2020: Vol 7 No 1

POEMS syndrome is a rare paraneoplastic condition associated with polyneuropathy, organomegaly, monoclonal gammopathy, endocrine and skin changes. We report a case of a man with Castleman disease and monoclonal gammopathy, with a history of chronic diarrhoea and asthenia. Gastrointestinal involvement in POEMS syndrome is not frequently referred to in the literature and its physiopathology is not fully understood. Diagnostic criteria were met during hospitalization but considering the patient’s overall health condition, therapeutic options were limited. Current treatment for POEMS syndrome depends on the management of the underlying plasma cell disorder. This report outlines the importance of a thorough review of systems and a physical examination to allow an attempted diagnosis and appropriate treatment.

Pseudoxanthoma elasticum (PXE) is a rare genetic disorder characterized by calcification of elastic fibres, skin lesions, fundus lesions and systemic vascular complications. PXE affects approximately 1 in 160,000 people, typically appearing as a formation of yellow papules containing abnormally calcified elastic fibres. The renal involvement of PXE has been reported. Several factors are known to promote soft tissue and accelerated arterial calcification in chronic kidney disease, including systemic inflammation, altered calcium and phosphate homeostasis, hypertension and a deficiency of endogenous calcification inhibitors. Given the impact of this disease, nephrologists may seek additional supportive features to improve and avoid the risk of complications. Moreover, PXE per se represents an interesting model to evaluate vascular disease in the early stages of renal disease.

Penile strangulation is a rare clinical entity which if left untreated can cause serious urogenital problems. We present the case of 45-year-old male patient who presented with penile strangulation after applying a metallic ring. Strangulation of the external male genitalia is a serious problem that requires urgent intervention in order to avoid serious complications. If left untreated, it can result in gangrene and penile amputation. Management of penile strangulation is a real challenge for the treating surgeon and should be treated as an emergency in order to avoid vascular compromise.

Objectives: Baricitinib is an orally active Janus kinase (JAK) inhibitor used in the treatment of moderate to severe rheumatoid arthritis (RA).
Materials and methods: Here, we report the case of a 56-year-old Caucasian male diagnosed with RA who developed palmoplantar pustulosis (PPP) while being treated with baricitinib.
Results: The patient’s PPP resolved after discontinuation of baricitinib and recurred when this was restarted. Based on causality assessment, it was considered a drug-induced PPP. Conclusion: To the authors’ knowledge, this is the first case of baricitinib-induced PPP.

Hemichorea is a rare clinical manifestation of type 2 diabetes (DMT2). The patient presents with non-ketotic hyperglycemia, hemichorea (characterized by rapid and involuntary movements of a specific part of the body) and the CT imaging reveals the presence of alterations involving the ganglia of the base.

We describe a 66-year old patient with a recurrent ulcer on her right ankle. Biopsy revealed medium-vessel vasculitis consistent with cutaneous polyarteritis nodosa. There were no signs or symptoms suggestive of systemic vasculitis, but a 18FDG-PET scan showed areas of increased uptake around the large arteries and the pelvic and shoulder girdles. These findings suggested polymyalgia rheumatica in the setting of large-vessel vasculitis. This case thus supports the statement from the Chapel-Hill consensus conference that classification of systemic vasculitis by vessel size is based on the vessels predominantly involved, but vessels of other sizes may also be affected.

Cystic lymphangioma mainly occurs in children and develops from the lymphatic vessels. The present study reports the case of a 36-year-old female patient with a giant cystic mediastinal lymphangioma. She was asymptomatic and the diagnosis was accidental. Imaging studies are important for referral to the Cardio-Thoracic Surgery Department for surgical intervention in order to remove the lesion. It is important that in the case of an enlargement of the mediastinum observed in a thoracic X-ray, the possible diagnosis takes into consideration various hypotheses. Early diagnosis may prevent further growth and infiltration of structures that hinder the surgical approach.

A 20-year-old Swiss male presented at the emergency department with acute onset of febrile temperatures and hemoptysis and a 3-month history of productive cough. An X-ray and CT scan of the chest, sputum samples for acid-fast bacilli, polymerase chain reaction(PCR), and cultures for Mycobacteria revealed pulmonary infection with Mycobacterium tuberculosis. None of the classical risk factors for tuberculosis were present, but the patient reported regularly smoking a water pipe. Water-pipe smoking poses a serious risk of M. tuberculosis transmission.

Infectious purpura fulminans (PF) is a rare presentation of disseminated intravascular coagulopathy (DIC) due to diffuse intravascular thrombosis and haemorrhagic infarction of the skin. PF can present in infancy/childhood or adulthood and usually presents as ecchymotic skin lesions, fever and hypotension. It is most commonly a consequence of sepsis related to Neisseria meningitidis, Streptococcus pneumoniae or Haemophilus influenzae. Despite aggressive management of sepsis with intravenous fluids, antibiotics, and conventional and nonconventional therapies, the condition still carries a mortality rate of 43%^[1]. Streptococcus pneumoniae mostly presents with community-acquired pneumonia. We present a case of PF secondary to DIC related to Pneumococcal sepsis in an otherwise healthy and immunocompetent patient.

