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|Year : 2014 | Volume
| Issue : 2 | Page : 198-199
Acute infectious purpura fulminans due to probable spotted fever
A Kundavaram1, NR Francis1, AP J Jude2, GN Varghese1
1 Department of Medicine, Christian Medical College, Vellore, Tamilnadu, India
2 Department of Microbiology, Christian Medical College, Vellore, Tamilnadu, India
|Date of Submission||13-Jun-2013|
|Date of Decision||17-Sep-2013|
|Date of Acceptance||30-Dec-2013|
|Date of Web Publication||13-May-2014|
Dr. A Kundavaram
Department of Medicine, Christian Medical College, Vellore, Tamilnadu
Source of Support: None, Conflict of Interest: None
Purpura fulminans (PF) is associated with several infections, most notably with meningococcus, staphylococcus, and streptococcus infections. However, there are few reports of association of this entity with spotted fever from India. We report the case of a 55-year-old man who presented with fever, headache, and myalgia. On the seventh day of fever he developed nonblanching purple hemorrhagic purpura on the trunk and most prominently on the extremities consistent with purpura fulminans. Immunofluorescent assay confirmed the diagnosis of spotted fever. PF though common with rocky mountain spotted fever (RMSF) is rarely seen in association with Indian tick typhus, the usual cause of spotted fever in India.
Purpura fulminans, rickettsia, spotted fever
|How to cite this article:|
Kundavaram A, Francis N R, Jude AJ, Varghese G N. Acute infectious purpura fulminans due to probable spotted fever. J Postgrad Med 2014;60:198-9
| :: Introduction|| |
Purpura fulminans (PF) is a rare syndrome of intravascular thrombosis and infarction of the skin resulting in haemorrhagic purpura that is often accompanied by vascular collapse and disseminated intravascular coagulation. PF is usually described with meningococcemia, staphylococcal sepsis, and in RMSF. Rickettsia conorii, the Indian tick typhus, causing spotted fever group (SFG) rickettsiosis in India infects monocytes, macrophages, and endothelial cells resulting in vasculitis. The rash due to spotted fever starts between the third and fifth day of illness and usually consists of pink macules and then evolve into nonpruritic papules that spread centripetally from the extremities. In severe cases vascular damage can result in purpuric or petechial rash that can progress to purpura fulminans and is generally indicative of bad prognosis. However, PF due to SFG rickettsiosis is rarely reported in the literature. , We report a case of spotted fever that presented with purpura fulminans due to disseminated vasculitis and successfully treated with doxycycline.
| :: Case Report|| |
A 55-year-old man with no premorbid illnesses presented with history of high-grade intermittent fever, headache, and myalgia since 14 days. Seven days after the onset of fever he developed an erythematous haemorrhagic rash over both the legs and hands that rapidly spread to the trunk over 24 h. On examination he was febrile (temperature-101°F) with a pulse rate of 120/min, blood pressure of 110/70 mm Hg, and a respiratory rate of 22/min. He was noted to have pallor and icterus. An extensive purpuric rash with areas of hemorrhage was seen over both the legs, hands, and the trunk [Figure 1]. Rest of the systemic examination was normal. Complete blood count showed a total WBC count of 9.5 × 109/ l (83% neutrophils, 12% lymphocytes, and 5% monocytes), hemoglobin of 8.7 g/dL and platelet count of 304 × 109/l. Prothrombin time (PT) and activated partial thromboplastin time (aPTT) were normal. Liver function tests and renal function tests were normal. Protein C, Protein S, and antithrombin III levels were normal. Blood cultures were sterile. The acute serum had a spotted fever IgM titer of 1:16 and the convalescent serum sent 17 days later showed IgM titer of 1:64. The rickettsial IgM antibodies were detected by immunofluorescence assay (IFA) using the Rickettsial Screen (Fuller Laboratories, Fullerton, CA, USA). Purified Rickettsia rickettsii and Rickettsia typhi were used as antigens. Serology for scrub typhus was negative. Our patient was treated successfully with doxycycline for 1 week and was afebrile within 48 h. Antipyretics, antiallergic drugs and topical emollients were also applied. He recovered completely including regression of the rash on follow-up after 3 weeks.
