Wujanto Lareina, Ross Clare
Dermatology Department, Ealing Hospital, Southall, Middlesex, UK.
BMJ Case Rep. 2010 Oct 22;2010:bcr0320102834. doi: 10.1136/bcr.03.2010.2834.
A 22-year-old man presented with recurrent palpable purpuric rash. His clubbing relates to underlying cystic fibrosis (CF) and his rash was identified as CF-related vasculitis, a rare extrapulmonary manifestation of the disease. It occurs predominantly on the lower limbs, mainly over the dorsa of the feet, ankles and tibial surfaces. The rash occurred while the patient had an infective exacerbation of CF (IECF), however, there had also been previous occurrences without worsening of his pulmonary symptoms, to which the rash remitted spontaneously. The patient responded well to immunosuppression, which was given on this admission due to worsening of his CF-related vasculitis. He died 18 months within the onset of his initial rash.
一名22岁男性出现反复可触及的紫癜性皮疹。他的杵状指与潜在的囊性纤维化(CF)有关,其皮疹被诊断为CF相关血管炎,这是该疾病一种罕见的肺外表现。皮疹主要出现在下肢,主要分布在足背、脚踝和胫前表面。皮疹在患者CF感染加重期(IECF)出现,但之前也有过发作,且肺部症状并未加重,皮疹可自行消退。由于CF相关血管炎恶化,此次入院给予免疫抑制治疗,患者反应良好。他在初次出现皮疹后的18个月内死亡。