Assessment of the Interplay between Blood and Skin Vascular Abnormalities in Adult Purpura Fulminans

Abstract

Thrombi and extensive vascular damage with multifaceted prothrombotic local imbalance are characteristics of purpura fulminans. A "vascular wall infection" hypothesis, responsible for endothelial damage and subsequent skin lesions, can be put forward.

Purpura Fulminans in an adult with Pseudomonas aeruginosa septicemia

“…Acute sepsis secondary to certain organisms, such as Neisseria meningitis, Streptococcus pneumonia, and Haemophilus influenzae can also precipitate purpura fulminans, most commonly in children [1]. The pathogenesis of acute infectious purpura fulminans is related to the systemic activation of coagulation and complement pathways, which commonly accompanies sepsis and DIC [2]. In adults, infectious PF is most commonly caused by sepsis from similar organisms as children.…”

“…[1]. Skin biopsy can be performed to support the diagnosis, and would include the presence of thrombi in dermal vessels, extensive intravascular platelet aggregates, and massive vascular congestion [2]. However, empiric treatment should be started without delay.…”

“…It most commonly occurs in infants and children in association with a heritable deficiency of protein C or protein S and/ or an acquired deficiency secondary to an infection with N. meningitis or S. pneumoniae [1]. Purpura fulminans in an adult with Pseudomonal sepsis is not typical [2][3][4].…”

“…Typical laboratory values include prolonged plasma clotting times, thrombocytopenia, and markedly reduced Protein C and Protein S levels [1]. Biopsy can be performed to confirm diagnosis, but empiric treatment should be initiated while waiting for the results [2]. Standard treatment of purpura fulminans includes antibiotic therapy for any underlying infection, surgical debridement to remove necrotic tissue, and correction of the various coagulation cascade abnormalities characteristic of the disease [1,5].…”

Purpura on the truncus and extremities

“…PF is a rare, fatal syndrome accompanied by skin necrosis and disseminated intravascular coagulation, caused by protein C-deficiency or overwhelming sepsis,1 typically caused by Neisseria meningitidis and S. pneumoniae 2. This patient had hyposplenia, a risk factor for invasive N. meningitidis and S. pneumoniae infection 3.…”