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Background: Hand eczema (HE) is a common skin disease characterized by itch, pain and visible skin changes such as fissures, erythema and vesicles. It is not yet clear which outcome domains are most important for patients. The Hand Eczema Core Outcome Set (HECOS) initiative is developing a consented set of core domains and suitable measurement instruments for the future application in all HE trials. This includes an online Delphi survey about core domains, which requires a ‘Long List’ of all domains that might be important to measure. Objectives: To compile a ‘Long List’ of candidate outcome domains for therapeutic HE trials with suggestions from patients and experts. Methods: First, 60 patients with chronic HE were interviewed at seven study sites in Croatia, Denmark, Germany, the Netherlands and Spain. Patients were asked about domains that were important from their perspectives. Second, 185 HE experts were invited by email to complete an online survey. With an open question, they were asked to suggest up to six domains. Results: Suggestions were provided by 58 patients and 82 experts. Most patients and experts suggested to measure the domains ‘signs’, ‘symptoms’ and ‘HE-related quality of life’. Specifically, >25% of patients said that less itch, pain or fissures indicated a successful treatment. Among experts, >25% suggested ‘itch’ and ‘ability to work’ as core sub-domains. Further outcomes from the domains ‘HE control over time’, ‘patient-reported treatment experience’ and ‘skin barrier function’ were mentioned. Conclusion: ‘Itch’ was rated high among patients with HE and professional HE experts. While patients emphasized fissures as important, experts underlined the ability to work. This investigation allowed us to define a ‘Long List’ of 7 candidate outcome domains with 58 sub-domains. From this list, a panel of stakeholders will select core domains during an online Delphi survey.

Few cases of isolated (nonsyndromic, nonendocrinopathy) familial acanthosis nigricans (FAN) have been described, and most have not included genetic testing. We present a three-generation family with isolated FAN, whose genetic study revealed a heterozygous FGFR3 gene variant [NM_0001425: c.2302G>T; p.(Glu768Ter); E768X]. We propose genetic mechanisms that could explain the pro-mitogenic effect on the skin without bone involvement and the spectrum of clinical manifestations associated with germline FGFR3 mutations.

We describe a patient with unilateral ulcerations on the forehead and scalp, occurring 3 months after herpes zoster infection. Further investigations were unremarkable. Histology showed epidermal and upper dermal ulceration associated with a mild nonspecific dermal inflammatory infiltrate composed of lymphoid cells and histiocytes.

Purpose of review: contact urticaria syndrome includes contact urticaria and protein contact dermatitis. Underreport, underdiagnosis, or misdiagnosis of entities within the contact urticaria syndrome is believed to be common, especially in the occupational setting. This review provides a structured overview of the entities comprised in this syndrome as well as the diagnostic work-up and management strategies. Recent findings: contact urticaria syndrome has been increasingly described due to personal protective equipment and hand sanitizers in the context of the COVID-19 pandemic. The use of legal cannabis products has led to a rise in occupational cases of contact urticaria to cannabis. A declining trend in the evolution of contact urticaria has been described for natural rubber latex allergy due to the use of synthetic gloves. Prick test has been proposed as a screening method, particularly if multiple products are to be tested, instead of the classical sequential scheme. Summary: physicians should be aware of the growing number of culprit agents leading to contact urticaria syndrome. Clinical presentation may be challenging since it includes immediate urticaria and/or eczema and even more generalized reactions. Diagnosis requires a high degree of suspicion, detailed occupational history, and complementary tests, including skin testing. The best treatment is to avoid contact with the culprit agent and to implement preventive measures.

Background: We aim to investigate the effects of the Bruton’s tyrosine kinase (BTK) inhibitor remibrutinib on human in vitro cell activation induced by serum obtained from chronic spontaneous urticaria (CSU) and chronic inducible urticaria (CIndU) patients. Methods: 22 patients with CSU (mean age 52 years, 27% women) and 22 with CIndU (46 years, 27% women) were included. A sex-equivalent control group was enrolled. Total IgE serum levels and the FcεR1a expression on blood basophils were determined before the initiation of omalizumab therapy. Patients were then classified as responders or non-responders to anti-IgE therapy, according to their clinical data and IgE and FcεR1a receptor expression. Mast cell generation and stimulation and basophil stimulation were performed. Flow cytometry was used to analyze samples. Percentage of activation, degranulation and inhibition in basophils and mast cells are studied. Results: Main results show that remibrutinib inhibits mast cells and basophil degranulation induced by IgE cross-linking. Also, serum from patients induced a greater degranulation of mast cells and basophils compared to controls. We found that activation of mast cells and basophils by patient sera was not related to omalizumab responsiveness. Conclusion: Our results show that remibrutinib is able to inhibit the degranulation of human basophils and mast cells in vitro, independently of the patient’s expression of FcεR1 expression and response to omalizumab.

