Pulmonary hypertension (PH) can be related to several diseases, such as connective tissue disorders and pulmonary embolism, or to drugs; it may also be idiopathic. Few cases have been reported demonstrating an association between ascorbic acid deficiency and reversible PH. We report the case of a patient who arrived at the emergency department with dyspnoea, tachycardia and lower limb perifollicular haemorrhage. Examinations, including a transthoracic echocardiogram, revealed enlarged right chambers and an estimated pulmonary artery systolic pressure of 61 mmHg. Further evaluation revealed poor food intake due to paranoid personality disorder, leading to ascorbic acid deficiency and manifestations of scurvy.

Cardiopulmonary resuscitation-induced consciousness is a rarely described and often misunderstood phenomenon, although it can be encountered. High quality cardiopulmonary resuscitation (CPR) may lead a patient to recover consciousness while in cardiac arrest. The authors present the case of an 89-year-old male patient who received CPR after a cardiac arrest. Spontaneous movements during CPR were noted and prompted several CPR interruptions. These movements immediately stopped during chest compression pauses. Physical restraint was used in order to be able to continue with the CPR algorithm, but sedation may be the best approach. Guidelines on how to identify and manage these cases need to be developed.

Chemotherapy usually causes complications affecting several tissues such as oral mucosa. In this case report, a soft palate oral ulcer caused by chemotherapy was treated by ozone gas. This kind of treatment is known for its antimicrobial, regenerative and analgesic proprieties. The results show a complete resolution of the lesion within 2 weeks of treatment. Ozone therapy demonstrates greater effectiveness with respect to this kind of oral lesion compared to traditional therapy. Considering this evidence, ozone therapy should be considered as a useful tool for the adjuvant therapy of oral complications in oncologic patients.

Objectives: Agents targeting the epidermal growth factor receptor (EGFR)-mediated signalling pathway are increasingly being used for the treatment of advanced lung, pancreatic, colorectal and head and neck cancers.
Case presentation: Here, we report the first case of eruptive seborrhoeic keratosis following panitumumab treatment, an anti-EGFR monoclonal antibody, in a 73-year-old patient with stage 4 (IV) colorectal cancer with hepatic metastasis.
Conclusion: While panitumumab is an emerging therapy for RAS wild-type metastatic colorectal cancer, physicians should consider panitumumab as a potential cause of eruptive seborrhoeic keratosis.

Behçet’s disease (BD) is a systemic vasculitis characterized by recurrent orogenital ulceration and several systemic manifestations (such as gastrointestinal involvement, vascular disease or arthritis). The pathogenesis is still unknown but the trigger role of certain pathogens such as Mycobacterium tuberculosis is well documented. Furthermore, patients with BD are more susceptible to tuberculosis due to immunity defects. Here, we describe the case of a 70-year-old woman with a history of recurrent oral aphthae and inflammatory arthritis presenting with extensive thrombosis of left upper limb major veins, a positive HLA B51 genotype and colon ulceration; hence, BD diagnosis was made after excluding other causes. Simultaneously, the patient had cutaneous abscesses not associated with immunosuppressive therapy with continuous development, and after recurrent negative tuberculosis work-up, M. tuberculosis was isolated in an abscess culture.

Heat stroke (HS) is a life-threatening condition characterized by hyperthermia and multiple organ failure. Mild to moderate hepatocellular injury is a well-documented complication but severe liver injury and acute liver failure are rare. There are neither established criteria nor optimal timing for liver transplantation and conservative management seems to be the cornerstone treatment. The authors report a case of a patient with severe liver injury related to HS who recovered completely under conservative treatment.

Thrombotic disease represents a rare manifestation of leprosy. In this study, we report the case of an external jugular vein thrombosis associated with tuberculoid leprosy in a 23-year-old male patient. The patient presented with a 3-month history of painful cord-like swelling on the left side of the neck and a nearly 3-week history of skin lesions on the left cheek and right leg. Physical examination revealed cord-like, tender swelling on the left lateral aspect of the neck overlying the sternocleidomastoid muscle, and a hypopigmented, hypoaesthetic 6×7 cm lesion with an irregular margin on the left cheek. A Doppler ultrasound examination of the jugular vein showed thrombosis of the left external jugular vein. Three-dimensional reconstruction of the computed tomography scan showed the enlarged and enhanced left external jugular vein, as well as 1 of its tributaries, and the thickened skin patch. A skin punch biopsy from the left cheek lesion revealed granulomatous inflammation with occasional peri-adnexal granulomas, consistent with the clinical impression of tuberculoid leprosy. A diagnosis of leprosy with external jugular vein thrombosis was established. Anticoagulation therapy was initiated, and the patient was referred to an infectious disease clinic for treatment with anti-leprosy medications.

