Empiric treatment of protracted idiopathic purpura fulminans in an infant: a case report and review of the literatureReportar como inadecuado

Empiric treatment of protracted idiopathic purpura fulminans in an infant: a case report and review of the literature - Descarga este documento en PDF. Documentación en PDF para descargar gratis. Disponible también para leer online.

Journal of Medical Case Reports

, 5:201

First Online: 23 May 2011Received: 06 October 2010Accepted: 23 May 2011DOI: 10.1186-1752-1947-5-201

Cite this article as: Macheret, F., Pundi, K.N., Broomall, E.M. et al. J Med Case Reports 2011 5: 201. doi:10.1186-1752-1947-5-201


IntroductionIdiopathic purpura fulminans is a cutaneous thrombotic disorder usually caused by autoimmune-mediated protein C or S deficiency. This disorder typically presents with purpura and petechiae that eventually slowly or rapidly coalesce into extensive, necrotic eschars on the extremities. We present the first known case of idiopathic purpura fulminans consistent with prior clinical presentations in the setting of a prothrombotic genetic mutation, but without hallmark biochemical evidence of protein C or protein S deficiency. Another novel feature of our patient-s presentation is that discontinuation of anti-coagulation has invariably led to recurrence and formation of new lesions, which is unexpected in idiopathic purpura fulminans because clearance of autoimmune factors should be followed by restoration of anti-coagulant function. Although this disease is rare, infants with suspected idiopathic purpura fulminans should be rapidly diagnosed and immediately anti-coagulated to prevent adverse catastrophic outcomes such as amputation and significant developmental delay.

Case presentationA six-month-old Caucasian boy was brought to our pediatric hospital service with a low-grade fever and subacute, symmetric, serpiginous, stellate, necrotic eschars on his forearms, legs and feet that eventually spread non-contiguously to his toes, thighs and buttocks. In contrast to his impressive clinical presentation, his serologic evaluation was normal, and he was not responsive to corticosteroids and antibiotics. Full-thickness skin biopsies revealed dermal vessel thrombosis, leading to a diagnosis of idiopathic purpura fulminans and successful treatment with low-molecular-weight heparin, which was transitioned to warfarin. Long-term management has included chronic anti-coagulation because of recurrence of lesions with discontinuation of treatment.

ConclusionIn infants with necrotic eschars, it is important to first consider infectious, inflammatory and hematologic etiologies. In the absence of etiology for protracted idiopathic purpura fulminans, management should include tissue biopsy, in which thrombotic findings warrant a trial of empiric anti-coagulation. Some infants, including our patient, may need long-term anti-coagulation, especially when the underlying etiology of coagulation remains unidentified and symptoms recur when treatment is halted. Given that our patient still requires anti-coagulation, he may have a yet to be identified autoimmune-mediated mechanism for his truly idiopathic case of protracted purpura fulminans.

AbbreviationsACEangiotensin-converting enzyme

ANAanti-nuclear antibody

aPTTactivated partial thromboplastin time

ASDatrial septal defect

AST-ALTalanine-aspartate transaminase

AT IIIanti-thrombin III

BUNblood urea nitrogen


CRPC-reactive protein

DRVVTdilute Russell-s viper venom test


ESRerythrocyte sedimentation rate

HSPHenoch-Schonlein purpura

PDApatent ductus arteriosus

POper os by mouth


RBC ALAred blood cell Δ-aminolevulinic acid


Electronic supplementary materialThe online version of this article doi:10.1186-1752-1947-5-201 contains supplementary material, which is available to authorized users.

Download fulltext PDF

Autor: Fima Macheret - Kavitha N Pundi - Eileen M Broomall - Dawn M Davis - Vilmarie Rodriguez - Chad K Brands

Fuente: https://link.springer.com/

Documentos relacionados