Purpura Fulminans: An Ominous Sign of Disseminated Intravascular and Rishi Sharma,* Imran Ahmed, DO,* Andleeb A. Usmani, DO**

*Medical Student, 3rd year, Kansas City University College of Osteopathic Medicine, Kansas City, MO **Dermatologist, Wellington Medical Center, Wellington, FL

Disclosures: None Correspondence: Rishi Sharma; [email protected]

Abstract fulminans is a rare manifestation of disseminated intravascular coagulation (DIC) and may be secondary to sepsis. While the skin manifestations may not be the most emergent cause of death in septic patients – cause of death is typically shock due to decreased vascular resistance caused by endovascular toxins and from DIC – it is important to recognize this physical exam finding when parameters such as temperature, heart rate, and blood pressure are not readily available. We present a case of an assisted-living patient with to illustrate the importance of recognizing purpuric skin lesions that could be consistent with PF.

Case Report The patient was kept in the critical care unit for The patient’s vital signs were the indication ofan An 86-year-old female was brought by ambulance monitoring of hemodynamic stability, cell counts, emergent underlying cause. Hypothermia may be from an assisted living facility with complaints and administration of . Due to the present in sepsis, especially if the temperature is of altered mental status and weakness. The inability to maintain adequate respiratory drive, less than 95 °F. In addition, an elevated heart rate nursing staff at the facility had promptly called an the patient required immediate intubation. The and low blood pressure are signs of shock. Altered ambulance due to her symptoms. En route to the following day, head imaging demonstrated hypoxic mental status is one of the most important and easily emergency room, she was noted to have a blood brain injury, which could have been due to both her overlooked signs of or other disruption pressure of 90/62 and a rectal temperature of 94 septic shock and respiratory distress. Several days to the patient’s homeostasis. The presence of these degrees Fahrenheit. Upon arrival to the ER, a sepsis later, given the patient’s lack of brainstem reflexes findings around the same time as a new eruption alert was called. and failure to improve, a diagnosis of brain death of should raise concerns and calls for prompt was made by two separate intensivists, and it was transfer to an emergency department. The nursing facility was called for further history. agreed to cease treatment. Gram-negative sepsis is the most common cause They stated that the patient was having unusual 4 skin findings for about three weeks, including a Discussion of disseminated intravascular coagulation. The red, dry skin rash that started remotely to the chest A new finding of unexplained purpura, coagulation is caused by an inciting factor that and spread outward to the extremities over the especially in an older patient who may be damages the vasculature, such as next two weeks. More recently, she had developed immunocompromised, should be a red flag due on the surface of the bacterial lipid membrane. This to the possibility of a more emergent underlying damage results in quick consumption of coagulation darker skin lesions that were scattered on her hands 1 and legs. For the rash, a consultation cause. In the case presented here, the lesions factors, resulting in an increased risk for bleeding. was requested, but it was not completed before were non-blanching and nonpalpable with The perpetuation of coagulation by the offending her presentation to our hospital. A steroidal cream scattered dried petechiae, which is suggestive of agent results in increased production of thrombi was used by staff while awaiting dermatology a cause other than . The differential may consisting of fibrin and , which may occur consultation. Due to her unstable vital signs and include N. meningitidis, -induced skin in both large vessels and microvasculature. Examples the risk of possible sepsis, the patient was admitted , trauma resulting in ecchymosis, sepsis, of large-vessel involvement include pulmonary to the critical care unit. thrombotic , cellulitis, embolism and thrombotic cerebral stroke, two causes gangrenosum, and disseminated of death in this setting. Microvascular involvement 2,3 Physical exam revealed multiple dark, purpuric, intravascular coagulation. was demonstrated in our case, with necrotic skin non-blanching lesions scattered across the lower and upper distal extremities, worse behind both knees and calves. Behind the knees, the purpuric lesions coalesced, and sloughing with underlying weeping of serosanguinous fluid was appreciated. The lesions were mildly painful to palpation. In addition to the purpuric lesions, there were unrelated dry, excoriated, flaky and erythematous lesions visualized across the chest and proximal upper extremities.

