[Purpura fulminans (symmetric peripheral gangrene): 7-year consecutive case review in Japan]

Acute infectious purpura fulminans (AIPF), a rare syndrome of infection-induced ischemic necrosis of the extremities (symmetric peripheral gangrene), is due most often to Neisseria meningitidis in the US and the UK, but is not endemic to Japan.

We reviewed clinical AIPF manifestations in Japan.

(A) We retrospectively analyzed six cases of AIPF in the last seven years. (B) We reviewed the medical literature up to 2008 using PubMed search and Japana Centra Revuo Medicina (Igakuchuozasshi) findings.

(A) None of the 6 subjects-5 men and 1 woman aged 44 to 69 old- had a history of splenectomy. Except for one case, their disease was community-acquired and immunocompetent. Causative organisms were Streptococcus pneuomoniae (66.7%), Gram negative rods, and Legionella pneumophilla. No localized infectious focus was apparent except for one of Legionella pneumonia. Systemic purpura progressed rapidly within 12 to 96 hours of initial fever or influenza-like symptoms. Main AIPF manifestations are shock and disseminated intervascular coagulation, but not all subjects had both. Six-month mortality was 33.3%, with death within two days of admission. Survivors all required at least two limbs to be amputated. (B) The Japanese literature reported AIPF occurring in 64 cases- 11 neonatal due to congenital protein C deficiency, seven pediatric, and 46 adults. Organisms most commonly involved were S. pneumoniae at 41.3%, and Neisseria meningitidis at 15.2%. The most common pathogen reported in a PubMed search was Neisseria meningitidis.

In Japan, S. pneumoniae is the most frequent isolate associated with AIPF, and progression to AIPF occurs mainly in adults, who commonly suffer frequent amputations and high mortality. Rapid progressing systemic purpura following common-cold-like symptoms is the key to early diagnosis, even if the patient is immunocompetent, not splenectomized, or has no apparent infectious foci.