Basheer Tashtoush

Large pyoderma gangrenosum-like ulcers: a rare presentation of granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA), formerly known as Wegeners granulomatosis (WG), is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions.

Follicular Bronchiolitis: A Literature Review.

Follicular bronchiolitis (FB) also known as hyperplasia of the bronchial associated lymphoid tissue (BALT), or bronchiolar nodular lymphoid hyperplasia, is an entity characterized by the development of lymphoid follicles with germinal centers in the walls of small airways. FB is thought to be caused by antigenic stimulation of BALT, followed by a polyclonal lymphoid hyperplasia. It is currently classified as one of the reactive pulmonary lymphoid disorders in a group known as the lymphoproliferative pulmonary diseases (LPDs). FB is a pathological diagnosis that can be seen in several clinical settings, including connective tissue diseases, immunodeficiency states, autoimmune diseases, infections, obstructive airway diseases, as well as several types of interstitial lung diseases (ILDs). Its characteristics need to be carefully identified and differentiated from other closely related diseases in the group of LPDs due to significant differences in treatment and prognosis.

Granulomatous-lymphocytic interstitial lung disease as the first manifestation of common variable immunodeficiency

Common variable immunodeficiency (CVID) is one of the most common primary immunodeficiencies, which is characterized by reduced serum immunoglobulin levels and B‐lymphocyte dysfunction. There are many clinical manifestations of this disease, the most common of which are recurrent respiratory tract infections. Among the most recently recognized autoimmune manifestation of CVID is a disease described as granulomatous‐lymphocytic interstitial lung disease (GLILD), where CVID coexists with a small airway lymphoproliferative disorder, mimicking follicular bronchiolitis, or lymphocytic interstitial pneumonitis (LIP) on histology specimens. We herein describe the clinical and radiological features of GLILD in a 55‐year‐old woman where the diagnosis of CVID was actively pursued and eventually confirmed after her lung biopsy showed characteristic features of GLILD. The patient had dramatic response to treatment with IVIG and corticosteroids for 3 months followed by Mycophenolate mofetil for maintenance therapy.

Pulmonary Sequestration: A 29 Patient Case Series and Review.

INTRODUCTION Pulmonary sequestration also known as bronchopulmonary sequestration is a rare disease, with very few case series reviewed in literature. In this study, we review the demographics, presentation, imaging and treatment of pulmonary sequestration in 29 patients from our institution, and provide comparison data from previously published series with an overview of the disease history. MATERIALS AND METHODS Records reviewed for all patients evaluated and treated in our institution with a pathological proven diagnosis of pulmonary sequestration from January 2004 through December 2013. Collected data included demographics, clinical presentation, diagnostic imaging, location of the lesion, type of sequestration, and subsequent treatment. RESULTS Of the 29 patients reviewed 8 (28%) were children 0-2 years, 1 adolescent age 17, and 20 (69%) adults 21-70 years with a mean age of 42 among adults. Systemic arterial supply to the sequestered segment was demonstrated with computed tomographic angiography (CTA) in 25 patients (86%). In 19 patients (66%), the sequestered segment was located in the left lower lobe, and 16 (55%) were intralobar. CONCLUSION Diagnostic delays of pulmonary sequestration were common among the adult population as the presenting symptoms often mimicked other common pulmonary diseases, such as pneumonia and asthma. These findings were consistent among previously published series. CTA was the preferred imaging modality for preoperative planning with high sensitivity and specificity in identifying the lesion.

Syringomyelia with Chiari I Malformation Presenting as Hip Charcot Arthropathy: A Case Report and Literature Review

Neuroarthropathy (neuropathic osteoarthropathy), also known as Charcot joint, is a condition characterized by a progressive articular surface destruction in the setting of impaired nociceptive and proprioceptive innervation of the involved joint. It is seen most commonly in the foot and ankle secondary to peripheral neuropathy associated with diabetes mellitus. Cases of hip (Charcot) neuroarthropathy are rare and almost exclusively reported in patients with neurosyphilis (tabes dorsalis). We report a case of a 36-year-old man who presented to the emergency department complaining of right hip pain. On physical examination, pain and thermal sensory deficits were noted in the upper torso with a cape-like distribution, as well as signs of an upper motor neuron lesion in the left upper and lower extremities. A magnetic resonance imaging study (MRI) of the right hip showed evidence of early articular surface destruction and periarticular edema consistent with hip Charcot arthropathy. An MRI of the spine revealed an Arnold-Chiari type I malformation with extensive syringohydromyelia of the cervical and thoracic spine.

