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Sahar Z Elazab, Waheed F Hessam
Chronic spontaneous urticaria (CSU) is a common distressing disease caused mainly by autoreactive mechanisms. Autologous serum therapy (AST) has been tried for treatment of many autoimmune diseases including chronic urticaria. We assessed the effect of AST in autologous serum skin test (ASST)-positive and ASST-negative CSU patients. The study included 40 patients clinically diagnosed as CSU. Twenty ASST-positive patients and 20 ASST-negative patients received AST for 8 weeks. Urticarial activity was assessed at baseline, 4th week, 9th week, and at the 12th week by the weekly urticarial activity score (UAS-7) according to the EAACI/GA2LEN/EDF/WAO guidelines...
June 2017: Egyptian Journal of Immunology
Valentina Caputo, Fiorella Santi, Antonio Cascio, Marcello Trizzino, Claudia Colomba
Trombiculiasis is a common but underreported ectoparasitosis characterized by an infestation of the skin by the larval stage of various species of mites belonging to the phylum Arthropoda, class Arachnida, subclass Acarina. Clinical manifestations consist of pruritic erythematous and urticarial macules and papules located on covered thin and glabrous skin. In recent studies Neotrombicula autumnalis larvae have been described as the possible vectors of pathogens such as Borrelia burgdorferi and Anaplasma phagocytophilum...
March 1, 2018: Le Infezioni in Medicina
N Yoshimoto, H Ujiie, M Zheng, H Iwata, H Kosumi, H Hata, H Shimizu
Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering skin disease, characterized by tense bullae and urticarial erythema with a generalized distribution. BP is induced by autoantibodies to structural proteins of the basement membrane zone (BMZ) such as BP180 and BP2301 . Because the majority of BP skin shows complement deposition2 , complement activation is considered to be important for blister formation. Among IgG subclasses, IgG1 have a high ability to activate complements, whereas IgG2 and IgG4 have a low and no ability, respectively...
March 6, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
F Rodrigues, V Hentgen, C Bachmeyer, I Kone-Paut, A Belot, G Grateau, G Sarrabay, S Georgin-Lavialle
The auto-inflammatory diseases linked to NLRC4 mutations are recently described entities. Transmission is autosomal dominant in 80 % of cases; cases of somatic mutation have already been reported. The disease may display two very different clinical phenotypes: the phenotype 1 (30 %), severe, is dominated by a multisystemic inflammation starting in the first year of life with symptoms of chronic inflammatory bowel disease (IBD), macrophagic actication syndrome (MAS), or even a presentation suggesting a cryopyrinopathy in its CINCA form; the mortality of this phenotype is high (25 %)...
February 26, 2018: La Revue de Médecine Interne
Massimo Radin, Luca Bertero, Dario Roccatello, Savino Sciascia
The hypereosinophilic syndrome is a rare disease characterized by the association between high absolute eosinophil count and eosinophil-mediated organ damage. We describe a case of a 70-year-old male with an absolute eosinophil count of 2130 cells/µL. Clinical symptoms and signs included the following: severe asthenia, axonal sensitive motor neuropathy, basal pleural effusion with signs of hypoventilation on chest radiography, and gastrointestinal symptoms such as severe diarrhea, weight loss (-10 kg in 6 months), abdominal pain, and vomiting...
January 2018: Journal of Investigative Medicine High Impact Case Reports
Ashley K Clark, Vivian Y Shi, Raja K Sivamani
The association between guttate psoriasis and infection with group A Streptococcus (GAS) has been well established in the medical literature. However, responses to treatments aimed at GAS eradication such as systemic antibiotics or tonsillectomy are inconsistent. Further complicating treatment recommendations for a disease with a suspected microbial trigger, the standard therapy for severe psoriasis is with systemic immunosuppressant medications. This case report illustrates the role of GAS as a trigger for acute onset severe psoriasis in a child whose skin disease initially worsened with a trial of methotrexate...
August 15, 2017: Dermatology Online Journal
Inês Raposo, Susana Machado, Rita Sampaio, Manuela Selores
Bullous pemphigoid (BP) is an immune mediated bullous disease that is manifested by urticarial plaques with superimposed subepidermal blisters and significant pruritus. It is generally found in the elderly, but is rare in the pediatric population. A 5-month-old girl previously diagnosed with hand-foot-mouth disease was examined in our dermatology department owing to vesicles and bullae, initially located to the hands and feet, which progressed with new lesions. Tense vesicles and bullae distributed in an annular string of pearls pattern on the abdomen and facial and cervical regions were noted...