Background: Copper is an essential trace element of the human body. However, it is related to many diseases. Copper intoxication is not common in Western countries, but needs to be rapidly recognised because of its high lethality.
Case presentation: We report the case of a 40-year-old woman who presented to the emergency department after performing intrarectal administration of a blue powder sent from Cameroon by her family, in the belief that this would help her to get pregnant. Her evolution was complicated by multiorgan failure and the unusual circumstances. The diagnosis was suspected on the basis of the clinical presentation and the colour of the powder, and confirmed by blood dosage and toxicological analysis of the powder. She underwent symptomatic treatment, and the outcome was progressively favourable, apart from persistent chronic renal failure with dependence on dialysis.
Conclusion: Copper intoxications are rare but severe. Laboratory diagnosis of the condition is not an issue; the difficulty is suspecting it and quickly initiating chelation treatment associated with symptomatic treatments.

Introduction: Lumbar epidural anaesthesia is a commonly used technique for analgesia during labour. One of the rare complications of this technique is pneumocephalus.
Case description: We report the case of a 35-year-old female admitted to the Emergency Department with severe headache associated with fast head movements. Five days previously she had a eutocic delivery and lumbar epidural anaesthesia was performed. A brain computed tomography (CT) scan showed pneumocephalus and she was admitted to the hospital ward. A brain CT scan on the fourth day of hospitalization showed resolution of ventricular pneumocephalus.
Discussion: The most frequently occurring symptom with pneumocephalus is headache associated with fast brain motion resulting from air injection and meningeal irritation. When there is clinical suspicion of pneumocephalus, a brain CT scan should be performed for the diagnosis.

Brugada phenocopies (BrP) are clinical entities that present with an ECG pattern identical to either the type 1 or type 2 Brugada pattern without true congenital Brugada syndrome. This ECG pattern is associated with an identifiable condition and normalizes upon resolution or treatment of the underlying cause. We present a case of a 54-year-old man with extreme metabolic acidosis, hyperkalaemia and a Brugada type 1 ECG pattern in the setting of a suicidal methanol (MeOH) poisoning. Upon correction of these metabolic derangements with bicarbonate infusions and continuous veno-venous haemodiafiltration (CVVH), the Brugada type 1 ECG pattern normalized. Unfortunately, the patient developed signs of cerebral herniation followed by brain death and died on the first day of ICU admission.

Cystic mesotheliomas (also called mesothelial inclusion cysts) are rare benign neoplasms that occur more often in young women. Symptoms are usually non-specific, demanding a thorough work-up. We report a case of a 40-year-old female patient with 2 prior caesarean sections presenting with a 3-month history of abdominal pain. Laboratory tests revealed microcytic hypochromic anaemia and an elevated tumour marker CA-125. An investigation identified a large cystic abdominal mass, mostly in the left side of the abdomen. A laparotomy was performed with total resection of the lesion. Histological assessment resulted in a diagnosis of a mesothelial inclusion cyst. The patient had no recurrence after 3 years.

A 55 years-old Caucasian male presented initially in the emergency room reporting myalgia, chills and fever. Physical exam and laboratory tests were unremarkable and he was discharged with symptomatic care. He returned to our ER two weeks later reporting dizziness, loss of balance, blurred vision, mild dysarthria and bilateral hand paresthesia. On examination he presented complete bilateral ophthalmoplegia, mild dysarthria, left finger-to-nose dysmetria, ataxia, areflexia and bilateral hand hypoesthesia without fever. Blood tests and head computed tomography were normal. The patient was admitted to the Internal Medicine department. On second day inwards the patient presented dysphagia. Head magnetic resonance angiogram showed no signs of ischemia or vascular disease and a lumbar puncture was performed but no pleocytosis, albumin-cytological dissociation or hypoglycorrhachia was present. Despite the normal results we suspected of a Guillain–Barré syndrome variant, and started treatment with intravenous immunoglobulin (IVIG) in a dose of 400mg per kilogram and continued for five days with immediate neurological improvement. We present a rare overlapping case of Miller Fisher syndrome and Pharyngeal-Cervical-Brachial variant of Guillain–Barré syndrome.