Strongyloidiasis and diffuse alveolar hemorrhage in a patient with systemic lupus erythematosus.

The presence of Strongyloides stercoralis infection in patients with systemic lupus erythematosus (SLE) has been described previously. Strongyloides stercoralis hyperinfection syndrome (SHS) that usually develops in patients under immunosuppressive therapy may affect a variety of organs, but the presentation with diffuse alveolar hemorrhage (DAH) is rare with only a few cases described in the literature. We present the case of a 36-year-old Hispanic female with a past medical history relevant for SLE and a recent diagnosis of lupus nephritis and hypertension. The patient who developed sudden and progressive abdominal pain and respiratory distress, with the presence of bilateral crackles and severe hypoxemia, is currently under treatment with steroids and cyclophosphamide for worsening of lupus nephritis. The patient underwent endotracheal intubation and mechanical ventilation, and computed tomography showed the presence of bilateral pulmonary infiltrates suggestive of DAH. Bronchoalveolar lavage was done and showed the presence of filariform larvae, morphologically consistent with Strongyloides stercoralis. Treatment with ivermectin was started and patient responded to treatment with improvement of clinical status. In conclusion, the development of SHS in patients with lupus, especially when receiving immunosuppressive therapy, is a severe and potentially fatal complication. Early detection and treatment may decrease mortality.

Vanishing Lung Syndrome in a Patient with HIV Infection and Heavy Marijuana Use

Vanishing lung syndrome (VLS) is a rare and distinct clinical syndrome that usually affects young men. VLS leads to severe progressive dyspnea and is characterized by extensive, asymmetric, peripheral, and predominantly upper lobe giant lung bullae. Case reports have suggested an additive role of marijuana use in the development of this disease in young male tobacco smokers. We herein report a case of a 65-year-old Hispanic male previously diagnosed with severe emphysema and acquired immune deficiency syndrome (AIDS), with a history of intravenous heroin use and active marijuana smoking who presents to the emergency department with severe progressive shortness of breath he was found to have multiple large subpleural bullae occupying more than one-third of the hemithorax on chest computerized tomography (CT), characteristic of vanishing lung syndrome. The patient was mechanically ventilated and later developed a pneumothorax requiring chest tube placement and referral for surgical bullectomy. Surgical bullectomy has shown high success rates in alleviating the debilitating symptoms and preventing the life threatening complications of this rare syndrome. This case further emphasizes the importance of recognizing VLS in patients with severe emphysema and heavy marijuana smoking.

Spontaneous coronary artery dissection with multiple coronary artery aneurysms in a patient with diabetic ketoacidosis

Spontaneous coronary artery dissection (SCAD) is a very rare and commonly fatal condition that typically presents with signs and symptoms of an acute myocardial infarction and cardiogenic shock. We herein present a case of a 54-year-old woman who was diagnosed with diabetic ketoacidosis (DKA) as the first manifestation of her underlying diabetes mellitus, while being hospitalized for treatment, she was found to have a non ST-elevation myocardial infarction (NSTEMI), an urgent cardiac catheterization revealed a complete dissection of the right coronary artery (RCA) with an intraluminal thrombus formation and multiple aneurismal dilatations in the left anterior descending (LAD) and left circumflex (LC) arteries. The patient underwent coronary artery bypass grafting with a favorable outcome.

Food Particle Aspiration Associated with Hemorrhagic Shock: A Diagnostic Dilemma

The hemodynamic compromise caused by a large aspirated food particle in the airway can become the focus of medical attention and a distraction from rare but fatal Heimlich maneuver related injuries after an incident of food aspiration. We herein present a case of an 84-year-old man who was brought to the emergency department after an episode of choking at a restaurant followed by several failed Heimlich maneuver attempts. Despite relieving the airway obstruction by extracting a large piece of steak from the airway, the patient remained hypotensive and required continued hemodynamic support. Repeated laboratory tests within 24 hrs of aspiration showed a significant decline in the hemoglobin level. A computed tomography (CT) scan of the abdomen and pelvis showed a lacerated liver with a large subcapsular hematoma draining into the pelvis. Conclusion. Hepatic rupture is a rare complication of Heimlich maneuver; this paper represents the second case report in the literature. It emphasizes the necessity of early identification and surveillance of fatal Heimlich maneuver complications in a high risk population.

The "Inverted Square Root Sign": A Sign of Critical Variable Central Airway Obstruction During Mechanical Ventilation.

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