Background. Acute urticaria is a common condition in the pediatric emergency department (ED) and no data is available in Portugal.Objective. We aimed to characterize the prevalence, etiology and management of acute urticaria in children presenting at an ED of a portuguese central hospital and report the followup investigation when drug or food allergy was suspected. Methods.Retrospective study of clinical records from children admitted to the ED with acute urticaria during one year period.Results. 250 children were included, mean age of 7.4 ± 4.9 years (0-17 years). The most frequently suspected etiological factors were infections (22%), foods (12%), insect bites (9%) and drugs (8%), of which, upper respiratory tract infections, seafood and β-lactam antibiotics were the most frequent. In 44% of cases, the etiology of urticaria was not determined. After ED discharge, of the 50 patients with suggestive drug or food allergy, only 48% were sent to allergological workup and the allergy confirmed in 6 of them (2.4% of the 250 children). Conclusion.These data suggest that allergy is not the main trigger of acute urticaria in ED children, but when suspected, reference to an allergy department to complete allergological workup was insufficient.M a n u s c r i p t a c c e p t e d f o r p u b l i c a t i o n 5) Highlights boxAcute urticaria in children can be caused by a wide variety of factors, such as infections, food or drug hypersensitivity, physical triggers, insect bites and idiopathic causes. There is a lack of childhood acute urticaria detailed information in Portugal, with no data available. In our study we characterize the prevalence, etiology and management of acute urticaria in children presenting at an emergency department of a portuguese central hospital and reported the follow-up investigation when drug or food allergy was suspected.This study supports the opinion that allergy is not the main trigger of acute urticaria in children, representing 2.4% of the children admitted to the ED with acute urticaria. Most importantly, we found that in 52% of patients with suspected drug or food allergy, reference to an allergy department to complete allergological work-up was not performed.It is important that physicians practising emergency medicine provide appropriate aftercare instructions to patients with suspected allergy and refer these patients for allergological evaluation, in order to provide a complete and careful diagnostic work-up that is essential for a correct diagnosis. In fact, underestimated allergy diagnosis could lead to an increased risk in truly allergic patients, and overestimated diagnosis of allergy could contribute to an overrated avoidance measures in non-allergic children.

Autosomal recessive hyper‐IgE syndrome is a primary immunodeficiency that results from a mutation in the DOCK8 gene. We report a case of a patient presenting with severe eczema, atopy, and recurrent skin infections since the first months of life. The diagnosis of autosomal recessive hyper‐IgE syndrome was made at the age of 7 by a positive DOCK8 genetic test. The patient underwent hematopoietic stem cell transplantation, with complete remission of the various manifestations.

<b><i>Background:</i></b> Pyoderma gangrenosum (PG) is a rare and difficult-to-diagnose disease that often associates with inflammatory bowel disease. <b><i>Case:</i></b> We present a case of a 57-year-old female with ulcerative colitis receiving 5-ASA who presented with rapidly progressive ulcers in the right foot and on the inside of the thigh, extending from the left large vaginal lip to the perianal area, compatible with PG. She was initially treated with corticosteroids with no response. After multidisciplinary consultation, it was decided to initiate in­fliximab 5 mg/kg, and to perform ileostomy for fecal diversion and negative-pressure wound therapy. The patient presented with marked improvement of the lesions, being discharged after 2 months and demonstrating almost complete resolution of the lesions within 4 months. <b><i>Conclusion:</i></b> Due to the rarity of PG, there is no evidence of the optimal management. The role of surgery is controversial as PG lesions can demonstrate pathergy and theoretically could worsen with surgical intervention. In this case it was decided based on the extent of the lesions and the experience in other septic/ulcerative perianal conditions.

Non-specific lipid transfer proteins (LTP) are panallergens present in plant-foods, being the most relevant allergens of Rosacea family fruits in the Mediterranean area (1,2). These ubiquitous proteins are highly conserved and widely distributed in the plant kingdom, sharing a moderate-to-high homologous molecular structure, which put the patients allergic to LTP at risk of developing allergic reactions after the ingestion of an array of botanically unrelated foods, including fruits, tree nuts, seeds, vegetables and cereals (1,3-7).Despite the fact that milk and egg proteins are the main causes of food allergy in childhood, fruits and vegetables have been recognized as emergent allergens in pediatrics (8).Since LTP may cause severe systemic reactions it is essential to better understand this allergy in childhood.Even though multiple studies on LTP have been published in the last years, so far, not much is known about LTP allergy in children, especially regarding daily practice, since only a few case series focused on pediatric ages have been published (9-13). A recent study has shown that fresh fruits are the 5th cause of anaphylaxis in children, with tree nuts being the 2 nd , both potentially caused by LTP in this region (14).Our aim was to characterize a series of children with allergy to LTP, in order to better understand its characteristics. M a n u s c r i p t a c c e p t e d f o r p u b l i c a t i o n MethodsWe performed a retrospective analysis of medical records from patients under 18 years-old with confirmed LTP allergy (2013)(2014)(2015)(2016)(2017)(2018)(2019).Diagnosis was established based on a convincing history of immediate allergic reactions to plant-foods (i.e. repeated symptoms to LTP containing foods on several occasions) supported by positive skin prick tests (SPT) (defined as the mean diameter of the wheal ≥ 3 mm than negative control ( 15)) to LTP extract (Roxall, Bilbao, Spain) and positive specific IgE (sIgE) to LTP allergens (Pru p 3, Cor a 8 and/or Jug r 3) determined by ImmunoCAP (ThermoFisher Scientific, Uppsala, Sweden; cut-off: ≥0.35 kUA/L) and/or ImmunoCAP TM ISAC microarray

We report the case of a pregnant woman with recessive dystrophic epidermolysis bullosa. During pregnancy, she presents with a large, rapidly growing, tumor on her right forearm, whose biopsy revealed an invasive squamous cell carcinoma. Amputation by the middle third of the forearm was performed at 21 weeks of pregnancy, without intra- or post-operative complications. The remainder of pregnancy was unremarkable and, at 36 weeks, she gave birth to a healthy baby. One month after delivery, a large lymph node conglomerate was detected in the right axilla, highly suggestive of metastatic disease and complete lymph node dissection was then performed. Despite the prompt institution of chemotherapy, the patient died a few months later due to metastatic disease.