The authors present the case of a 27-year-old patient who suffered from spontaneous bleeding during infancy and from a severe and central venous thrombosis in adult years. The patient underwent a thorough laboratory work-up on both occasions and was diagnosed with hypofibrinogenaemia as well as protein S deficiency, 2 diseases that contrast in their intrinsic bleeding/thrombotic risk. The patient´s high-risk pregnancy was carried out up to a successful full-term eutocic delivery which required fibrinogen concentrate to reduce life-threatening bleeding. The patient´s child was also diagnosed with hypofibrinogenaemia, later on confirmed with the pathogenic mutation Fibrinogen Marseilles II. This case was used to conduct a literature review of congenital fibrinogen disorders, rare entities that require more awareness for early diagnosis and accurate management.

Cardiovascular involvement by Leptospira is unusual. We report a case of a 29-year-old male sanitation worker who was diagnosed with leptospirosis (PCR testing for Leptospira spp. was positive in blood and urine). He had no classical clinical findings, and the presence of shock was the main worrisome finding. Within 24 hours of admission, the emergence of chest pain led us to consider cardiac involvement, which was demonstrated by diffuse supra-ST segment elevation, elevated troponin and NT-proBNP and an echocardiogram showing mildly left ventricular ejection fraction (LVEF 48–50%). There are several reports of cardiovascular involvement in severe leptospirosis but most patients are asymptomatic. In this case, the main key to diagnosis of perimyocarditis was the new onset of chest pain.

Bilateral diaphragm paralysis due to bilateral isolated phrenic neuropathy (BIPN) is a very rare cause of unexplained respiratory failure.
We present a 65-year-old patient with no relevant previous medical history who presented in the Pulmonology Clinic with mMRC1 dyspnoea and orthopnoea. After the medical work-up, diaphragmatic paresis was diagnosed. Inspiratory muscle training resulted in mild symptomatic improvement and treatment with noninvasive mechanical ventilation (NIV) was initiated.
This condition is generally chronic and has a poorer prognosis, compared to other cases of phrenic nerve involvement. In this case, NIV restored near-normal daily function.

Introduction: Extramedullary plasmacytomas are present in 13% of multiple myeloma (MM) patients. Less than 5% of MM cases are non-secretory. The orbital location is uncommon and a minority of orbital tumours are plasmacytomas.
Description: The patient was a 71-year-old man, with right proptosis, retro-ocular pain and epistaxis with visual acuity 2/10, limitation of upper eye movement and scattered ecchymosis. Blood tests revealed severe anaemia, coagulopathy, increased serum creatinine, LDH and C-RP without improvement after antimicrobial treatment. Peripheral immunophenotyping showed 9.4% of plasma cells with intracytoplasmic clonal κ chains. IgG and λ chains were decreased with normal plasma and urine immunofixation. Orbital CT: retro-orbital superomedial tumour with bone destruction. Histology of the tumour and bone biopsy was consistent with plasmacytoma. The patient was deceased in 2 weeks.
Discussion: MM accounts for 10% of haematopoietic tumours; 7% of cases present with plasmacytomas at diagnosis. Orbital locations are rarely reported (frequently in the temporal region). Proptosis, ptosis and reduced visual acuity are common symptoms. However, orbital pain is less frequent. Most cases of MM demonstrate hypergammaglobulinaemia. Only 5% of MM cases are non-secretory.
The uncommon location, topography, symptom peculiarities and absence of monoclonality led to the diagnostic challenge of this fatal case of MM.