July 15, 2017: Dermatology Online Journal
Zhe Hou, Brian R Hinds, Philip R Cohen
Neutrophilic urticarial dermatosis (NUD) is a useful diagnostic term for urticarial lesions that are less pruritic and more painful than conventional urticaria. The histopathologic features include neutrophilic infiltrates in the interstitial dermis with a higher density than idiopathic urticaria. NUD has been associated with several systemic conditions, which are predominantly autoimmune and autoinflammatory in nature. A woman with Crohn disease who developed NUD is described. Literature reports of other conditions in which neutrophilic urticarial dermatosis have been observed are also reviewed and summarized...
November 15, 2017: Dermatology Online Journal
Sohail Abdul Salim, Tauqeer Yousuf, Asha Patel, Tibor Fülöp, Mohit Agarwal
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease characterized by multiple organ system involvement, including renal disease, with low complement levels. We report the case of a 31-year-old woman who presented with nonspecific symptoms including fatigue, diarrhea, macular rash and abdominal pain with acute renal failure leading to end-stage kidney disease. Laboratory results showed hematuria, nephrotic range proteinuria, worsening creatinine and low C1q levels. Left kidney biopsy showed proliferative glomerulonephritis with crescent formation...
February 2018: American Journal of the Medical Sciences
Michael Schatz, Scott H Sicherer, Robert S Zeiger
An impressive number of clinically impactful studies and reviews were published in The Journal of Allergy and Clinical Immunology: In Practice in 2017. As a service to our readers, the editors provide this Year in Review article to highlight and contextualize the advances published over the past year. We include information from articles on asthma, allergic rhinitis, rhinosinusitis, immunotherapy, atopic dermatitis, contact dermatitis, food allergy, anaphylaxis, drug hypersensitivity, urticarial/angioedema, eosinophilic disorders, and immunodeficiency...
January 31, 2018: Journal of Allergy and Clinical Immunology in Practice
A Escudier, F-X Mauvais, P Bastard, C Boussard, A Jaoui, V Koskas, E Lecoq, A Michel, M-C Orcel, P-E Truelle, D Wohrer, M Piram
Auto-inflammatory diseases are characterized by unexplained and recurrent attacks of systemic inflammation often involving the skin, joints, or serosal membranes. They are due to a dysfunction or dysregulation of the innate immunity, which is the first line of defense against pathogens. Early recognition of these diseases by the clinician, especially by pediatricians encountering such pathologies in pediatric patients, is primordial to avoid complications. Skin manifestations, common in most auto-inflammatory diseases, are helpful for prompt diagnosis...
January 29, 2018: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
M Vieira, I Rosmaninho, I Lopes, A Guilherme, J P Moreira da Silva
Aquagenic urticaria (AU) is a rare form of chronic inducible urticaria elicited by water at any temperature. We describe the case of a 25-year-old atopic woman who presented to our unit with a 4-year history of recurrent urticarial rashes, highly pruritic, confined to the neck and lower part of the face, occurring solely on contact with sea water. The lesions were reproduced by challenge tests with aqueous 3.5% NaCl and other hypertonic aqueous solutions but not with 20% glucose neither tap water. Our case supports the existence of a distinct salt-dependent subtype of aquagenic urticaria (SDAU), which seems to be triggered mostly by sea bathing, affects young women and has a characteristic localization on the inferior facial contours and neck...
November 20, 2017: European Annals of Allergy and Clinical Immunology
Charoen Choonhakarn, Suteeraporn Chaowattanapanit, Narachai Julanon
BACKGROUND: An increase in dosages up to fourfold of second generation antihistamines is recommended for recalcitrant chronic spontaneous urticaria (CSU). No regimen guidelines about dose de-escalation, however, are mentioned once the disease is controlled. OBJECTIVE: To demonstrate the treatment outcomes and dose reduction in desloratadine assessed using the urticarial activity score over 7 consecutive days. METHODS: Medical records of all patients with CSU treated with desloratadine were collected retrospectively during a period from January 2010 to December 2013...
January 30, 2018: International Journal of Dermatology
Nehir Kurtas, Filippo Arrigoni, Edoardo Errichiello, Claudio Zucca, Cristina Maghini, Maria Grazia D'Angelo, Silvana Beri, Roberto Giorda, Sara Bertuzzo, Massimo Delledonne, Luciano Xumerle, Marzia Rossato, Orsetta Zuffardi, Maria Clara Bonaglia
INTRODUCTION: Phelan-McDermid syndrome (PMS) is caused by SHANK3 haploinsufficiency. Its wide phenotypic variation is attributed partly to the type and size of 22q13 genomic lesion (deletion, unbalanced translocation, ring chromosome), partly to additional undefined factors. We investigated a child with severe global neurodevelopmental delay (NDD) compatible with her distal 22q13 deletion, complicated by bilateral perisylvian polymicrogyria (BPP) and urticarial rashes, unreported in PMS...
