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Abstract: Background: Mastocytosis is a heterogeneous disease characterized by a clonal expansion of mast cells in various organs. The vast majority of patients suffer from signs and symptoms caused by mediator release from mast cells. Although the disease burden is high, there is currently no specific and validated instrument to measure and monitor signs and symptoms in patients with mastocytosis. Objective: To develop and validate a disease‐specific tool to measure and monitor the activity of signs and symptoms in patients with mastocytosis, the Mastocytosis Activity Score (MAS). Methods: Nineteen potential MAS items were developed in a combined approach consisting of semi‐structured patient interviews, expert input and literature research. Item selection was performed by impact analysis with 76 patients followed by a review for face validity. The resulting MAS was tested for validity, reliability and influence factors. In parallel, a US American English version of the MAS was developed. Results: A total of 68 mastocytosis patients took part in the MAS validation study. The final 9‐item MAS was found to have a three‐domain structure (“skin,” “gastrointestinal tract” and “other”), a valid total score and an excellent test‐retest reliability. Multiple regression analysis revealed that disease duration, age or gender is not a significant determinant of the MAS results. Conclusions: The MAS is a disease‐specific, valid and reliable patient‐reported outcome measure for adult patients with cutaneous and indolent systemic mastocytosis. It may serve as a valuable tool to measure and monitor mastocytosis activity, both, in clinical trials and in routine care. [ABSTRACT FROM AUTHOR]

Background Histamine intolerance and pseudoallergy to foods have been suggested to be causes of chronic spontaneous urticaria ( CSU) with some patients reporting exacerbation with histamine-rich foods. Objective The study aim was to identify the rate of histamine-intolerant CSU patients and to characterize the relevance of histamine intolerance as an underlying cause of CSU. Methods A cohort of 157 of moderate to severe CSU patients ( UAS7 ≥ 10) was asked to provide a detailed clinical history, particularly in relation to symptom development after eating histamine-rich foods. They subsequently undertook a histamine-free pseudoallergen-low diet followed by a double-blind, placebo-controlled oral histamine provocation (75 mg). Results One third of patients (34%) had a positive history of histamine intolerance. There was no statistical difference between the mean UAS7 scores of patients with positive and negative histories (22.4 ± 1.0 vs. 22.7 ± 0.8). When kept on diet, 46% of patients responded with reduced CSU activity ( UAS7 reduction of ≥7). Following double-blind, placebo-controlled oral histamine provocation, 17% of patients gave a positive weal response. There appeared to be little relationship between patient history, response to diet and the weal response to oral histamine provocation. First, the history-positive and -negative groups contained similar proportions of diet and histamine provocation weal-positive patients. Second, the diet-positive and -negative groups contained similar proportions of history-positive and histamine provocation weal-positive patients. Third, the histamine provocation weal-positive and -negative groups had similar rates of history- and diet-positive patients. Finally, only 2 of the 157 patients were positive in all three domains. Conclusions CSU due to histamine intolerance appears to be rare and cannot be diagnosed based on the history. The study confirms that avoidance diets low in pseudoallergens can improve urticaria symptoms, this is probably not due to the absence of dietary histamine. [ABSTRACT FROM AUTHOR]

Abstract: This supplement reports proceedings of the second international Global Urticaria Forum, which was held in Berlin, Germany in November 2015. Despite the clear international guideline, there remain a number of controversies and challenges in the management of patients with chronic urticaria (CU). As a result of major advancements in urticaria over the past 4 years, the current EAACI/GA2LEN/EDF/WAO urticaria guideline treatment algorithm requires updating. Case studies from patients with chronic spontaneous urticaria (CSU) [also called chronic idiopathic urticaria (CIU)], chronic inducible urticaria (CIndU) or diseases and syndromes related to CU are useful in describing and exploring challenges in disease management. Case studies of specific CSU patient populations such as children with CU or patients with angio‐edema but no hives also require consideration as potentially challenging groups with unmet needs. The current EAACI/GA2LEN/EDF/WAO urticaria guideline provides a general framework for the management of patients with CU but, as these cases highlight, a personalized approach based on the expert knowledge of the physician may be required. [ABSTRACT FROM AUTHOR]