The authors report the case of an 86-year-old woman presenting with recurrent Klebsiella pneumoniae bacteraemia. She had severe aortic stenosis submitted to a recent transcatheter aortic valve implantation (TAVI). Initially, Klebsiella pneumoniae bacteraemia from a urinary source was diagnosed . Following another 4 episodes of bacteraemia with the same agent, the source was ultimately found to be a periprosthetic abscess. Considering the patient’s unsuitability for surgery, a decision was made for life-long antimicrobial therapy. This approach has been successful in preventing recurrences or complications. Endocarditis is one of the most severe complications seen following TAVI, often carrying a poor prognosis. Even though Klebsiella spp. are common pathogens for healthcare-associated infections among the elderly, they are seldom the causative agent for endocarditis. Being the first reported case of TAVI-related Klebsiella endocarditis, it was successfully managed using a medical approach.

Reactive arthritis (ReA) with the classic triad of arthritis, conjunctivitis and urethritis, previously termed Reiter’s syndrome, is a systemic illness, usually induced by genitourinary or gastrointestinal infections. However, it can be a rare complication of intravesical Bacillus Calmette-Guérin instillation (iBCG), a therapy prepared from attenuated strains of Mycobacterium bovis, a common and effective treatment for carcinoma in situ of the bladder (CisB). We report a case of a patient with CisB who developed ReA after iBCG. The symptoms resolved completely with corticosteroids. iBCG was stopped with no recurrence of carcinoma within 2 years.

Background: Few cases have been reported with respect to portal vein thrombosis in non-cirrhotic patients. Asymptomatic or non-specific symptoms of portal vein thrombosis may lead to misdiagnosis or may delay the diagnosis until complications develop. We report a case of portal vein thrombosis in a patient with type 1 diabetes presenting as acute pyelonephritis.
Case description: An 18-year-old female with type 1 diabetes on an insulin pump presented with epigastric abdominal pain for 3 days associated with nausea and vomiting. She was a conscious, alert, young female who appeared to be in pain. Vital signs were stable with a random blood sugar (RBS) level of 179 mg/dl. Abdominal examination revealed a soft and lax abdomen with tenderness in the epigastric area and right renal angle, as well as no sign of rigidity or rebound tenderness. No signs of ascites, splenomegaly or hepatomegaly were noted. Investigations showed a WBC count of 10.2, neutrophils at 65%, urine microsopy analysis revealed WBCs between 30–50 per high power field, with culture showing >105 CFU/ml. All parameters of a thrombophilic screen were within normal values. Computed tomography (CT) revealed reduced enhancement of the right kidney, likely indicating acute pyelonephritis, and left portal vein oedema with complete occlusion. Local factors and prothrombotic disorders were ruled out. The patient was managed with ciprofloxacin, enoxaparin and warfarin. Follow-up imaging revealed complete resolution of thrombosis.
Conclusions:Portal vein thrombosis is an uncommon condition in the absence of liver disease. Few case reports exhibit sepsis and portal vein thrombosis. Sepsis can create a predisposed environment for hypercoagulability. To our knowledge, this is the first case report of pyelonephritis with portal vein thrombosis.

We present a case of a 56-year-old man with a history of episcleritis (left) and cluster headache (left) who had a penetrating trauma of the left eye leading to amaurosis 1 month previously. Since then, he developed multiple cranial neuropathy of the right side (V, VII, VIII, IX, X, XI and XII cranial pairs). Magnetic resonance imaging (MRI) revealed an infiltrative lesion of the base of the skull which extended to the retropharyngeal and jugular space, which progressed to multiple leptomeningeal masses extending to the clivus, despite aggressive immunosuppression. Rebiopsy of 1 meningeal mass supported the diagnosis of neurosarcoidosis. The patient finally responded to high-dose prolonged infliximab therapy, with complete remission.

Background: Breast cancer is the most commonly diagnosed cancer in women, mainly at an early stage, allowing treatment with curative intent. Aromatase inhibitors are widely used in the adjuvant treatment of oestrogen receptor-positive breast cancer, mainly in postmenopausal women. The most frequent adverse events associated with these therapies are musculoskeletal symptoms and an increased risk of bone fractures. Cutaneous adverse events have been rarely described. Sweet’s syndrome can present as an idiopathic disorder in addition to being malignancy-associated or drug-induced.
Case presentation: We report the case of a 69-year old woman, diagnosed with early stage breast cancer, who underwent breast-conserving surgery, followed by adjuvant radio and endocrine treatment with letrozole 2.5 mg daily, for a foreseeable duration of 5 years. Three months after starting letrozole, she presented with sudden fever and exuberant and painful erythematous skin papules and plaques on her upper body. After a full work-up and exclusion of other potential causes, a skin biopsy confirmed the presence of dermal oedema and a diffuse neutrophilic infiltrate, suggesting Sweet’s syndrome. After discontinuation of letrozole and treatment with corticosteroids, the patient fully recovered. She resumed adjuvant treatment with tamoxifen, without symptom recurrence.
Conclusions: Sweet’s syndrome is a rare condition and an association with aromatase inhibitors has not been previously reported. Although its occurrence has already been observed in the onset of malignancies such as breast cancer, aromatase inhibitors must be added to the list of potential causes of drug-induced Sweet’s syndrome.