January 29, 2018: Journal of Medical Genetics
Silvia Alberti-Violetti, Emilio Berti, Angelo V Marzano
Cutaneous vasculitides encompass a wide and heterogeneous group of diseases affecting skin blood vessels that are clinically characterized by polymorphic skin lesions, including in particular palpable purpura as well as urticarial and necrotic-ulcerative lesions, with possible, albeit rare, extracutaneous involvement. Cutaneous leukocytoclastic angiitis and urticarial vasculitis, which are the two prototypic and most common variants of this group, are usually idiopathic but may also be induced by different triggers, notably drugs and infections, or may manifest in association with systemic disorders, particularly lupus erythematosus...
January 24, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Harun Gunes, Feruza Turan Sonmez, Ayhan Saritas, Yasin Koksal
An acute coronary syndrome (ACS) occurring during the course of an allergic reaction is called Kounis syndrome (KS). The second case of KS induced by diclofenac potassium (DP) is presented in this report. A 67-year-old man was brought to our emergency department with the possible diagnosis of anaphylactic shock by the ambulance staff. It emerged that widespread erythema and pruritus developed after taking DP. Then, he lost consciousness. Diffuse urticarial lesions were detected on physical examination at the emergency department...
December 2017: Iranian Journal of Allergy, Asthma, and Immunology
Kimberly B Golisch, Silvija P Gottesman, Patricia Ferrer, Keliegh S Culpepper
Cutis laxa is a rare connective tissue disease involving damage to dermal elastic fibers creating a clinical appearance of loose, sagging skin. The condition can be either acquired or genetic. Autoimmune diseases, neoplasms, infections, and medications have been proposed as the cause of, or in association with, the acquired form. In nearly 50% of cases, erythematous plaques present before the onset of cutis laxa. Separately, urticarial vasculitis and systemic lupus erythematosus have been linked to cutis laxa acquisita...
January 11, 2018: American Journal of Dermatopathology
Dorota M Rowczenio, Shelly Pathak, Juan I Arostegui, Anna Mensa-Vilaro, Ebun Omoyinmi, Paul Brogan, Dan Lipsker, Thomas Scambler, Roger Owen, Hadija Trojer, Anna Baginska, Julian D Gillmore, Ashutosh D Wechalekar, Thirusha Lane, Rene Williams, Taryn Youngstein, Philip N Hawkins, Sinisa Savic, Helen J Lachmann
To date, the pathogenic mechanisms underlying Schnitzler's syndrome remain obscure, in particular the interplay between the monoclonal protein and increased IL-1beta production, although interest in the contribution of genetic factors has been fuelled by detection of somatic NLRP3 mosaicism in two patients with the variant-type Schnitzler's syndrome. At two specialist UK centres we have identified 21 patients, who fulfilled diagnostic criteria for Schnitzler's syndrome with urticarial rash, fever, arthralgia and bone pain; 47% reported weight loss, 40% fatigue and 21% lymphadenopathy...
December 28, 2017: Blood
Papapit Tuchinda, Corinne Erickson, Antonella Tammaro, Miya Okada Paterniti, Sarbjit S Saini, Anthony A Gaspari
A 33-year-old woman presented with recurring pruritic, erythematous papules around the mouth and on the hands, of 1.5 years' duration. These flares typically began several days before her menstrual cycle and persisted for approximately 1 week. Physical examination revealed urticarial plaques on the neck. Due to the nature of the eruption, which corresponded with her menstrual cycle, a diagnosis of autoimmune progesterone urticaria was considered and workup pursued.
2017: Skinmed
Carlos Dominguez-Massa, Ana M Bel-Minguez, Manuel Perez-Guillen, Alberto Berbel-Bonillo, Fernando Hornero-Sos
The incidence of allergic reactions due to mechanical prosthesis or rings is not well established. We report the case of a 56-year-old male patient who presented a persistent urticarial rash and anaphylactic shock after mitral valve repair surgery. Prick skin tests were positive for nickel. After removing the nucleus from the mitral annulus, the urticarial clinic disappeared.
December 20, 2017: Annals of Thoracic Surgery
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