Introduction: PVT is a major cause of morbidity and mortality with Pemphigus vulgaris patients. The immune substrate, which amplifies systemic inflamma-tion, together with the immunosuppressive therapy are potential risk factors for this disease.
Case presentation: An 81 years old female was hos-pitalized in the dermatology department for pemphi-gus vulgaris. Onset of the skin involvement was two months prior to admission, with progressive extension up to disseminated cutaneous and oral mucosa lesions. The disease extension and severity was classified as se-vere by the Pemphigus Disease Area Index score. She began treatment with prednisone 1.5 mg/kg/day and azathioprine 50 mg b.i.d. with minimal improvement. She was also under treatment with zofenopril 30mg qd, moxonidine 0.2 mg bid, clopidogrel 75 mg qd and ro-suvastatin 5 mg qd. After two days presenting with a slight enlargement of the right thigh, on the morning of the 19th day of hospitalization she had a syncope while sitting. The blood pressure was 85/60 mmHg, the heart rate 80 bpm and O₂ saturation on room air 95%. The pulmonary auscultation was normal.
ECG showed sinus rhythm, low voltage, new onset complete right bundle branch block, negative T waves in precordial leads and a S1Q3 pattern. Chest CT scan confirmed bilateral pulmonary embolism. On arrival to the Cardiology department, the patient was hypo-tensive, with tachycardia and tachypnea. Unfractiona-ted heparin was started. After saline perfusion and ino-tropic support, the blood pressure normalized and the patient was weaned off the noradrenaline infusion. The cardiac ultrasonography showed enlarged right heart chambers, paradoxical movement of the interventricu-lar septum, severe tricuspid regurgitation with a gradi-ent of 18 mmHg, estimated pulmonary artery arterial pressure of 38 mmHg and a free-floating serpiginous thrombus in the right atrium moving back and forth towards the right ventricle. After 5 hours, suddenly her dyspnea worsened and the blood pressure started to de-crease. Low-dose norepinephrine was started together with oxygen therapy. Repeated cardiac ultrasound no longer showed the free-floating right heart thrombus. Under these circumstances, the patient received the two-hour protocol of alteplase administration. During the thrombolysis dyspnea improved considerably and the vital signs normalized progressively, allowing down-titration up to weaning off the noradrenaline drip. Minor bleeding occurred predominantly around the lips and eyelids, without significant decrease of he-moglobin levels.
Particularities: To our knowledge, this is the first re-ported case of a successful thrombolysis for high-risk pulmonary embolism on a patient with active extensive pemphigus vulgaris.
Conclusion: Due to its autoimmune and inflammato-ry substrate, pemphigus vulgaris is associated with an increased risk of venous thrombosis. Other potential risk factors such as immobilization, comorbidities or even corticosteroid treatment may be considered. In life-threatening high-risk pulmonary embolism, after multidisciplinary team assessment of risk-benefit ratio, thrombolysis may be a lifesaving procedure.