Hypocomplementemic Urticarial Vasculitis Syndrome or Systemic Lupus Erythematosus in Evolution?

Kesarwani, Vartika; Phachu, Deep; Trivedi, Ruchir

doi:10.7759/cureus.23429

Cited by 3 publications

(1 citation statement)

References 10 publications

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“…The diagnosis of HUVS is particularly challenging due to its overlap with more common conditions such as chronic idiopathic urticaria, other forms of vasculitis, and autoimmune disorders such as systemic lupus erythematosus (SLE) [ 9 ]. Key to diagnosing HUVS is the presence of urticarial lesions that persist for more than 24 hours, often leaving behind hyperpigmentation, systemic symptoms, and low complement levels [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

Breaking Out of the Hive: A Case Report on Hypocomplementemic Urticarial Vasculitis Syndrome

Louis,

Akhtar,

Gibson

2024

Cureus

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Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder characterized by chronic urticaria, systemic vasculitis, and hypocomplementemia, posing significant diagnostic challenges due to its overlap with common conditions and varied systemic manifestations. We report the case of a 36year-old female with a history of post-birth cerebral hemorrhage and seizure disorder, who presented with abdominal pain, diarrhea, and subtle urticarial lesions. Initial investigations by gastroenterology suggested inflammatory bowel disease (IBD), but persistent symptoms and evolving cutaneous signs prompted further evaluation. A skin biopsy demonstrated leukocytoclastic vasculitis, while serological tests showed hypocomplementemia and positive antineutrophil cytoplasmic antibodies (ANCA), confirming HUVS. The patient's management included high-dose corticosteroids and mycophenolate mofetil, with partial symptom relief. Subsequent introduction of rituximab markedly improved her gastrointestinal and dermatological symptoms, highlighting its effectiveness in treating refractory HUVS. This case emphasizes the necessity for vigilance, interdisciplinary collaboration, and personalized treatment adaptations in managing HUVS.

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Section: Discussionmentioning

confidence: 99%

Breaking Out of the Hive: A Case Report on Hypocomplementemic Urticarial Vasculitis Syndrome

Louis,

Akhtar,

Gibson

2024

Cureus

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Unmasking rare diseases: is it angioedema or pseudo angioedema?

Boustani,

Ciotoracu,

Băloiu

et al. 2024

Alergologia

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Case Report: Hypocomplementemic urticarial vasculitis syndrome in a pediatric patient with complement factor 1 deficiency

Lin,

Kafisheh,

Elder

2024

Front. Pediatr.

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Urticarial vasculitis (UV) is a type III hypersensitivity reaction, characterized by immune complex deposition in small vessels leading to complement activation. Hypocomplementemic urticarial vasculitis syndrome (HUVS) represents the most severe form of UV, manifesting as chronic and recurrent urticarial skin lesions with leukocytoclastic vasculitis on histology, hypocomplementemia, anti-C1q antibodies, and systemic organ involvement. This case study focuses on an adolescent who initially presented with invasive pneumococcal infection and was later diagnosed with two rare disorders: HUVS and coexisting complement factor 1 (CF1) deficiency by genotyping. The role of CF1 deficiency in the development of HUVS in this patient is uncertain but has not previously been described.

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Hypocomplementemic Urticarial Vasculitis Syndrome or Systemic Lupus Erythematosus in Evolution?

Cited by 3 publications

References 10 publications

Breaking Out of the Hive: A Case Report on Hypocomplementemic Urticarial Vasculitis Syndrome

Breaking Out of the Hive: A Case Report on Hypocomplementemic Urticarial Vasculitis Syndrome

Unmasking rare diseases: is it angioedema or pseudo angioedema?

Case Report: Hypocomplementemic urticarial vasculitis syndrome in a pediatric patient with complement factor 1 deficiency

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