Vasculitides
Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang in Roxburgh's Common Skin Diseases, 2022
Overview: This is a rare and often misdiagnosed entity that presents similarly to acute and chronic urticaria. Most cases are idiopathic, but associations with autoimmune diseases and infections, including the novel COVID-19 virus, have been drawn. There is a female predominance, particularly for patients in their 40s. Clinically and histopathologically, urticarial vasculitis is varied and can share clinical presentation with other diseases.
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).
Unremitting chronic skin lesions: a case of delayed diagnosis of glucagonoma
Published in Journal of Community Hospital Internal Medicine Perspectives, 2019
Hameem I. Kawsar, Alma Habib, Azhar Saeed, Anwaar Saeed
A 54-year-old Caucasian male with past medical history of hypertension, hyperlipidemia, insulin-dependent diabetes mellitus, and chronic skin rash presented to the emergency department (ED) with skin rash of 4-year duration that had worsened over the preceding 2 weeks. He was diagnosed with eczematous skin disease about 4 years ago and treated with topical steroid intermittently with variable response. About 2 months ago, he was started on doxycycline and oral prednisone for unremitting skin lesions. However, he had poor glycemic control with use of prednisone, and hemoglobin A1c (HbA1c) increased from 6.0% to 10.0%. A skin biopsy was done for a definitive diagnosis of this chronic skin lesion, and it was reported as urticarial vasculitis. Prednisone was discontinued for poor glycemic control, and dapsone was added. During the following 2 weeks, the rash progressed to involve both lower extremities, upper extremities, and trunk with swelling and bullae with no other identifiable trigger. In the ED, he reported of unintentional weight loss of 30 lbs in the preceding 3 months and denied any family history of malignancy or autoimmune disease. He also denied history of smoking, anorexia, or abdominal pain. On examination, the affected area of the skin was warm with diffuse erythema and edema throughout both lower extremities. Ulceration was prominent in the right lower extremity, trunk, bilateral upper extremities, and palms and soles (Figure 1). There were bullae with associated paresthesia. The patient was unable to bear weight and experienced sharp shooting pain in his bilateral feet. Further work up was directed to rule out other etiologies of skin rash and weight loss, including nutritional deficiency, infection, autoimmune disease, and occult malignancy. He had elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) at 32 (reference range 0–20 mm/h) and 8.05 (reference range <1.0 mg/dl), respectively. Comprehensive work up for autoimmune disease and infectious etiology were negative. Because of his recent significant weight loss, a computed tomography (CT) scan of chest, abdomen, and pelvis with contrast was done to rule out occult malignancy, which showed a large, heterogenously enhancing mid to distal pancreatic mass measuring 9.4 × 3.8 cm with surrounding low-attenuation soft tissue thickening (Figure 2). Tumor marker, CA 19–9 was 2.0 (reference range < 35 U/ml); however, glucagon was elevated to 2,178 (normal < 80 pg/dl). Prolactin, T4, and TSH levels were within normal range. Hemoglobin and hematocrit were low at 12.2 (reference range 13.5–16.5 gm/dl) and 36.1 (reference range 40–50%), respectively. Liver enzymes were within normal limit. There was no leukocytosis or fever, indicating cellulitis less likely.
Related Knowledge Centers
- Classical Complement Pathway
- Dapsone
- Histology
- Hydroxychloroquine
- Inhibitor Protein
- Mycophenolic Acid
- Vasculitis
- Colchicine
- Corticosteroid
- Methotrexate