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NORMOCOMPLEMENTEMIC URTICARIFORM VASCULITIS: ONE OF THE VARIOUS FORMS OF LEUCOCYTOCLASTIC VASCULITIS

BACKGROUND
Urticarial vasculitis is a cutaneous vasculitis characterized by urticarial lesions lasting more than 24 hours, histopathologically presenting as leukocytoclastic vasculitis. It is a clinical-pathological entity caused by the deposition of immune complexes in arterioles, capillaries, and post-capillary venules in the skin. It can be divided into two groups based on complement levels: normocomplementemic and hypocomplementemic.
OBJECTIVES
To discuss a rare rheumatological disease of high severity and morbidity raises awareness of the condition within the medical community, thereby aiding its identification.
METHODS
Case report of a patient treated at a university hospital in the interior of the ``Triângulo Mineiro`` region.
RESULTS
Female patient, 31 years old, previously healthy. She reported a fever for 3 weeks, associated with profuse sweating, chills, occipital headache, and irregular erythematous non-scaling, painless plaques on the trunk and limbs. Initially, a non-specific infectious condition was treated with antibiotics without clinical improvement. The condition recurred after 5 days, evolving with neuropathic pain on the posterior aspect of the right leg and dorsal region of the right foot, of intense severity, unresponsive to analgesics. The patient sought medical attention again in the outpatient service, and laboratory tests showed evidence of leukocytosis with a predominance of segmented and rod-shaped white blood cells. The patient was then referred for investigation at the University Hospital. During hospitalization, the condition fluctuated, with persistent fever spikes and previously reported pains. Infectious screening was conducted without alterations, including cerebrospinal fluid analysis. Imaging tests revealed left lung atelectasis and pleural effusion, along with signs of idiopathic intracranial hypertension on magnetic resonance imaging. A skin biopsy was performed, suggestive of normocomplementemic urticarial vasculitis.
CONCLUSION
Although a rare rheumatological pathology with imprecise incidence and prevalence, normocomplementemic urticarial vasculitis is potentially serious, highlighting the need to consider this diagnosis when a patient presents with chronic urticaria or acute urticarial lesions. Early diagnosis is crucial for proper management, involving corticosteroid therapy and immunosuppression tailored to the patient’s profile and associated comorbidities.

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NORMOCOMPLEMENTEMIC URTICARIFORM VASCULITIS: ONE OF THE VARIOUS FORMS OF LEUCOCYTOCLASTIC VASCULITIS

  • DOI: https://doi.org/10.22533/at.ed.1594702430076

  • Palavras-chave: Urticarial vasculitis, Normocomplementemic, Leukocytoclastic vasculitis, Persistent urticaria theoretical.

  • Keywords: Urticarial vasculitis, Normocomplementemic, Leukocytoclastic vasculitis, Persistent urticaria theoretical.

  • Abstract:

    BACKGROUND
    Urticarial vasculitis is a cutaneous vasculitis characterized by urticarial lesions lasting more than 24 hours, histopathologically presenting as leukocytoclastic vasculitis. It is a clinical-pathological entity caused by the deposition of immune complexes in arterioles, capillaries, and post-capillary venules in the skin. It can be divided into two groups based on complement levels: normocomplementemic and hypocomplementemic.
    OBJECTIVES
    To discuss a rare rheumatological disease of high severity and morbidity raises awareness of the condition within the medical community, thereby aiding its identification.
    METHODS
    Case report of a patient treated at a university hospital in the interior of the ``Triângulo Mineiro`` region.
    RESULTS
    Female patient, 31 years old, previously healthy. She reported a fever for 3 weeks, associated with profuse sweating, chills, occipital headache, and irregular erythematous non-scaling, painless plaques on the trunk and limbs. Initially, a non-specific infectious condition was treated with antibiotics without clinical improvement. The condition recurred after 5 days, evolving with neuropathic pain on the posterior aspect of the right leg and dorsal region of the right foot, of intense severity, unresponsive to analgesics. The patient sought medical attention again in the outpatient service, and laboratory tests showed evidence of leukocytosis with a predominance of segmented and rod-shaped white blood cells. The patient was then referred for investigation at the University Hospital. During hospitalization, the condition fluctuated, with persistent fever spikes and previously reported pains. Infectious screening was conducted without alterations, including cerebrospinal fluid analysis. Imaging tests revealed left lung atelectasis and pleural effusion, along with signs of idiopathic intracranial hypertension on magnetic resonance imaging. A skin biopsy was performed, suggestive of normocomplementemic urticarial vasculitis.
    CONCLUSION
    Although a rare rheumatological pathology with imprecise incidence and prevalence, normocomplementemic urticarial vasculitis is potentially serious, highlighting the need to consider this diagnosis when a patient presents with chronic urticaria or acute urticarial lesions. Early diagnosis is crucial for proper management, involving corticosteroid therapy and immunosuppression tailored to the patient’s profile and associated comorbidities.

  • Gabriella Stefenoni Kruger
  • Aurélio Rosa Borges
  • Juliana Tereza Nose
  • Laura Gualberto Menezes
  • Luana Farid Guimarães de Castro
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