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Allergic and Immunologic Reactions
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Saira N. Agarwala, Aspen R. Trautz, Sylvia Hsu
Differential diagnosis: Urticarial vasculitis is often indistinguishable from urticaria, but lesions last greater than 24 hours. The differential includes urticaria pigmentosa, familial cold urticaria, bradykinin-mediated angioedema (e.g., hereditary angioedema), and urticarial pemphigoid.
Urticaria and Angioedema
Published in Pudupakkam K Vedanthan, Harold S Nelson, Shripad N Agashe, PA Mahesh, Rohit Katial, Textbook of Allergy for the Clinician, 2021
Jenny M Stitt, Stephen C Dreskin
Three important conditions that are on the differential diagnosis of urticaria are urticarial vasculitis, urticaria pigmentosa and systemic mastocytosis. Urticarial vasculitis is characterized by hives that are typically painful or burning. The lesions can be non-blanching, typically last longer than 24 hours and often have associated ecchymosis, and resolve with residual hyperpigmentation of the skin or frank scar formation (Davis and Brewer 2004). Urticaria pigmentosa presents with brownish-red cutaneous lesions that become erythematous, pruritic and edematous after gentle stroking, called ‘Darier’s sign’. This physical stimulation activates the nest of mast cells that comprise the lesions of urticaria pigmentosa and is pathognomonic for urticaria pigmentosa (Brockow 2004). Of note, this sign may be absent if a patient is taking antihistamines. Systemic mastocytosis is a rare disorder of mast cell hyperplasia and proliferation. Mast cells may infiltrate the skin, bone marrow and other organs. Classification and severity are based in part on the extent of mast cell burden. Patients may have urticaria pigmentosa but also may experience episodic flushing, urticaria, angioedema, gastrointestinal distress, anaphylaxis and neuropsychiatric abnormalities (Lim et al. 2009, Metcalfe 2008).
Current and emerging pharmacotherapy for chronic spontaneous Urticaria: a focus on non-biological therapeutics
Published in Expert Opinion on Pharmacotherapy, 2021
Kam Lun Hon, Joyce T. S. Li, Alexander K.C. Leung, Vivian W. Y. Lee
Diagnosis of CSU is mainly made by detailed history and clinical examination. A thorough history includes onset and duration of wheals and/or angioedema, family and past medical history, associated symptoms, and possible triggers (e.g., stress, physical stimuli, alcohol, food allergy and medications). Since wheals or angioedema may be caused by other conditions, differential diagnoses should be excluded. The diagnosis of urticarial vasculitis or auto-inflammatory disorders is less likely in patients who only present with wheals. However, urticarial vasculitis can also present with angioedema when the vasculitis involves the capillaries and postcapillary venules of the deeper layer of the dermis and submucosa. In this regard, patients with urticarial vasculitis may have pain and burning sensation in addition to pruritus at the affected sites. In addition, the lesions in urticarial vasculitis persist longer (more than 24 hours, sometimes 72 hours) than those in CSU. Residual hyperpigmentation may be seen in about one-third of patients with urticarial vasculitis. In patients with recurrent episodes of angioedema but not wheals, angiotensin-converting enzyme inhibitor-induced angioedema, hereditary angioedema, and acquired C1 inhibitor deficiency should be excluded [5,9].
The COVID rash that puts the ‘U’ in GROUCH!
Published in Baylor University Medical Center Proceedings, 2021
Landon Hope, Brianna Hope, Jeannie Nguyen, Richard Hope, Michelle Tarbox
Urticarial vasculitis is believed to be a type III hypersensitivity reaction with immune complex depositions and is one of the clinical expressions of leukocytoclastic vasculitis.5 Many causes of urticarial vasculitis exist, the most frequent of which are drug reactions, viruses, and autoimmune diseases. Clinically, urticarial vasculitis is characterized by the appearance of urticarial lesions similar to wheals, with individual lesions present >24 hours. Although the lesions may be asymptomatic, they can be accompanied by a burning sensation, pain, and fever. Laboratory findings can include hypocomplementemia, especially in cases linked to connective tissue diseases.5
Hypocomplementemic urticarial vasculitis case with hemophagocytic lymphohistiocytosis following SARS-CoV-2 mRNA vaccination
Published in Immunological Medicine, 2023
Narumichi Iwamura, Katsumi Eguchi, Tomohiro Koga, Kanako Tsutsumi, Takeshi Araki, Toshiyuki Aramaki, Ayuko Takatani, Kaoru Terada, Yukitaka Ueki
Urticarial vasculitis (UV) was then suspected due to severe hypocomplementemia and urticarial rash, which is extremely rare disease; morbidity rate is 9.5 per million [4]. Urticaria-like skin lesions noticed in UV differed from urticaria as they occur as a chronic or recurrent course, with limited individual lesions (0.5–5 cm in diameter), lasting for more than 24 h, and leaving a dark red pigmentation [5]. The urticarial rash observed in the present case was 0.5–2 cm in size and faded away leaving pigmentation, something that is consistent with the characteristics of the urticarial rash observed in UV patients. The urticaria-like rash observed in this patient faded within 12 to 24 h, which is not characteristic of the skin rash seen in UV, but it could result from bepotastine administration. UV patients are reported to present arthralgia, myalgia observed in most of these patients [6], renal involvement is observed in 20% of them [7], COPD was observed in 20 to 30% [8], gastrointestinal symptoms in up to 30% [9], as well as other systemic symptoms. This case also presented arthralgia, myalgia, and tubular disorders. Hypocomplementemia is a useful predictor [8], while increased erythrocyte sedimentation and hypocomplementemia are the most common laboratory aberrations in UV patients. In the case described in the present study, the patient presented an increased erythrocyte sedimentation rate of 131 mm/h and acute hypocomplementemia; CH50 was below sensitivity measurement, C3 22 mg/dL, C4 1.5 mg/dL and complement transition reflected the disease status efficiently (Figure 4). UV without hypocomplementemia is defined as normocomplementemic urticarial vasculitis (NUVS), while UV with hypocomplementemia is defined as HUVS, which is the more critical type [9]. Despite the fact that anti-C1q antibodies could not be examined in the case presented herein due to technical difficulties. Autoantibodies, such as antinuclear antibodies are detected in 50% of HUVS patients [10]. In the case presented herein, rheumatoid factor and anti-CCP antibodies were detected that were consistent with the characteristics of HUVS. In addition, Antinuclear antibodies were negative on admission to our hospital, although they were positive in the previous test at Hospital A (anti-nuclear antibodies titer 160).