Introduction: Turicella otitidis, described as a new species over 20 years ago, has been isolated mainly from the external ear canal and middle ear fluid. Here, we report the first case of palmoplantar eczema related to T. otitidis.
Case presentation: Here, we report the first case of palmoplantar eczema in a 74-year-old female related to T. otitidis.
Conclusions: The question as to whether T. otitidis is a potential pathogen in cases of dyshidrotic eczema is still open, but this could be better elucidated if corynebacteria were speciated more often.

Background: The causes of inflammatory bowel disease (IBD) have not yet been clearly elucidated, but it is known that genetic susceptibility, altered gut microbiota and environmental factors are all involved, and that a combination of these factors causes an inappropriate immune response, resulting in impaired intestinal barrier function. With regard to the treatment of IBD, the use of conventional immunosuppressive drugs has been complemented by more specific therapeutic agents, including biological drugs. Systemic immune suppression is a risk factor for cytomegalovirus (CMV) infection, which is associated with considerable morbidity and mortality in immunocompromised hosts.
Case Report: A 33-year-old male patient was admitted to our medical unit complaining of a 10-day history of fever, fatigue and headache. He had been suffering from ulcerative colitis and primary sclerosing cholangitis for five years and was currently being treated with azathioprine and vedolizumab. In the past he had already taken infliximab, adalimumab and golimumab without any clinical response. After the exclusion of systemic infectious diseases, his serology was consistent with a primary CMV infection. This was successfully treated with intravenous ganciclovir therapy.
Conclusion: Vedolizumab is an anti-integrin biological agent approved for IBD treatment. Its gut-selective mechanism of action would appear to increase its safety profile, however data on this are still limited. Moreover, it should always be remembered that IBD patients have an increased risk of CMV infection, both primary and reactivation, because of their concurrent immunosuppression.

Manifestations of chronic cutaneous lupus erythematosus are variable. Periorbital and facial swelling occurs in dermatomyositis and systemic lupus, but it has been rarely reported as a manifestation of exclusively cutaneous lupus. A 48-year-old woman presented with a 16-year history of asymptomatic, bilateral swelling and erythema of her face with marked worsening after sun exposure. No systemic symptoms were associated. A complete evaluation did not reveal other findings. Cutaneous biopsy showed features of lupus erythematosus. She was treated with photoprotection, topical tacrolimus, hydroxychloroquine and azathioprine with a partial response. Facial swelling with erythema represents quite an unusual manifestation of chronic cutaneous lupus erythematosus. Dermatomyositis, systemic lupus and Morbihan disease are the main differential diagnoses.

We report the case of a 27-year-old man presenting with slowly progressive extrapyramidal dysfunction and learning disability considered to have a syndromic intellectual disability. The re-evaluation of the clinical features and the investigations performed led to the diagnosis of atypical pantothenate kinase-associated neurodegeneration (PKAN).