The patient’s white blood cell count was (21.8 x 103)/mm3 with 78% segmented neutrophils, hemoglobin 13.5, hematocrit 42.3, and count 170. Chemistry showed: sodium 141 mEq/L, potassium 4.9 mEq/L, chloride 108 mEq/L, bicarb 24 mEq/L, BUN 46 mEq/L, creatinine 2.08 mg/dL, glucose 100, calcium 8.3 mg/dL, total bilirubin 1 mg/dL, AST 65 U/L, and ALT 40 U/L. Total alkaline phosphatase was 177 U/L, albumin was 1.7 g/dL, and total protein was 6.2 g/dL. A coagulation panel showed: INR 1.7, PT 13.5 seconds, and PTT 35 seconds. A urine panel showed: cloudy urine with protein, positive blood, positive leukocyte esterase, 10 to 12 red Figure 1 Figure 2 blood cells, and 25 to 50 white blood cells, with a urine culture growing E. Coli. D-Dimer was 2585. Figures 1, 2. Scattered, widespread, non-blanching purpuric lesions to the distal lower extremities. Under the calves, the skin sloughs to moderate horizontal tension with underlying serosanguinous fluid extravasation.

PURPURA FULMINANS: AN OMINOUS SIGN OF DISSEMINATED INTRAVASCULAR COAGULATION AND SEPSIS lesions resulting in a nonpalpable, non-blanching, References widespread purpura. This necrosis may result in 1. Levi M, Ten Cate H. Disseminated intravascular sloughing of the skin with subsequent weeping of coagulation. N Engl J Med. 1999;341:586. sanguineous fluid due to the increased susceptibility to bleeding. In addition, our patient had new and old 2. Bauer KA. Coumarin-induced skin necrosis. areas of small petechiae, further demonstrating the Arch Dermatol. 1993;129:766. clinical picture of DIC. This points to an infectious etiology for the patient’s purpuric lesions. Infectious 3. Bovill EG, Bauer KA, Dickerman JD, et al. purpura fulminans is more commonly encountered 5 The clinical spectrum of heterozygous in the adult population. deficiency in a large New England kindred. Blood. 1989;73:712-7. There are several subtypes of purpura fulminans other than infectious. Neonatal purpura fulminans 4. Levi M. Current understanding of disseminated is a rare, life-threatening disease caused by either intravascular coagulation. Br J Haematol. a congenital or an acquired deficiency of protein 2004;124:567. C or .6 The activated form of protein C, an antithrombotic protein derived from the 5. Harikrishna J, Mohan A. Infectious purpura liver, inactivates factors V and VIII, preventing fulminans. Indian J Med Res. 2015;141(1):130-1. excessive formation in the coagulation cascade. In the event of an acquired or congenital 6. Jafarri S, AlAttas K, Bajawi S, et al. Neonatal , factors V and VIII are free purpura fulminans in newborn with severe to perpetuate thrombus formation, leading to congenital protein C deficiency: Case report. J micro- and macrovascular thromboses. Clinically, Dermatol Dermatolog Surg. 2017; 21:2 Pages 104- a neonate with congenital protein C deficiency 106 may develop multiple well-defined, dark-purple to blackish patches involving random areas of the 7. Bektas F, Soyuncu S. Idiopathic purpura skin and extremities as soon as a few hours after fulminans. Am J Emerg Med. 2011;29(4):475. birth. An acquired protein C deficiency may occur as a manifestation of sepsis, infection from gram- negative organisms and species.

Cases in which the etiology of the purpuric lesions is not clearly demonstrated may be termed “idiopathic purpura fulminans.” While not many cases of idiopathic purpura fulminans have been described in the literature, it reportedly presents with well- demarcated, purple to black patches anywhere on the skin in patients demonstrating relatively normal lab values, including normal prothrombin time, activated prothrombin time, protein C, protein S, and III levels.7 Usually, idiopathic purpura fulminans affects the breasts or lower limbs, and it may arise up to 10 days after an antecedent infection, such as meningococcemia, Haemophilus, , or varicella. Conclusion The skin lesions found in purpura fulminans may be the result of microvascular resulting in skin necrosis. It is a rare skin manifestation secondary to multiple causes, including disseminated intravascular coagulation, sepsis, warfarin skin necrosis in a setting of acquired protein C deficiency, -induced , and thrombotic thrombocytopenic purpura. A new finding of dark, purpuric lesions in a patient demonstrating no identifiable trauma or other immediate cause calls for prompt evaluation by a health professional. Quickly attainable vital signs such as blood pressure, core temperature, heart rate, and an assessment of mental-status changes compared to baseline are helpful in identifying not only the severity of the skin findings but also the cause. Lab work demonstrating decreases in platelets, coagulation factors and hemoglobin, along with an increased D-Dimer, should prompt one to think DIC. A skin biopsy is helpful in ruling out vasculitides if the cause of the purpura is still uncertain. Appropriate management, including IV antibiotics, fluids, and adrenergics to treat a possible underlying sepsis, should be promptly initiated.

SHARMA, AHMED, USMANI