World Rural Health Conference
Home Print this page Email this page Small font size Default font size Increase font size
Users Online: 1474
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents 
CASE REPORT
Year : 2013  |  Volume : 2  |  Issue : 4  |  Page : 393-395  

Purpura fulminans associated with hemiparesis following measles infection in a Nigerian girl: A case report


Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria

Date of Web Publication31-Dec-2013

Correspondence Address:
Aliyu Ibrahim
Department of Paediatrics, Aminu Kano Teaching Hospital, +234 Kano - 700 001
Nigeria
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2249-4863.123938

Rights and Permissions
  Abstract 

Purpura fulminans is a life-threatening hematologic emergency characterized by extensive skin necrosis with hemorrhagic infarction, hypotension and gangrene which may arise from severe sepsis; mostly gram negative sepsis though also associated with some gram positive organisms, similarly viral infections like varicella and measles have been implicated; it may also arise from congenital deficiency of the anticoagulants protein C, S, and antithrombin III; and it may also be idiopathic. Its clinical manifestation may be quite extensive resulting in multiorgan failure. Treatment will require aggressive management with use of fresh frozen plasma, heparin, antibiotics, and surgical debridement of necrotic tissue.

Keywords: Hemiparesis, honey dressing, measles, purpura fulminans


How to cite this article:
Ibrahim A. Purpura fulminans associated with hemiparesis following measles infection in a Nigerian girl: A case report. J Family Med Prim Care 2013;2:393-5

How to cite this URL:
Ibrahim A. Purpura fulminans associated with hemiparesis following measles infection in a Nigerian girl: A case report. J Family Med Prim Care [serial online] 2013 [cited 2019 Jul 23];2:393-5. Available from: http://www.jfmpc.com/text.asp?2013/2/4/393/123938


  Introduction Top


Purpura fulminans was first described by Guelliot in 1884 [1] ever since several cases have been reported. Purpura means bleeding into the skin or mucous membrane; it is classified into petechiae (less 2 mm) and ecchymoses based on the size of the lesion. [2] Purpura may be palpable or nonpalpable and do not blanch to pressure. However, purpura fulminans is a life-threatening hematologic emergency characterized by hemorrhagic infarction of the skin and/or multiple organs with extensive tissue necrosis which may result in gangrene, disseminated intravascular coagulopathy, hypotension and multiple organ failure. [3]

Acute infectious purpura fulminans may complicate sepsis associated with meningococcamia, [4] Streptococcus pneumoniae, [5] Klebsiella pneumoniae, [6] and viral infection like varicella; however its association with measles though previously reported still remains a rare event. [7],[8] Therefore, a case of a 3-year-old girl who had measles with subsequent purpura fulminans and an associated hemiparesis of the right upper and lower limbs is reported.


  Case Report Top


A 3-year-old girl presented with darkened and swollen right lower limb and in ability to utilize the right side of the body following febrile illness with associated generalized body rash which started from the face and progressed to the whole body; this was preceded with complaint of coryza; she also had bilateral reddish eye discharge; this occurred during the 2013 period of measles outbreak in her community and she was not immunized for measles. A week after skin desquamation the mother noticed darkened and swollen right foot which was painful, progressive, and by 24 h it had involved up to the ankle; the toes then became blackened [Figure 1]. The patient had several episodes of generalized tonic-clonic seizures. At about same time after seizure control, mother noticed she could not use the right upper and lower limbs [Figure 2]. She was transferred to our hospital after initial management in a general hospital. On examination, she was not febrile; the cardiac and respiratory examinations were not remarkable. All peripheral pulses were palpable except for the right posterior tibial and dorsalis pedis arteries. At that point, she had regained consciousness, the right upper and lower limbs had reduced power (estimated power were 3/5 in both limbs); the left limbs were normal, there were increased tone in both right upper and lower limbs; and the right foot had areas of darkened and shrunken toes which were not tender with proximal areas of tender swollen purpuric skin. She had deranged clotting profile, that is, activated partial thromboplastin time (aPTT) of 90 s (aPTT = 60-85 s) and prothrombin time (PT) of 28 s (PT = 11-15 s) and thrombocytopenia of 90,000/mm 3 (150,000-400,000/mm 3 ) results from referring hospital; but the repeat in our hospital were normal, and there was no site of active bleeding. Magnetic resonance imaging (MRI) of the brain was normal [Figure 3], similarly the echocardiograph was normal but the vascular Doppler study confirmed reduced flow in the right posterior tibial artery. Neither fresh frozen plasma infusion nor platelets transfusion was given at the referring hospital. The diagnosis was measles encephalitis with purpura fulminans. She was billed for transtibial amputation of the right lower limbs, but parents declined amputation despite adequate counseling that the grandparents refused amputation. She had honey dressing of the affect limb for 2-weeks with some good effect [Figure 4] before they opted to leave against medical advice.
Figure 1: Gangrenous right foot

Click here to view
Figure 2: Hemiparesis of the right upper and lower limbs

Click here to view
Figure 3: Normal brain magnetic resonance imaging (MRI)

