Streptococcus pneumoniae–induced purpura fulminans in a woman with functional asplenia

Abstract

Purpura fulminans is a rare complication of septic shock, often associated with Streptococcus pneumoniae. Patients with anatomic or functional asplenia are at increased risk for infection because of impairment of their ability to defend against encapsulated pathogens. We report the case of a previously healthy, unimmunized 33-year-old female with functional asplenia who presented in septic shock and purpura fulminans and died in spite of maximal resuscitative measures. The clinical presentation, diagnosis, and management of purpura fulminans are reviewed. Purpura fulminans is a rare condition that requires early diagnosis and aggressive management by emergency physicians. RÉ SUMÉLe purpura fulminant est une complication rare du choc septique, souvent associé à une infection à Streptococcus pneumoniae. Les personnes atteintes d'asplé nie anatomique ou fonctionnelle sont pré disposé es aux infections en raison de l'incapacité de l'organisme de se dé fendre contre les agents encapsulé s. Il sera ici question du cas d'une femme de 33 ans, non vacciné e, auparavant en bonne santé , mais souffrant d'asplé nie fonctionnelle, qui est arrivé e en é tat de choc septique, accompagné de purpura fulminant, et qui est morte malgré toutes les mesures possibles de ré animation. Nous passerons en revue le tableau clinique du purpura fulminant ainsi que le diagnostic et la prise en charge de la maladie; il s'agit d'une affection qui appelle un diagnostic pré coce et une prise en charge é nergique par les urgentologues.

Streptococcus pneumoniae induced purpura fulminans in a patient with splenic hypoplasia

“…Although scintigraphic methods are most reliable for assessing splenic function, they are not the best options for screening large populations [16]. The presence of Howell-Jolly bodies, which are small round bodies representing nuclear remnants within erythrocytes, indicates splenic dysfunction, although these findings may not be seen in those with only mild impairment of splenic function [5,16]. Other abnormalities associated with splenic dysfunction that can be seen on peripheral blood smears are acanthocytes (spur cells), target cells, hemoglobin remnants (Heinz bodies), siderocytes, and iron granulocytes [16].…”

“…The most common causative agents of purpura fulminans are N. meningitides infections, followed by varicella, S. pneumoniae, and measles infections [8]. Reduced splenic function, asplenism, and protein S, or C deficiency can also be risk factors for this condition [2,4,5]. The skin lesions usually start as well-demarcated erythematous macules, which worsen rapidly with hemorrhagic necrosis, followed by the formation of dark lesions with vesicles or bullae.…”

“…Fresh frozen plasma can be used to replete these coagulation factors. Replacement therapy may contribute to arresting the progression of the disease and avoiding amputation of the limbs [5]. Early administration of protein C corrects the deficiency and might contribute to the restoration of peripheral perfusion according to a previous case report [5].…”

“…The differential diagnosis of purpura fulminans includes idiopathic thrombocytopenic purpura, thrombotic thrombocytopenic purpura, Henoch-Schönlein purpura, and warfarin-induced skin necrosis [9]. Usually, only purpura fulminans and warfarin-induced skin necrosis present with necrotic skin lesions [5].…”

“…Common causes include Neisseria meningitidis, Streptococcus pneumoniae (S. pneumoniae), Haemophilus influenzae, Staphylococcus aureus, and fungal or viral infections [2,3]. Purpura fulminans occurs mainly in immunocompromised hosts including those with prior splenectomy, functional asplenia, or other impaired host-immune defense mechanisms [4,5]. It is rare for immunocompetent adults to have this condition.…”