Lenalidomide: An Alternative Treatment for Refractory Behçet's Disease and Relapsing Polychondritis.

INTRODUCTION: Behçet's disease (BD) is a chronic systemic condition characterized by recurrent oral and genital ulcers, neurological and vascular manifestations, ocular and skin lesions, and a positive pathergy test. Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is rare and consists of features of BD together with relapsing polychondritis. The treatment of this syndrome is similar that of BD and depends on disease expression and symptoms. CASE REPORT: We report the case of a 50-year-old man diagnosed with BD with relapsing polychondritis (MAGIC syndrome) which had evolved over approximately 35 years, with exuberant and frequent oral and genital ulcers. Various treatments were unsuccessful and had adverse effects. Lenalidomide was initiated at a dose of 5 mg per day with remission of oral and genital ulcers and no haematological or adverse effects. CONCLUSION: BD is a complex disorder with a variety of presentations. Its treatment can be challenging but sometimes off-label drugs, like lenalidomide, can be effective. LEARNING POINTS: Behçet's disease (BD) is a chronic systemic autoinflammatory disease whose treatment can be challenging and is dependent on disease expression and symptoms.Thalidomide can be effective for treating recurrent oral aphthae, but its use is limited by adverse effects, mostly sensory polyneuropathy.Lenalidomide is a more powerful thalidomide analogue with fewer adverse effects and may be effective for the treatment of BD.

Breast Lump: A Rare Presentation of Tuberculosis in an Elderly Man.

INTRODUCTION: Chest wall masses are caused by various entities and have diverse aetiologies. A careful history and physical examination are crucial to establish the correct diagnosis. CASE REPORT: A 77-year-old man presented with depressive mood, anorexia (weight loss of 20 kg) and a 1-month history of a non-painful breast lump with well-defined contours, which was about 6 cm in diameter. There was no history of trauma. Computed tomography of the thorax revealed a collection of liquid in the left anterior thoracic wall, associated with discontinuity of the 4th left costal cartilage, and upper left lobe cavitation, suggesting pulmonary tuberculosis. The patient was started on quadruple therapy with anti-tuberculosis drugs and discharged after a negative smear. CONCLUSION: In this case, the indolent onset of unspecific symptoms made it difficult to reach a diagnosis of pulmonary tuberculosis, which was confirmed by positive culture and imaging. A breast lump in an elderly patient with unspecific clinical manifestations is an unusual presentation of pulmonary tuberculosis. It is important to be aware of rib invasion and exclude tuberculosis in a patient with a chest wall mass. As tuberculosis is treatable, early diagnosis is vital as diagnostic delay can lead to contagion. LEARNING POINTS: Chest wall tuberculosis is a rare complication of pulmonary tuberculosis.As smears and acid-fast bacilli cultures are often negative, polymerase chain reaction and imaging should be performed.Tuberculosis should be treated with first-line drugs; the role of surgery is still controversial.

Superior Vena Cava Syndrome: An Uncommon Presentation of a Rare Colon Carcinoma Metastasis.

INTRODUCTION: Superior vena cava (SVC) syndrome is caused by obstruction of the superior vena cava due to vascular compression by a mass or intrinsic obstruction. The authors describe SVC syndrome caused by an isolated metastatic mediastinal mass from a resected primary colon carcinoma. CASE REPORT: An 81-year-old woman was referred to the hospital with swelling of the neck and upper left limb, dysphonia and dysphagia, associated with an involuntary weight loss of 16 kg. Mediastinal metastasis of colon adenocarcinoma was found, causing the SVC syndrome. The mass was unresectable and the patient was referred to palliative radiotherapy. DISCUSSION: Only 12 cases of mediastinal metastasis from colorectal cancer have been reported in the English literature. CONCLUSION: As a rare manifestation of colorectal cancer, the presented case highlights the need for clinicians to be aware of rare metastases at the time of diagnosis. LEARNING POINTS: Superior vena cava (SVC) syndrome can result from vascular compression by a mass.Although mediastinal lymph node metastasis is rare in colorectal cancer, physicians should be aware of less common locations.Patients should have a close follow-up in order to avoid the growth of unresectable metastases, since surgery, when possible, can lead to a better prognosis.

Escherichia coli endocarditis of a native mitral valve.

Escherichia coli (E.coli) is a rare cause of endocarditis, although is a common causative agent of bacteremia. An 89-year-old woman presented with recurrent episodes of fever and persistent E. coli bacteremia with 3-month duration, despite antimicrobial therapy. At first, a urinary tract infection was diagnosed and later a mycotic aneurysm of the abdominal aorta was found and required an endovascular repair. The persistence of fever and the evidence of a systolic murmur at the mitral focus raised the suspicion of endocarditis. A transesophageal echocardiogram and a cardiac Magnetic Resonance Imaging (MRI) confirmed the presence of a vegetation at the mitral valve and the patient was treated with ceftriaxone. The presence of comorbid conditions and certain bacterial virulence factors predispose to this rare condition. A high level of suspicion is important to early diagnosis and prompt therapy.

Rare case of symmetrical peripheral gangrene due to septic shock, disseminated intravascular coagulation and inotropic use.

INTRODUCTION: Symmetrical peripheral gangrene (SPG) is a rare syndrome defined by the peripheral ischemic lesion of two or more extremities in the absence of major vascular obstructive disease. PRESENTATION OF CASE: A 45yo woman, admitted in intensive care unit due to urinary septic shock, in need of high doses of amines, developed cold extremities with acrocyanosis that rapidly progressed to gangrene. Laboratory analysis revealed increased inflammatory parameters, liver shock, thrombocytopenia, prolonged coagulation times, increased D-Dimers and isolation of Acinetobacter baumanni in urine culture. An intravenous vasodilator was initiated with clinical benefits. After improvement and delimitation of the lesions, the patient underwent the amputation of the distal phalanges of the 2nd, 3rd and 4th fingers of the right hand and the toes of both feet. DISCUSSION/CONCLUSION: Even though there is no consensus regarding SPG treatment, consequences should be mitigated, particularly when vasodilators are used, in order to avoid major amputation.