Purpura fulminans (PF) is a life-threatening condition with different etiologies characterized by confluent purpura, evolving rapidly into hemorrhagic infarction of the skin due to disseminated intravascular coagulopathy (DIC) and dermal vascular thrombosis.1
PF of the penis has rarely been reported.2
We describe a patient who, 3 days after stent removal of endoscopic drainage of pancreatic cysts developed an acute pancreatitis and a penile necrosis due to PF that has been misdiagnosed as Fournier's gangrene, rapidly followed by a lethal multiorgan failure due to an extensive DIC.
The patient was a 63-year-old man with a significant medical history of episodes of abdominal pain and chronic pancreatitis for 2 years. He had been under treatment with metformin for diabetes, telmisartan and nebivolol for arterial hypertension, and fentanyl and tramadol hydrochloride for chronic pain. After the detection of pancreatic cysts, he underwent an endoscopic surgery of drainage positioning. One month later, 3 days after stent removal, he developed a penile necrosis. The patient was diagnosed with Fournier's gangrene and was placed on broad-spectrum antibiotic therapy. Despite treatment, he developed extensive skin lesions, acute renal failure, hypovolemic shock, and profound coagulopathy with thrombocytopenia and was brought to the emergency department. Physical examination showed extensive necrosis of the penile shaft and the entire scrotum associated with large, erythematous, violaceous, hemorrhagic patches on the thighs, groin area, hands, and forearms where the dark purple patches began to slough (Fig. 1). Scattered purpuric lesions were present on his trunk. Laboratory data showed, in addition to profound hemostatic abnormalities, marked leukocytosis; however, all blood cultures were negative. In the suspicion of Fournier's gangrene, a surgical debridement of the inguinocrural and perineal region was performed. A skin biopsy was also performed, revealing thrombosis of small vessels and massive epidermal and dermal necrosis, without neutrophilic infiltrates or vasculitis consistent with a diagnosis of PF.
Unfortunately, the patient developed severe multiorgan failure, massive tissue destruction with hypovolemic shock, and quickly died.
To make a definite diagnosis, an autopsy was performed.
Histopathology of the penis confirmed previous findings of occlusive vasculopathy with epidermal necrosis, subepidermal bullae, fibrin thrombi within small and medium cutaneous vessels, and with minimal to absent inflammatory infiltrate in the absence of vasculitis (Fig. 2A). Similar findings were seen for purpuric lesions on the thigh showing thrombi within the lumina with extravasated erythrocytes (Fig. 2B).
Pancreatic samples revealed pancreatolithiasis triggering chronic and acute pancreatitis, with large areas of hemorrhagic necrosis (Fig. 3A). Soft tissues samples from mesentery, peripancreatic region, abdominal wall, and inguinocrural region showed features of pancreatic steatonecrosis and panniculitis (Fig. 3B), with typical necrotic “ghost adipocytes,” characterized by finely granular and basophilic material in the cytoplasm, because of calcium deposits (Fig. 3C). Histopathology of the lung (Fig. 3D), spleen, and kidney confirmed the presence of multiple infarcts with diffuse phenomena of thrombosis in small vessels in the absence of neutrophilic or massive inflammatory infiltrate. Pancultures did not show any evidence of infection.
DIC is a severe disorder characterized by systemic activation of blood coagulation, leading to thrombi formation and bleeding. Many conditions are associated with DIC, and in a retrospective study, the underlying disorders included solid neoplasms (34%), hemo-lymphoproliferative malignancies (13%), aortic aneurysm (11%), infections (6%), postoperative complications (4%), liver disorders (3%), obstetric complications (3%), and others (27%).3
PF is one of the clinical presentations of DIC manifesting as erythematous, violaceous, and hemorrhagic patches that develop blue–black central necrosis.4
Three forms of PF are recognized: acute infectious; neonatal, which results from hereditary deficiency of protein C or S; and idiopathic, which usually follows a febrile illness.5 Histopathology of PF is nonspecific but is recognizable with a good clinicopathologic correlation, showing an occlusive vasculopathy without vasculitis.
Penile necrosis has been previously described in the setting of severe atherosclerosis, diabetes mellitus, and calciphylaxis in end-stage renal disease,6,7 but it is an infrequent clinical presentation of PF.2 The condition is a mimicker of Fournier's gangrene, a potentially fatal condition, characterized by a necrotizing fasciitis due to polymicrobial infection with a rapid and progressive necrosis spreading from subcutaneous fat tissue to fascial planes. Histopathology, however, is totally different from PF showing, in addition to thrombotic vasculopathy and hemorrhages, an extensive necrosis of the skin, soft tissue, and fascia with an inflammatory infiltrate of neutrophils, lymphocytes, and histiocytes. Special stains may highlight microorganisms.8
In our patient, PF, presenting as acute penile necrosis, was the initial manifestation of a systemic coagulopathy that leads to a lethal multiorgan thrombotic failure with circulatory collapse.
PF due to an acute infection with septic shock could be easily ruled out in our case for the negative microbiological workup.9
Many other conditions, however, are related to systemic coagulopathies including factor V Leiden mutation; antithrombin III deficiency; proteins C and S deficiency; prothrombin G20210A mutation; hyperhomocysteinemia; plasminogen deficiency; and elevated factors VIII, IX, or X; hospitalization; surgery; immobilization; catastrophic antiphospholipid syndrome; solid neoplasm or hemo-lymphoproliferative disorders; pregnancy; obesity and smoking; drugs; acquired hyperhomocysteinemia; and acquired antithrombin III.1 Although we cannot rule out all causes of systemic coagulopathy in our patient as specific laboratory examinations could not be performed for the early exitus, his medical history suggests that coagulation abnormalities were triggered by an acute pancreatitis with postsurgical complications that is a rare event.10,11 Actually, various alterations in coagulation have been reported during acute pancreatitis10,12,13 and such hemostatic abnormalities seem to be related to early intravascular consumption of coagulation factors secondary to the delivery of circulating pancreatic enzymes or are secondary to vascular injury.
In conclusion, dermatologists may be called to assist in the diagnosis and management of PF, especially when unusual sites are involved. Histopathologic studies of the occlusive vasculopathic lesions are the first step to achieve an accurate diagnosis, and they should be correlated with clinical and laboratory findings to reach the final diagnosis.
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