed with FDD in Spain (2014-2022). Patient demographics, clinical presentations, histopathological findings, and treatment outcomes were collected. FDD shows as erythematous-orange, nummular papules and/or plaques with dry scales that primarily affect the facial region. Histopathological analysis revealed a consistent pattern of psoriasiform hyperplasia with confluent parakeratosis, without neutrophils within the epidermal layers. This study provides essential insights into the clinical and histopathological features of FDD within a predominantly Caucasian patient cohort. The presence of distinct clinical features and consistent histological findings underscores the uniqueness of FDD. However, the variability in histological features and persistence of lesions despite various treatments highlight the need for further research to better understand the pathogenesis of FDD and develop effective therapies., (Copyright © 2025. Publicado por Elsevier España, S.L.U.)

Introduction: Localized bullous pemphigoid (LBP) is an infrequent bullous pemphigoid (BP) variant restricted to a body region. According to the most compelling evidence, LBP occurs in patients with pre-existent serum antibodies against the basement membrane zone, which occasionally acquire the capacity to induce disease after the influence of different local factors acting as triggers., Methods: We hereby present a multicenter cohort of 7 patients with LBP developed after local triggers: radiotherapy, thermal burns, surgery, rosacea, edema and a paretic leg. In addition, we conducted a review of the literature, and we propose a set of diagnostic criteria for LBP, also based on our case series and the 2022 BP guidelines from the European Academy of Dermatology and Venereology., Results: During follow-up, three of the patients from our series evolved to a generalized BP, with only one requiring hospitalization. Our literature search retrieved 47 articles including a total of 108 patients with LBP, with a 63% with a potential local precipitating factor previous to their diagnosis. LBP mostly affected older females, and a subsequent generalized progression occurred in 16.7% of the cases. The most frequently involved areas were the lower limbs. Radiation therapy and surgery were responsible for the inducement of nearly 2 in 3 cases of LBP. We observed a significantly higher risk of generalization in cases where the trigger led to the developing of LBP earlier (p=0.016). Our statistical analysis did not detect any other prognosis factor for generalization when assessing direct immunofluorescence, histological and serological results, or other patient related factors., Conclusion: LBP should be suspected in patients with recurrent localized bullous eruptions. The presence of a trauma history in the same anatomic area is reported in most cases., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Corbella-Bagot, Gil-Lianes, Fernández-Vela, Martí-Martí, Alegre-Fernández, Fuertes, Garbayo-Salmons, Bosch-Amate, Guilabert and Mascaró.)

Competing Interests: Competing interests: The authors have no conflicts of interest to disclose.

There has been no previous clinical-immunological study of pemphigus in Spain. The aim of this study was to perform a retrospective analysis of pemphigus patients who had been followed for a period of 18 years in our centre. We characterized the autoantibody response, compared diagnostic assays and correlated the immunobiological data with phenotype and prognosis. Clinical, epidemiological and immunopathological data were collected from 40 patients. Patients sera were characterized by indirect immunofluorescence and enzyme-linked immunosorbent assay (ELISA). Epidemiological and clinical findings were comparable to other series. Mortality during follow-up was 0% and 6% in pemphigus foliaceus and vulgaris, respectively. Importantly, higher indirect immunofluorescence titres and anti-desmoglein 3 ELISA values of samples from untreated patients correlated significantly with a potentially worse clinical course. Moreover, there was a positive correlation between indirect immunofluorescence titres and anti-desmoglein 3 ELISA levels in pemphigus vulgaris patients. Based on our findings, initial high anti-desmoglein 3 antibodies in pemphigus patients correlate with a more adverse prognosis, which raises the question as to whether a more aggressive initial therapy is indicated in patients with this immunological pattern.