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Urticaria is an inflammatory skin disorder that may occur in isolation or associated with angioedema and/or anaphylaxis. Clinically, it is characterized by the presence of smooth, erythematous or blanching, itchy swelling, called wheals or hives, which greatly vary in size and shape and last less than 24 h before fading to leave normal skin. Urtica...
Citations
... In particular, dermographism should also be considered, since this can often co-occur with CSU [5]. Other, albeit often rare, differential diagnoses include urticarial vasculitis, mastocytosis, mast cell activation syndrome, autoinflammatory syndromes (e.g., cryopyrin-associated periodic syndromes or Schnitzler's syndrome), bradykinin-mediated angioedema, urticarial drug eruption, eczematous urticarial dermatitis, Well's syndrome, bullous pemphigoid, insect bite reactions, and erythema multiforme [5, 54]. In patients with wheals only, urticarial vasculitis, urticarial pemphigoid, and other autoinflammatory disorders should be ruled out, and in patients with angioedema only, bradykinin-mediated angioedema-like angiotensinconverting enzyme (ACE) inhibitor-induced angioedema and hereditary angioedema should be ruled out [5]. ...
Chronic spontaneous urticaria (CSU) is an unpredictable inflammatory skin condition characterized by the spontaneous onset of itchy wheals, angioedema, or both, which occurs for longer than 6 weeks overall. Despite the relatively straightforward diagnostic algorithm for CSU, relying primarily on a detailed medical history and only limited laboratory tests, patients often wait years to be diagnosed, with many cycling through different healthcare practitioners before a diagnosis is made. Even then, current treatment options for CSU are limited, with approximately half of patients resistant to standard-of-care second-generation antihistamines at standard or higher doses. As such, there is an unmet need for improved, streamlined management for patients with CSU. Here, we review the evidence-based diagnostic algorithm for CSU, consider the required steps of the diagnostic workup, and provide practical, real-world advice on the management of CSU to improve the timely diagnosis and care of patients with this debilitating disease.
... Wheals, a common skin symptom in both urticaria and anaphylaxis, result from mediators released primarily from mast cells and basophils. The underlying mechanisms for urticaria and anaphylaxis share similarities, encompassing both immunological and non-immunological components (Macy, 2016;Montañez et al., 2017;Schettini et al., 2023). The immunological mechanisms can be IgE-dependent or IgE-independent. ...
Adverse drug reactions (ADRs) are an inherent aspect of drug use. While approximately 80% of ADRs are predictable, immune system-mediated ADRs, often unpredictable, are a noteworthy subset. Skin-related ADRs, in particular, are frequently unpredictable. However, the wide spectrum of skin manifestations poses a formidable diagnostic challenge. Comprehending the pathomechanisms underlying ADRs is essential for accurate diagnosis and effective management. The skin, being an active immune organ, plays a pivotal role in ADRs, although the precise cutaneous immunological mechanisms remain elusive. Fortunately, clinical manifestations of skin-related ADRs, irrespective of their severity, are frequently rooted in immunological processes. A comprehensive grasp of ADR morphology can aid in diagnosis. With the continuous development of new pharmaceuticals, it is noteworthy that certain drugs including immune checkpoint inhibitors have gained notoriety for their association with ADRs. This paper offers an overview of immunological mechanisms involved in cutaneous ADRs with a focus on clinical features and frequently implicated drugs.
Introduction:
Recent research has shown that blood coagulation and the extrinsic coagulation cascade are involved in the pathogenesis of chronic spontaneous urticaria (CSU), but little is known about the coagulation factors in angioedema.
Methods:
This study included 58 participants: 29 patients with chronic angioedema (14 with isolated angioedema and 15 with angioedema with wheals) and 29 healthy controls (HCs). We compared the values of coagulation factors in patients with isolated angioedema to those with wheals. Plasma levels of D-dimer, fibrinogen, and factor VII were measured by enzyme-linked immunosorbent assay (ELISA) for all participants.
Results:
Significantly higher D-dimer (p = 0.016; ε² = 0.381) and fibrinogen (p = 0.044; ε² = 0.331) levels were recorded in patients with angioedema (both groups) than in the HCs, with higher levels for angioedema with wheals. Factor VII and fibrinogen levels did not differ significantly between the groups with angioedema, but coagulation factors were more often elevated in both angioedema groups than in HCs.
Conclusions:
One characteristic of angioedema is an elevated blood coagulation potential, which may help produce fibrin and may be important in controlling angioedema attacks.