Introduction: Diabetic myonecrosis is an uncommon complication of diabetes mellitus, most often occurring in patients with poorly controlled, insulin-dependent diabetes. Its etiology is poorly understood, with many suggesting microvascular occlusion to be a key factor resulting in necrosis of skeletal muscle.
Case presentation: A 28-year-old male with a history of poorly controlled type I diabetes mellitus and end-stage renal disease requiring dialysis presented to the emergency department with severe pain of the lower extremities bilaterally.
Results: Work-up included an x-ray, which demonstrated no acute fractures but extensive vascular calcification of the lower extremities, and Doppler ultrasonography, which showed no DVT. MRI demonstrated severe muscular edema with patchy, geographic areas of sparing, which, in conjunction with the patient’s clinical presentation, allowed for a diagnosis of diabetic myonecrosis. He underwent conservative treatment, consisting of rest and pain management, leading to resolution of symptoms.
Discussion: Diabetic myonecrosis, although uncommon, can be easily diagnosed with a high degree of clinical suspicion. It typically presents in a patient with poorly controlled diabetes, and will commonly involve the proximal muscles of the lower extremity unilaterally, but may present bilaterally and involve the distal muscles, as demonstrated in this case. Diagnosis is made via MRI, and it is treated conservatively with pain management and rest. Most cases resolve with conservative management but recurrence is common.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary condition characterized by diffuse proliferation of neuroendocrine cells in the epithelium of the bronchial wall. DIPNECH may be easily missed in daily clinical practice and diagnosis is often delayed, which may impair prognosis since this condition is considered a pre-invasive lesion for lung carcinoid tumours. We report a clinical case of DIPNECH in order to discuss the diagnostic and therapeutic approach for this entity, the management of which is not yet well established in the literature.

Polyarteritis nodosa is a systemic necrotizing vasculitis that typically affects medium-sized muscular arteries, with occasional involvement of small muscular arteries. Most cases of polyarteritis nodosa are idiopathic but multiple infectious agents have been associated with this disease. We present a clinical case of a 72-year-old male with fever, diarrhoea and haemodynamic instability, diagnosed with a bacterial infection caused by Salmonella Typhi. One week after clinical resolution of the infection, the patient developed purpuric lesions with ulcers, pustules and necrotic areas accompanied by testicular pain and weight loss of 5 kg over the previous 15 days. A skin biopsy was performed and it revealed typical histologic signs of polyarteritis nodosa. The aetiologic association between bacteria of the genus Salmonella and polyarteritis nodosa has been previously described in the scientific literature but seldom meeting classification criteria and with histologic confirmation.

Mycobacterium tuberculosis affects the middle ear in rare cases and is a challenging diagnosis. In this case, we present a 57-year-old patient diagnosed with anti-neutrophil cytoplasmic antibody (ANCA)-negative granulomatosis with polyangiitis (GPA) following a biopsy result of nasal granulomas, who was immediately started on immunosuppressive treatment. Years later, she developed progressive hypoacusis. Magnetic resonance imaging (MRI) revealed an extensive mass in the tympanic cavity extending to the mastoid. A biopsy of the mass was positive for Mycobacterium tuberculosis. Immunosuppressants were weaned and the patient was started on anti-tuberculous therapy with resolution of the complaints and findings. Tuberculous infections are difficult to diagnose and frequently mimic other illnesses, but in our case, we believe that an indolent tuberculous process was present from the beginning and evolved under immunosuppressive therapy.

A 77-year-old man was admitted to the internal medicine department for a 5-day history of progressive preauricular swelling. Two lines of antibiotic treatment failed to achieve any improvement. Fine needle aspiration cytology was conducted and smear staining with the Ziehl-Neelsen stain as well as a PCR test were positive for Mycobacterium tuberculosis. These results were confirmed with culture of the sample. A diagnosis of tuberculosis parotitis was made and anti-tuberculous drugs were initiated.

Mesenteric cysts are a rare nosologic entity, the diagnosis of which is complex due to their nonspecific presentation. They may emerge from any part of the mesentery and grow to any size, thus conditioning a wide range of clinical manifestations that renders them easily mistaken for different gastrointestinal pathologies. Diagnosis encompasses a mixture of clinical suspicion, imaging techniques and sometimes surgery, and curative treatment is based on complete surgical resection of the cyst. We hereby present a case of a mesenteric cyst that developed on the anterior abdominal wall of a 59-year-old man awaiting allogeneic bone marrow transplantation after being diagnosed with chronic myeloid leukaemia. He was admitted to the emergency room with complaints of an increased abdominal perimeter and increased weight, not associated with alterations to his dietary or physical exercise habits. Suspecting ascites in the context of leukaemic progression, the patient was admitted to the medical ward; however, subsequent study identified a mesenteric cyst as the most probable diagnosis and the patient was proposed to undergo surgery. He underwent laparotomic cyst excision without complications and the histological evaluation of the surgical specimen confirmed the diagnosis.