Cutaneous necrosis in pregnancy secondary to activated protein C resistance in hereditary angioedema.
A 26-year-old woman with hereditary angineurotic oedema (HAE) presented at 22 weeks gestation with severe cutaneous necrosis similar to that seen in coumarin skin necrosis. Protein S deficiency secondary to HAE and pregnancy was postulated. Treatment with heparin, C1-inhibitor concentrates, systemic steroids and surgical debridement resulted in a successful outcome for both mother and child. Subsequent investigations revealed normal levels of protein C, antithrombin III, total protein S, free protein S but reduced function protein S activity with evidence of activated protein C resistance. Cutaneous necrosis has not been reported in associated with activated protein C resistance previously and the possible mechanisms are discussed.
Perkins
W
W
Department of Dermatology, Royal South Hants Hospital, Southampton, UK.
Downie
I
I
Keefe
M
M
Chisholm
M
M
D000328:Adult; D000799:Angioedema / Q000235:genetics*; D005260:Female; D006801:Humans; D009336:Necrosis; D011247:Pregnancy; D011248:Pregnancy Complications; D020151:Protein C Deficiency; D012867:Skin / Q000473:pathology*