European Journal of Cardiovascular Medicine

Purpura fulminans (PF) is a life-threatening disorder of coagulation clinically characterized by acute onset, rapidly progressive haemorrhagic skin infarction and necrosis, accompanied by vascular collapse and disseminated intravascular coagulation1. The diagnosis is made by laboratory evidence of consumptive coagulopathy and histopathological evidence of widespread thrombosis1,2. The condition is fatal (up to 50% mortality) and survivors may suffer loss of digits, limbs or areas of skin. We report 3 cases of this rare condition, 1child and 2 adults which are of different causes. One case was related to non-inflammatory (intravascular) causes other 2 causes were related to inflammatory (infectious) causes of purpura fulminans.

Case 1

An 8-year-old female from a tribal area, came with complaints of fever for 10 days, rashes associated with pain and itching for 7 days, which started initially over left leg and rapidly progressed to involve both lower limbs, upper limbs and turned to bluish-black discoloration over time. A provisional diagnosis of Acute infectious purpura fulminans secondary to rickettsial infection was made and treated with IV antibiotics for 10 days and Oral Doxycycline 50mg twice daily for 7 days and oral pentoxyphylline 200mg TID. 

Case 2

20-year-old female, para-2 living -2, 8 days postpartum after normal vaginal delivery presented with fever, loose stools and cough for 3 days, bluish discoloration of skin associated with pain and intense itching for 2 days, initially started over both ankle and rapidly progressed to involve both upper and lower limbs, abdomen and face. Patient was discharged after 45 days with deep scaring over preexisting lesions.

Case 3

A 45-year-old male, presented with chief complaints of red raised lesions associated with burning sensation over both legs for 10 days, lesions started on both feet which rapidly progressed to involve both legs, thighs, abdomen, arms and back over 3-4 days, older lesions healed with dusky brown pigmentation over a period of 4-5 days. A provisional diagnosis of Purpura fulminans with polycythaemia vera was made. Patient was referred to higher centre and lost for follow up.

Purpura fulminans (PF) is a fatal, heterogenous group of disorders clinically characterized by rapidly progressive purpuric lesions which evolve into extensive areas of cutaneous necrosis and peripheral gangrene1,2.Purpura fulminans can be classified (table 1) as Acute infectious, Idiopathic or Post infectious, Inherited (neonatal PF) or Acquired disorders of coagulation and Others 1,2, where the principle pathos-mechanism is deficiency of in vivo anticoagulants, which may be congenital or acquired (fig 8). Neonatal PF occurs due to homozygous or compound heterozygous protein C gene mutation in chromosome 23, resulting in deficiency of protein C, protein S and/or antithrombin III, clinically presents with purpuric lesions mainly over perineal region, inner thighs and abdomen within 72 hours of birth2.

Idiopathic/ post infectious PF occurs 7-10 days after a bacterial or viral infection (varicella/ streptococcal infections), commonly occurring in children. The pathogenesis involves acute transient autoimmune mediated deficiency of natural anticoagulants which occurs in convalescence period of an infectious episode.

Acute infectious PF is most commonly secondary to bacterial infections occurring in the period of overwhelming infection. The pathogenesis is imbalance in anticoagulant and procoagulant endothelial activity, precipitated by bacterial endotoxin which consumes antithrombin III as well as proteins C and S resulting in state of consumptive coagulopathy. In our first case report the cause is rickettsial infection, showed Weil Felix test- OX 19- positive and OX2 OXK- negative (indicates rickettsia prowazekii, typhi or conorii infection usually louse/flea/tick borne typhus1) similar to case reports by katoch et al1, Budamakuntla et al4. The Weil Felix test is usually first diagnostic tool used in developing countries, it has a sensitivity of 33% and specificity of 46%, can be used as reliable screening test. It is a heterophile antibody test, demonstrates agglutinins to Proteus vulgaris strain OX 19, OX 2 and OX K, a fourfold rise in the agglutinin titre is considered diagnostic1,4. Doxycycline is the drug of choice for rickettsiosis, the other drugs commonly used are chloramphenicol, macrolides, and rifampicin. Hence a high index of suspicion and prompt initiation of therapy are keys to favourable outcomes in cases of rickettsia associated purpura fulminans5.

Our second case was acute infectious PF secondary to puerperal sepsis, clinically purpuric rash as well as symptoms and signs of sepsis is mandatory in sepsis induced PF. It has been reported that purpura fulminans was associated with septic shock in 67% of patients and in 78% of patients with DIC6. The causative agent was Escherichia coli isolated from both blood and vaginal swab culture, similar cases with purpura fulminans in postpartum was reported by Viderman D et al6 and Jain Set al7, the causative agents were mixed infection (multi drug-resistant Escherichia coli, Pseudomonas aeruginosa, and Acinetobacter baumannii) and E coli infection respectively, though the mortality is high (up to 50%) patients survived with deep scaring in both the reports similar to our case. Early diagnosis, prompt management of septic shock with fluid resuscitation, inotropic therapy, respiratory support, anticoagulants and treatment of underlying infection is mandatory in favourable outcomes in postpartum sepsis related purpura fulminans.

With respect to case 3, a case of purpura fulminans with polycythaemia vera, the cause of DIC/haemorrhagic diathesis in PRV is varied or not clearly established. It has been suggested that the coagulation defects of PRV may be due to abnormal platelet function but in this patient the coagulation defect was not correlated with the platelet count which was normal in this scenario. It is considered that hyper viscosity in PRV results in small vessel stagnation and DIC with consumption of clotting factors and platelets8. The other reason can be due to idiopathic thrombocytopenic purpura (ITP) which can occur in myeloproliferative conditions similar to polycythaemia vera. Acik DY9 has reported 2 cases of polycythaemia vera with ITP. However, the exact etiology was not known in this patient, the limitations in this scenario was a bone marrow biopsy and other few investigations for ITP as the patient did not give consent for further investigations.

Though the dermatologist role is less in management of purpura fulminans, dermatological signs can be decisive in arriving at the correct diagnosis and treatment (possible infectious origin on the basis of the first presenting symptoms). The problem is to distinguish a PF from a vasculitis with cutaneous involvement and this is not always easy to do- acral involvement, haemorrhagic bullae, a tendency to necrosis and hypovolemic shock are all suggestive of PF. Hence, a high index of suspicion and prompt treatment with multidisciplinary approach always enhance therapeutic outcomes in such fatal condition. We report these 3 cases for its rarity, very few cases has been reported in literature especially puerperal sepsis and polycythaemia vera related PF and hence proves its heterogeneity with common clinical presentation.

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