Celiac disease (CD) is an autoimmune enteropathy resulting from intolerance of an individual genetically predisposed to gluten. It has a large clinical polymorphism ranging from a classic digestive clinical presentation due to the malabsorption syndrome to extra-intestinal symptoms. Among the hematologic abnormalities, venous thromboembolic disease (VTE) has been reported, and they are most often located in the abdomen or lower limbs, but the cerebral localization was exceptionally described. We report a case of CD revealed by cerebral thrombophlebitis. A 44-year-old patient with no medical history and no drug intake, presented with hemiplegia followed by a status epilepticus in a context of apyrexia, initially hospitalized in intensive care. Magnetic imaging resonance displayed a cerebral venous thrombosis of the sigmoid sinus requiring anticoagulant treatment, then transferred to our department for the etiological investigation. On questioning, the patient reported chronic diarrhea and weight loss with no other associated symptoms. The examination revealed an underweight patient with pale conjunctiva, improvement of her deficit symptoms, and no other abnormalities. Laboratory tests noted biological signs of malabsorption. The thrombophilia assessment revealed a protein C deficiency with a slight increase in anticardiolipin antibodies and anti-Beta 2 glycoprotein 1 antibodies. Immunological tests noted positives anti-transglutaminase and IgA anti-endomysium antibodies. Duodenal biopsy demonstrated villous atrophy. After ruling out the other causes of VTE, the diagnosis of cerebral venous thrombosis secondary to CD was retained. Early diagnosis and treatment of CD improves the quality-of-life for patients and may spare them various long-term or even fatal complications.

Celiac disease (CD) is an autoimmune enteropathy resulting from intolerance of an individual genetically predisposed to gluten. It has a large clinical polymorphism ranging from a classic digestive clinical presentation due to the malabsorption syndrome to extra-intestinal symptoms. Among the hematologic abnormalities, venous thromboembolic disease (VTE) has been reported, and they are most often located in the abdomen or lower limbs, but the cerebral localization was exceptionally described. We report a case of CD revealed by cerebral thrombophlebitis. A 44-year-old patient with no medical history and no drug intake, presented with hemiplegia followed by a status epilepticus in a context of apyrexia, initially hospitalized in intensive care. Magnetic imaging resonance displayed a cerebral venous thrombosis of the sigmoid sinus requiring anticoagulant treatment, then transferred to our department for the etiological investigation. On questioning, the patient reported chronic diarrhea and weight loss with no other associated symptoms. The examination revealed an underweight patient with pale conjunctiva, improvement of her deficit symptoms, and no other abnormalities. Laboratory tests noted biological signs of malabsorption. The thrombophilia assessment revealed a protein C deficiency with a slight increase in anticardiolipin antibodies and anti-Beta 2 glycoprotein 1 antibodies. Immunological tests noted positives anti-transglutaminase and IgA anti-endomysium antibodies. Duodenal biopsy demonstrated villous atrophy. After ruling out the other causes of VTE, the diagnosis of cerebral venous thrombosis secondary to CD was retained. Early diagnosis and treatment of CD improves the quality-of-life for patients and may spare them various long-term or even fatal complications.

Neurofibromatosis type 1 is a common neurocutaneous syndrome, caused by an alteration of the NF-1 gene, which is a tumor suppressor. It has been reported to be associated with different types of benign and malignant tumors but its association to systemic diseases is uncommon and has not been reported previously to be associated with vasculitis particularly granulomatosis with polyangiitis (GPA). We report a case of a 17-year-old male patient, who, during his follow-up for neurofibromatosis type 1, in our outpatient consultation; we objectified hypereosinophilia at 1700 eosinophils/μl without a history of asthma or atopy. He reported a nasal obstruction with epistaxis and no rhinorrhea or pruritus. Physical examination revealed afebrile patient with the lesions of his neurofibromatosis type 1 without cutaneous rash or urticaria. Rhinoscopy didn’t show any lesion. Laboratory tests revealed a normal renal function, negative 24-hour urine protein, and no biological inflammatory syndrome. Immunological tests noted positives cytoplasmic antineutrophil cytoplasmic antibodies, and a slight increase in antinuclear antibodies at 1/180. Extensive infectious research was negative. Computed tomography (CT) of the sinuses revealed a non-obstructive nasal septum deviation with anatomical variations, and a chest scan showed multiple bilateral pulmonary nodules and micronodules. After ruling out the other etiologies, we retained the diagnosis of granulomatosis with polyangiitis according to American College of Rheumatology (ACR) criteria 1990 and we could start early the treatment. To our knowledge, the association between Neurofibromatosis type 1 and vasculitis, particularly GPA, has not been reported previously, which makes our case original and confirms the utility of an extensive lesion assessment during the follow-up.