Management of eosinophil-associated inflammatory diseases: the importance of a multidisciplinary approach.

Elevated eosinophil counts in blood and tissue are a feature of many pathological processes. Eosinophils can migrate and accumulate in a wide variety of tissues and, by infiltrating a target organ, can mediate the development of several inflammatory diseases. The normalization of eosinophilia is a common biomarker of a treatable trait and can also be used as a prognostic and predictive biomarker since it implies a reduction in type 2 inflammation that contributes to disease pathogenesis. Biological therapies targeting this cell type and its proinflammatory mediators have been shown to be effective in the management of a number of eosinophilic diseases, and for this reason they constitute a potential common strategy in the treatment of patients with various multimorbidities that present with type 2 inflammation. Various biological options are available that could be used to simultaneously treat multiple target organs with a single drug, bearing in mind the need to offer personalized treatments under the umbrella of precision medicine in all patients with eosinophil-associated diseases (EADs). In addition to reviewing these issues, we also discuss a series of perspectives addressing the management of EAD patients from a multidisciplinary approach, with the collaboration of health professionals from different specialties who manage the different multimorbidities that frequently occur in these patients. We examine the basic principles of care that this multidisciplinary approach must cover and present a multidisciplinary expert opinion regarding the ideal management of patients with EADs, from diagnosis to therapeutic approach and follow-up.

Generalized morphea following the COVID vaccine: A series of two patients and a bibliographic review.

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 cross-reactivity 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.

Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases.

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.

Diffuse dermal mucinosis secondary to colony-stimulating factor 1 receptor monoclonal antibody treatment: A novel and peculiar drug-induced diffuse cutaneous mucinosis.

Colony-stimulating factor 1 receptor (CSF1R) inhibitors represent a new class of immune-modulatory drugs, mostly investigated in clinical trials in different malignant neoplasms. Four patients, diagnosed with recurrent or advanced malignant neoplasm and treated with a combination of anti-programmed death ligand 1 and anti-CSF1R monoclonal antibodies, developed an asymptomatic cutaneous eruption characterized by an ill-defined pseudoedematous to waxy diffuse infiltration with a reticular cobblestone-like pattern. Histopathological examination revealed diffuse mucin deposition involving the superficial and mid-dermis with fragmented and scattered elastic fibers. The exact pathogenic mechanisms implicated in the development of mucin deposits in patients treated with CSF1R inhibitors remain to be elucidated. A reduced degradation and clearance of components of the extracellular matrix by macrophages secondary to CSF1 pathway inhibition may be hypothesized. Shredding and fragmentation of elastic fibers may be a result of the increased accumulation of mucopolysaccharides. This observation illustrates the new spectrum of skin-related toxicities secondary to new targeting therapies. This may contribute to a better understanding of the underlying pathogenic mechanisms in skin diseases characterized by a persistent dermal glycosaminoglycan deposition.

Primoinfección por parvovirus B19: análisis de 46 pacientes / Acute parvovirus B19 infection: analysis of 46 patients

INTRODUCCIÓN: La infección por parvovirus B19 (PVB19) tiene una incidencia elevada y distribución mundial. Su espectro clínico es amplio, destacando las manifestaciones cutáneas, articulares y hematológicas. El objetivo del presente estudio fue estudiar epidemiología y manifestaciones clínico-analíticas de la primoinfección por PVB19. Pacientes y método: Estudio retrospectivo (10 años) de pacientes con serología IgM positiva para PVB19. Se incluyeron 46 pacientes y se estudiaron sus características demográficas, clínicas y analíticas. RESULTADOS: La primoinfección fue más prevalente en mujeres (ratio 2,2:1), y en edad media de 41años. La afectación articular fue la más frecuente (65%). En más de la mitad de los pacientes (24 casos) se observaron alteraciones cutáneas: exantema (28%), megaloeritema (9%), afectación «en guantes y calcetines» (6,5%), afectación periflexural (4%) y edema (4%). De entre las alteraciones hematológicas destacó la anemia (35%). El cuadro clínico se autolimitó en 1-2 semanas en la mayoría de los pacientes. CONCLUSIONES: A pesar de que existe un espectro clínico variable, las poliartralgias y el exantema maculopapular generalizado junto con fiebre y anemia son las manifestaciones típicas y más frecuentes de la primoinfección por PVB19 y suelen autolimitarse

INTRODUCTION: Parvovirus B19 (PVB19) infection has a high incidence and worldwide distribution. It has a broad clinical spectrum, with skin, joint and haematological manifestations being the most common. The objective of this study was to determine the epidemiology and clinical-analytical manifestations of acute PVB19 infection. PATIENTS AND METHODS: A retrospective study of patients with a positive IgM serology for PVB19 (10 years). Forty-six patients were included and their demographic, clinical and analytical characteristics were analyzed. RESULTS: Primary infection was most prevalent in women (ratio 2.2:1) aged 41 (mean age). Joint involvement was the most common manifestation (65%). Skin abnormalities were observed in more than half of patients (24 cases): rash (28%), megalerythema (9%), "gloves and socks" involvement (6.5%), periflexural rash (4%) and oedema (4%). Anaemia was the main haematological alteration (35%). The symptoms were self-limiting and resolved in 1-2 weeks in most patients. CONCLUSIONS: Although there is a variable clinical spectrum, polyarthralgias and generalized maculopapular rash with fever and anaemia are the typical and most frequent manifestations of primary infection by PVB19 and are usually self-limiting

A case of unilateral linear capillaritis.

Unilateral linear capillaritis is a rare variant of the pigmented purpuric dermatoses that can be misdiagnosed due to confusion with other cutaneous diseases that follow a linear distribution. We present the case of an 8-year-old boy with hyperpigmented patches linearly distributed on the right arm, initially diagnosed with segmental neurofibromatosis.

Acute parvovirus B19 infection: analysis of 46 patients. / Primoinfección por parvovirus B19: análisis de 46 pacientes.

INTRODUCTION: Parvovirus B19 (PVB19) infection has a high incidence and worldwide distribution. It has a broad clinical spectrum, with skin, joint and haematological manifestations being the most common. The objective of this study was to determine the epidemiology and clinical-analytical manifestations of acute PVB19 infection. PATIENTS AND METHODS: A retrospective study of patients with a positive IgM serology for PVB19 (10 years). Forty-six patients were included and their demographic, clinical and analytical characteristics were analyzed. RESULTS: Primary infection was most prevalent in women (ratio 2.2:1) aged 41 (mean age). Joint involvement was the most common manifestation (65%). Skin abnormalities were observed in more than half of patients (24 cases): rash (28%), megalerythema (9%), "gloves and socks" involvement (6.5%), periflexural rash (4%) and oedema (4%). Anaemia was the main haematological alteration (35%). The symptoms were self-limiting and resolved in 1-2 weeks in most patients. CONCLUSIONS: Although there is a variable clinical spectrum, polyarthralgias and generalized maculopapular rash with fever and anaemia are the typical and most frequent manifestations of primary infection by PVB19 and are usually self-limiting.