|Figure 1: Extensive purpuric rash with areas of hemorrhage over the legs|
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| :: Discussion|| |
Spotted fever in India is considered to mostly due to Indian Tick Typhus or Rickettsia conorii, an obligate intracellular organism transmitted by ticks to humans. It has previously been reported from Tamil Nadu, Maharashtra, Himachal Pradesh, and Haryana. It clinically manifests with an acute onset of fever, headache and myalgia. Other symptoms can include malaise, anorexia, nausea, vomiting, abdominal pain, diarrhea, and photophobia. The typical rash seen in 50-90% of cases is maculopapular and spreads centripetally from the extremities. The rash usually persists for 10-20 days after remission of the clinical symptoms. A pathognomonic eschar is seen in a variable proportion (10-92%) of patients with rickettsial infections.  Our patient had an extensive hemorrhagic rash covering most parts of the trunk and extremities and an eschar could not be identified. Diagnosis of SFG rickettsiosis can be confirmed by rickettsial immunofluorescence assay (IFA) which is considered the gold standard test for diagnosis. Enzyme-linked immunosorbent assay (ELISA) IgM test for SFG rickettsiosis is also quite sensitive and specific but does not help in species identification. Western blot immunoassay and polymerase chain reaction (PCR) analysis on a biopsy sample of a pathognomonic eschar can be used for diagnosis and species identification. Doxycycline is the drug of choice for SFG rickettsiosis, the other drugs commonly used being chloramphenicol, macrolides, and rifampicin. Antibiotic susceptibility does not vary much among the different species and hence exact species identification is not essential.
PF is a hemorrhagic condition due to tissue necrosis, small vessel thrombosis and disseminated intravascular coagulation. The three types of PF are: 1. Neonatal PF due to congenital deficiency of protein S, protein C, or anti-thrombin III; 2. Idiopathic PF that occurs after a bacterial or viral infection with a variable latent phase. 3. Acute infectious PF is the most common type and is usually seen with meningococcus, staphylococcus, streptococcus, and with certain rickettsial infections.  Usually PF is synonymous with severe meningococcemia, which is relatively rare. SFG rickettsial infections are more common and benign and hence under reported.
The main pathogenesis of PF in SFG rickettsial infections is focal or disseminated vasculitis causing an infiltration of acute inflammatory cells and thrombus formation in small blood vessels. Massive skin necrosis with DIC requiring surgical intervention has been reported with RMSF, the most severe of the spotted fever rickettsiosis.  A fatal case of Rickettsia conorii subsp. Israelensis infection with purpura fulminans was reported by Weinberger et al.  PF without DIC has also been reported in a patient after a varicella infection. 
Our patient was confirmed to have acute infectious PF probably due to SFG rickettsiosis and showed a dramatic response to doxycycline within 48 h. This case highlights the presentation of presumed Indian tick typhus with PF and should be considered in the etiological workup of any patient who presents with a severe purpuric and hemorrhagic rash.
| :: References|| |
|1.||McBride WJ, Hanson JP, Miller R, Wenck D. Severe spotted fever group rickettsiosis, Australia. Emerg Infect Dis 2007;13:1742-4. |
|2.||Lalitha AV, Aruna D, Prakash A, Nanjunda S, Subbarao SD. Spectrum of Purpura Fulminans. Indian J Pediatr 2009;76:87-9. |
|3.||Kim DM, Won KJ, Park CY, Yu KD, Kim HS, Yang TY, et al. Distribution of eschars on the body of scrub typhus patients: A prospective study. Am J Trop Med Hyg 2007;76:806-9. |
|4.||Griffith GL, Luce EA. Massive skin necrosis in Rocky Mountain spotted fever. South Med J 1978;71:1337-40. |
|5.||Weinberger M, Keysary A, Sandbank J, Zaidenstein R, Itzhaki A, Strenger C, et al. Fatal Rickettsia conorii subsp. israelensis Infection, Israel. Emerg Infect Dis 2008;14:821-4. |
|6.||Sharma VK, Dubey TN, Dave L, Agarwal A. Post varicella purpura fulminans with no evidence of disseminated intravascular coagulation (DIC) or protein S deficiency. J Indian Med Assoc 2010;108:529-30. |
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