A 59-year-old woman presented with a persistent eruption manifested as multiple agminated miliary facial papules. Histopathological examination showed prominent nodular dermal lymphoid infiltrates with hyperplastic follicles that were initially interpreted as B-cell reactive lymphoid hyperplasia. Several years later, an additional biopsy showed a dense perifollicular infiltrate with reactive primary and secondary follicles. Accompanying T cells corresponded to CD3/CD4/PD1/CXCL13-positive cells and scattered Epstein-Barr virus-positive B cells were identified by in situ hybridization. A monoclonal T-cell population was demonstrated by TCRγ and TCRβ Polymerase Chain Reaction amplification, as well as a minor abnormal circulating T-cell population by flow cytometry (0.62% of the white blood cells, CD4+CD3s-CD7-). A biopsy specimen from an enlarged right supraclavicular lymph node disclosed nodal involvement by angioimmunoblastic T-cell lymphoma. The observation of B-cell dermal nodular infiltrates with well-demarcated lymphoid aggregates forming primary lymphoid follicles may lead to overlook the T-cell component in some cases of angioimmunoblastic T-cell lymphoma. In such cases, a careful assessment of the apparently minor T-cell component is important to establish a correct diagnosis.

Background: cold urticaria (ColdU), that is, the occurrence of wheals or angioedema in response to cold exposure, is classified into typical and atypical forms. The diagnosis of typical ColdU relies on whealing in response to local cold stimulation testing (CST). It can also manifest with cold-induced anaphylaxis (ColdA). We aimed to determine risk factors for ColdA in typical ColdU. Methods: an international, cross-sectional study COLD-CE was carried out at 32 urticaria centers of reference and excellence (UCAREs). Detailed history was taken and CST with an ice cube and/or TempTest(R) performed. ColdA was defined as an acute cold-induced involvement of the skin and/or visible mucosal tissue and at least one of: cardiovascular manifestations, difficulty breathing, or gastrointestinal symptoms. Results: of 551 ColdU patients, 75% (n = 412) had a positive CST and ColdA occurred in 37% (n = 151) of the latter. Cold-induced generalized wheals, angioedema, acral swelling, oropharyngeal/laryngeal symptoms, and itch of earlobes were identified as signs/symptoms of severe disease. ColdA was most commonly provoked by complete cold water immersion and ColdA caused by cold air was more common in countries with a warmer climate. Ten percent (n = 40) of typical ColdU patients had a concomitant chronic spontaneous urticaria (CSU). They had a lower frequency of ColdA than those without CSU (4% vs. 39%, p = .003). We identified the following risk factors for cardiovascular manifestations: previous systemic reaction to a Hymenoptera sting, angioedema, oropharyngeal/laryngeal symptoms, and itchy earlobes. Conclusion: coldA is common in typical ColdU. High-risk patients require education about their condition and how to use an adrenaline autoinjector., GA(2)LEN UCARE Network

With COVID-19 mass vaccination, the spectrum of cutaneous reactions to mRNA COVID-19 vaccines described is increasing.1 While some of these clinical pictures are believed to have an allergic background, others may be related to the activation of pro-inflammatory immune responses.2,3 We present two cases of psoriasis associated to COVID-19 vaccination.

International audience; Background: Different textile constituents may act as allergens and/or irritants and provoke textile contact dermatitis (TCD).Objectives: To report a case of TCD caused by ethylene glycol monododecyl ether and 2.4-dichlorophenol present in a bikini.Methods: A woman presented with an eczematous, pruritic rash in the area of the straps and back. Patch testing was performed with the European baseline, textile, sunscreen and photo-patch series, the bikini “as is”, and ethanol and acetone extracts of the bikini. Thin-layer chromatography (TLC) of the extracts and gas chromatography-mass spectrometry (GC-MS) analysis were used to elucidate the culprit agents.Result: Positive reactions were found to the bikini “as is”, and to the ethanol and acetone extracts. Patch testing with TLC strips showed a strong reaction to particular fractions (3 and 4). GC-MS was performed to identify substances in each fraction, and those suspected to be skin sensitizers were patch tested. On day 4 (D4) positive reactions to ethylene glycol monododecyl ether (IR) and 2.4-dichlorophenol (++) were observed.Conclusion: A myriad of chemical compounds can be found in clothing. Ethylene glycol monododecyl ether and 2.4-dichlorophenol were identified as the potential culprits of this bikini TCD.

Immediate contact skin reactions are considered a subset of the contact urticaria syndrome, which includes contact urticaria and protein contact dermatitis (PCD). These entities clinically manifest as development of wheals, erythema, and/or eczema within minutes after contact with proteins or low-molecular-weight allergens. Herein, we report a case compatible with a PCD caused by exposure to mango fruit.