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Urticaria haemorrhagica profunda

A Wollenberg1, S Hänel, M Spannagl

  • 1Department of Dermatology, Ludwig-Maximilians University, Munich, Germany.

The British Journal of Dermatology
|January 1, 1997
PubMed
Summary

A rare case of adolescent subcutaneous hemorrhage linked to urticarial vasculitis is presented. This unique bleeding manifestation, occurring without trauma or bleeding disorders, suggests a potential link between superficial and deep urticarial vasculitis.

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Area of Science:

  • Dermatology
  • Immunology
  • Hematology

Background:

  • Subcutaneous hemorrhage without trauma or bleeding disorders is rare, primarily seen in childhood.
  • Urticarial vasculitis typically presents with cutaneous inflammation and vasculitic lesions.

Observation:

  • A 16-year-old male experienced massive subcutaneous hemorrhages in angioedema-prone areas (periorbital, perioral, lingual, laryngeal).
  • The patient had a history of atopic diathesis, hay fever, and alopecia areata.
  • Transient antiphospholipid antibodies, including lupus anticoagulant, were detected.

Findings:

  • The patient's presentation combined features of urticarial vasculitis with deep subcutaneous hemorrhage.
  • The hemorrhage occurred in areas characteristic of angioedema, suggesting a deep-seated inflammatory process.

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  • The presence of transient antiphospholipid antibodies may play a role in the pathogenesis.

    • Implications:

    • This case suggests a potential pathophysiological link between superficial urticarial vasculitis and urticaria profunda angioedema.
    • Understanding this connection may aid in diagnosing and managing similar unique bleeding manifestations in adolescents.
    • Further research into the relationship between vasculitis, angioedema, and antiphospholipid antibodies is warranted.