Abstract: This supplement reports proceedings of the second international Global Urticaria Forum, which was held in Berlin, Germany in November 2015. In 2011, a report of the GA2LEN task force on urticaria outlined important and unanswered questions in chronic urticaria (CU). These included, but were not limited to, questions on the epidemiology and course of chronic spontaneous urticaria (CSU) [also called chronic idiopathic urticaria (CIU)], the resources allocated for the diagnosis and treatment of CSU, whether patients with angioedema as an isolated symptom can be regarded as a subgroup of CSU, and the efficacy and long‐term safety of therapies. Many of these questions have been addressed by recent studies. Some of the answers obtained raise new questions. Here, we summarize some of the key insights on CU obtained over recent years, and we discuss old and new unmet needs and how to address them with future studies. We need to analyze the influence of recent advances in understanding of the burden of CU on patients and society, disease management and the CU patient journey. Our increased understanding of urticarial pathophysiology and consideration of the patient as a whole will need to be translated to better treatment algorithms and protocols. Actions to address these challenges include the 5th International Consensus Meeting on Urticaria, which will take place later this year. The formation of a global network of Urticaria Centers of Reference and Excellence over the next few years has also been proposed, with the aim of providing consistent excellence in urticaria management and a clear referral route, furthering knowledge of urticaria through additional research and educating/promoting awareness of urticaria. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 71-year old woman with systemic mastocytosis with history of smoking. It is noted that she was further diagnosed with tripe palms that are characterized clinically by thickened velvety palms with pronounced dermatoglyphics. The article mentions that Anaplastic lymphoma kinase (ALK) fusion oncogenes represent a novel molecular target in a small subset of non-small cell lung cancers (NSCLC).

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Background: Patients with chronic urticaria (CU) frequently exhibit positive skin test reactions to autologous serum (ASST). Therapies aimed at inducing tolerance to circulating histamine-releasing factors in ASST+ CU patients, e.g. by treatment with autologous whole blood (AWB), have not yet been tested. Objective: To test whether ASST+ CU patients can benefit from repeated low-dose intramuscular injections of AWB. Methods: We characterized CU severity and duration, anti-FcεRI and anti-IgE expression, use of antihistamines, and quality of life in 56 CU patients (ASST+: 35, ASST–: 21) and assessed the therapeutic effects of 8 weekly AWB injections in a randomized, placebo-controlled, single-blind, parallel-group trial. Results: Numbers, size, intensity, and/or duration of CU symptoms, quality of life, as well as expression of anti-FcεRI or anti-IgE were similar in ASST+ and ASST– CU patients. However, CU in ASST+ patients was of longer duration and required markedly more antihistaminic medication. Interestingly, ASST+ patients, but not ASST– patients, showed significantly (1) reduced CU activity, (2) decreased use of antihistamines, and (3) improved quality of life after AWB treatment. Placebo treatment was ineffective in both groups, but differences of AWB and placebo treatment responses did not achieve statistical significance in either group, most likely due to the limited number of patients treated. Conclusion: Our findings suggest that ASST+ CU is clinically different from other CU subforms and that ASST+ CU patients can benefit from AWB therapy. Copyright © 2006 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Biró T, Ko MC, Bromm B, Wei ET, Bigliardi P, Siebenhaar F, Hashizume H, Misery L, Bergasa NV, Kamei C, Schouenborg J, Roostermann D, Szabó T, Maurer M, Bigliardi-Qi M, Meingassner JG, Hossen MA, Schmelz M, Steinhoff M. How best to fight that nasty itch– from new insights into the neuroimmunological, neuroendocrine, and neurophysiological bases of pruritus to novel therapeutic approaches.While the enormous clinical and psychosocial importance of pruritus in many areas of medicine and the detrimental effects of chronic‘itch’ on the quality of life of an affected individual are widely appreciated, the complexity of this sensation is still often grossly underestimated. The current Controversies feature highlights this complexity by portraying pruritus as a truly interdisciplinary problem at the crossroads of neurophysiology, neuroimmunology, neuropharmacology, protease research, internal medicine, and dermatology, which is combated most successfully if one keeps the multilayered nature of‘itch’ in mind and adopts a holistic treatment approach– beyond the customary, frequently frustrane monotherapy with histamine receptor antagonists. In view of the often unsatisfactory, unidimensional, and altogether rather crude standard instruments for pruritus management that we still tend to use in clinical practice today, an interdisciplinary team of pruritus experts here critically examines recent progress in pruritus research that future itch management must take into consideration. Focusing on new insights into the neuroimmunological, neuroendocrine, and neurophysiological bases of pruritus, and discussing available neuropharmacological tools, specific research avenues are highlighted, whose pursuit promises to lead to novel, and hopefully more effective, forms of pruritus management. [ABSTRACT FROM AUTHOR]