Severe digital necrosis in an elderly patient with heterozygous factor V Leiden mutation

Abstract

We report an 81-year-old female with a heterozygous factor V Leiden mutation who developed purpura fulminans. Digital necrosis, a characteristic clinical feature of purpura fulminans was prominent. Purpura fulminans is more common in children and adult cases are rare. Of eight reported cases of purpura fulminans resulting from a heterozygous factor V Leiden mutation recorded in the literature, only two were in adults: 40 and 42 years of age, respectively. This is the first report of this condition arising in a patient in her eighties.

Symmetrical peripheral digital gangrene following severe Plasmodium falciparum malaria‐induced disseminated intravascular coagulopathy

“…As in our case, symmetrical peripheral digital necrosis is often a manifestation of a life‐threatening illness (1–4). For those who survive the acute illness, symmetrical peripheral digital necrosis is a major debilitating complication.…”

“…In this situation there is often excessive disproportionate consumption of one or more anti‐coagulant factors, such as protein C or S, leading to the hypercoagulable state (3). Rarely an inherited deficiency of an anti‐coagulant factor, or in the case of anti‐phospholipid syndrome an acquired state, can manifest with symmetrical peripheral digital gangrene following infection or trauma (3,4). Here we describe an unusual case of severe malaria that resulted in symmetrical peripheral digital gangrene and highlight the management issues that arise after the acute illness has resolved.…”

Phenylephrine-Induced Microvascular Occlusion Syndrome in a Patient With a Heterozygous Factor V Leiden Mutation

“…Symmetrical peripheral gangrene, which has been described by some authors as a form of purpura fulminans, is considered a severe form of MOS and is most commonly associated with septic shock and disseminated intravascular coagulation (DIC). [7][8][9][10][11][12] Patients with preexisting vascular disorders, such as peripheral vascular disease, diabetes mellitus, Raynaud phenomenon, and prior cold injury, as well as patients who have been exposed to vasoactive medications, are further predisposed to develop SPG. [11][12][13] Molos and Hall's 8 review of 71 cases of SPG revealed that 48% of the patients required amputation and 35% cases proved fatal.…”

“…Few cases of cutaneous MOS have been reported in patients who are heterozygous for FVL. Patel et al 7 described an 81-year-old woman with heterozygous FVL who developed extensive acral cutaneous necrosis with no clear precipitant, resulting in septicemia and death. Jackson and Luplow 15 reported 2 cases of heterozygous FVL that were complicated by sepsis and DIC, both of which resulted in digital amputations.…”