Click here to view
Figure 4: Regression of right foot swelling on 2nd week of honey dressing

Click here to view



  Discussion Top


Purpura fulminans occurs following an imbalance in the procoagulants and anticoagulants resulting in heightened risk of vascular thrombosis and bleeding. It has been classified into neonatal purpura fulminans, which is due to homozygous deficiencies of protein C; acute infectious purpura fulminans, which could be due to deficiencies of protein S, antithrombin III, and in heterozygous deficiency of protein C; and idiopathic purpura fulminans, which is due to autoantibodies against protein S. [9],[10] Acute infectious purpura fulminans involves extensive vascular injury from endotoxins, exotoxins, resulting in microthrombi formation with bleeding due to consumption coagulopathy; the extent of damage may be enormous affecting the skin, the lungs, Kidneys and brain resulting in multiorgan failure; which explains the gangrene and hemiparesis noticed in this case. Usually the limbs especially the lower limbs are mostly affected in a symmetrical distribution in infectious purpura fulminans, but this case had a single limb involvement which coincided with same side of hemiparesis; why this is so is not clear. Hemiplegia is a documented complication of some viral infections such as herpes zoster opthalmicus and varicella; this usually occur weeks following the infection as a result of vasculitis; whether the same mechanism is operational in measles is not clear.

Measles as an etiologic agent in purpura fulminans is poorly documented; [8] therefore this could pose a diagnostic challenge because body rash, fever, and coryza may also be associated with bacterial sepsis like meningococcemia. Diagnosis of measles is usually clinical; the rash in this case was typical of measles in its distribution and the blood culture was negative; furthermore this occurred during the outbreak of measles in Kano which was way before known period of  Neisseria More Details meningitidis outbreaks. Furthermore, the gangrene and hemiparesis occurred after the resolution of fever; which is not the typical picture in septicemic illness.

Treatment of acute infectious purpura fulminans involves: Replacing the deficient hemostatic factors which restores the balance between the procoagulants and the anticoagulant factors; identifying and treating the cause. Therefore fresh frozen plasma corrects deficient clotting factors and use of heparin prevents further thrombi formation; in our case none of these were given; probably due to difficulty in securing them at the referring hospital; the decision not to commence them in our institution was based on her clinical improvement; there were no new lesions, no further extension of the purpura or gangrene, rather some of the skin lesions resolved and she remained afebrile; this further heightens the controversy surrounding the use of these medications in purpura fulminans. Surgical debridement of necrotic tissue remains a standard of care; parents declined amputation despite adequate counseling, however the patient was commenced on honey dressing with good effect; sloughing was noticed by the 2 nd week of treatment.


  Conclusion Top


Purpura fulminans is a life-threatening hematologic emergency associated mostly with gram negative septicemia which requires aggressive management; however we have reported a case which was associated with hemiparesis following measles infection.

 
  References Top

1.Nolan J, Sinclair R. Review of management of purpura fulminans and report of two cases. Br J Anaesth 2001;86:581-6.  Back to cited text no. 1
[PUBMED]    
2.Peck SM, Rosenthal N, Lowell ER. Purpura classification and treatment, with special reference to treatment with snake venom. Arch Derm Syphilol 1937;35:831-67.  Back to cited text no. 2
    
3.Adcock DM, Brozna J, Marlar RA. Proposed classification and pathologic mechanisms of purpura fulminans and skin necrosis. Semin Thromb Hemost 1990;16:333-40.  Back to cited text no. 3
[PUBMED]    
4.Darmstadt GL. Acute infectious purpura fulminans: Pathogenesis and medical management. Pediatr Dermatol 1998;15:169-83.  Back to cited text no. 4
[PUBMED]    
5.Ward KM, Celebi JT, Gmyrek R, Grossman ME. Acute infectious purpura fulminans associated with asplenism or hyposplenism. J Am Acad Dermatol 2002;47:493-6.  Back to cited text no. 5
[PUBMED]    
6.Kalra OP, Agarwal, Khaira SA. Acute infectious purpura fulminans due to klebsiella pneumoniae: A case report. JIACM 2003;4:156-8.  Back to cited text no. 6
    
7.Lokeshwar MR, Singhal T, Udani SV, Kapadia FN. Treatment of acute infectious purpura fulminans with activated protein C. Indian Pediatr 2006;43:535-8.  Back to cited text no. 7
[PUBMED]    
8.Kelly EH. Case of purpura fulminans following measles. Proc R Soc Med. 1922; 15(Sect Study Dis Child): 45-46.  Back to cited text no. 8
    
9.Auletta MJ, Headington JT. Purpura fulminans. A cutaneous manifestation of severe protein C deficiency. Arch Dermatol 1988;124:1387-91.  Back to cited text no. 9
[PUBMED]    
10.Marlar RA, Neumann A. Neonatal purpura fulminans due to homozygous protein C or protein S deficiencies. Semin Thromb Hemost 1990;16:299-309.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

Top
   
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed1624    
    Printed46    
    Emailed0    
    PDF Downloaded153    
    Comments [Add]    

Recommend this journal