Meningitis is a very uncommon complication of spinal anesthesia, and drug-induced aseptic meningitis (DIAM) is even rarer. We present two cases of DIAM following spinal anesthesia with bupivacaine and ropivacaine, respectively. The patients presented shortly after the procedure with typical meningitis symptoms. Since CSF (cerebrospinal fluid) analysis could not initially exclude bacterial meningitis, they were started on empirical antibiotics.
CSF was subsequently found to be negative for viruses and bacteria in both cases, and antibiotics were promptly stopped. Both patients improved rapidly and without neurological sequelae. While it remains a diagnosis of exclusion, it is important to be aware of DIAM as recognition of the condition can lead to shorter admission times and avoid unnecessary use of antibiotics.

Tuberculosis (TB) is one of the top 10 causes of death worldwide. Multidrug-resistant tuberculosis (MDR-TB) occurs when at the minimum there is resistance to isoniazid and rifampin. Prevention of new infections of Mycobacterium tuberculosis and progression to TB disease is critical to reduce the burden and mortality of this disease. We present the case of a 73-year-old human immunodeficiency virus (HIV)-negative female who presented with cervical lymphadenopathy and who was diagnosed with MDR-TB.

Autoimmune hepatitis (AIH) was the first liver disease for which an effective therapeutic intervention was carried out, using prednisolone; its usefulness was demonstrated in several clinical trials. Nevertheless, AIH still remains a difficult diagnosis in some cases, because it is necessary to dismiss other possible diagnoses, and perhaps due to it being a heterogeneous disease. The relationship between drug-induced liver injury (DILI) and AIH is complex and not fully understood. There are three possible scenarios: (1) DILI with a strong immunoallergic component mimicking AIH; (2) AIH mimicking a DILI due to drug exposure and (3) AIH triggered by exposure to an offending drug (drug-induced AIH). Drug-induced AIH is well described and documented for some drugs such as nitrofurantoin and minocycline. Histologically distinguishing DILI from AIH remains a challenge. We present an interesting case report which met serologic criteria and histological confirmation to establish AIH, but discontinuation of a suspected drug resolved hypertransaminasaemia.

Autoimmune diseases (AID) have been associated with a variety of lymphoproliferative disorders. Multiple myeloma (MM), one of the most common haematologic malignancies characterized by clonal proliferation of bone marrow plasma cells, has been associated with a range of autoimmune disorders. In this report, we described a case study of a patient admitted to our Internal Medicine Department for a bone marrow biopsy and myelogram due to a monoclonal peak observed by his general practitioner. However, at admission he presented typical giant cell arteritis (GCA) complaints, suggesting the coexistence of both diseases. The possible pathogenesis, as found in the literature, explaining the association will be discussed.

Cutaneous metastasis has a frequency of 1 to 10% among all metastatic cancer forms and breast cancer accounts for 30% of all cases. We report the case of a 73-year-old woman who presented with 4 skin lesions distributed across the upper trunk and abdomen; these had developed over a period of 12 months. Over the previous 6 months she had also developed anorexia, asthenia and weight loss. Upon investigation, a nodular mass was found in the left breast. Skin and breast mass biopsy were performed. Histology confirmed the diagnosis: infiltrating lobular breast cancer with cutaneous metastasis. The patient underwent hormonal treatment, mastectomy and radiotherapy.
In rare cases, cutaneous metastasis appears as the first clinical manifestation of breast cancer. It is therefore crucial for patients and health professionals alike to be aware of new skin lesions. Cutaneous metastasis is a diagnostic sign of cancer that, it must be emphasised, is not restricted exclusively to later forms of the disease.

A 43-year-old man complaining of abdominal angina for several months showed a large suprarenal aneurysm of the abdominal aorta with extensive circumferential wall thrombosis, complete occlusion of the right renal artery and a critically stenosed left renal artery on CT angiography. He suffered from severe hypertension and renal failure. A percutaneous transluminal angioplasty (PTA) was planned. After the PTA procedure, which was complicated by the development of left renal artery occlusion, successful rescue revascularization surgery was performed. Since we were hesitant to start anticoagulant treatment because of a high bleeding risk, magnetic resonance direct thrombus imaging was performed to assess the age of the extensive arterial thrombosis. The aortic thrombus showed a low signal intensity, which is indicative of chronic rather than acute thrombosis. Therefore, oral anticoagulant treatment was not started. The patient recovered without major complications.