Urticaria haemorrhagica profunda

Abstract

Substantial subcutaneous haemorrhage without preceding trauma or underlying bleeding disorder is a rare occurrence in dermatological practice, essentially restricted to early childhood (acute haemorrhagic oedema of childhood). We report an adolescent with a morphologically unique bleeding manifestation. A 16-year-old boy presented with two episodes of massive subcutaneous haemorrhage in association with urticarial vasculitis. There was no history of preceding trauma or haemorrhagic disorder. Haemorrhage was observed in areas typically affected by angioedema, such as the periorbital, perioral, lingual, sublingual and laryngeal areas. History revealed an atopic diathesis with hay fever and examination showed alopecia areata. An antinuclear antibody titre and the presence of lupus anticoagulant indicated transient antiphospholipid antibodies. As urticaria corresponds to urticaria profunda angioedema, we hypothesize a pathophysiological relationship between superficial urticarial vasculitis and the deep variant of urticarial vasculitic disease, leading to the unique morphology present in our patient.

Chronic urticaria in childhood

“…Systemic lupus may occasionally manifest urticarialvasculitis as a presenting feature ( 59 ). Acute haemorrhagicoedema of infancy is probably a rare form of urticarial vasculitis manifestingas purpuric angioedema ( 60 ). However, it has alsobeen taken to be a presentation of Henoch‐Schönlein purpura ( 61 ).…”

A Challenging Case of Subcutaneous and Mucosal Haemorrhage Caused by Urticarial Vasculitis (Urticaria Haemorrhagica Profunda)

“…The first case was described more than 100 years ago by Wills & Lond ( 16 ). In 1997, Wollenberg et al ( 1 ) described the exact same symptoms in a 16 year old boy and another similar case was reported in 2003 ( 17 ). Histopathologically, the haemorrhagic lesions in all reported cases are similar to those of acute haemorrhagic oedema of childhood, and show signs of leucocytoclastic vasculitis.…”

“…As angioedema is the deep dermal and subcutaneous equivalent of urticaria, there may be a pathophysiological relationship between superficial urticarial vasculitis and a deeper haemorrhagic vasculitis, as presented in our case. Wollenberg et al ( 1 ) were the first to present this hypothesis and the authors suggested the term “urticaria hemorrhagica profunda” to describe this rare clinical picture.…”

“…The term urticaria haemorrhagica profunda was first proposed by Wollenberg et al ( 1 ) in 1997 to describe the unusual clinical presentation of urticarial vasculitis and subcutaneous haemorrhage. Urticarial vasculitis is a rare disease characterized by recurrent long-lasting urticarial lesions with histopathological evidence of leucocytoclastic vasculitis.…”