A Rare Case of Severe Stevens-Johnson Syndrome Triggered by Topical Ofloxacin.

BACKGROUND Stevens-Johnson syndrome (SJS) is a rare dermatologic disorder that is characterized by nonspecific flu-like prodrome with fever, malaise, myalgia, cough, rhinitis, and sore eyes, followed by a characteristic rash and mucocutaneous manifestations. It is triggered by medications in up to 80% of cases in adults. In each of these cases, the medication is oral or parenteral. Severe and progressive SJS can result in life-threatening complications. Adult-onset medication-induced SJS presents within 8 weeks of exposure to the offending substance, lasting 8 to 12 days. Recovery of denuded skin generally is complete within a month. There is no consensus on treatment, but supportive care with corticosteroids is often the initial intervention. CASE REPORT A 36-year-old woman with a flare of allergic rhinitis and tearing resistant to over-the-counter options was treated with topical ophthalmic ofloxacin. She began experiencing a diffuse mucocutaneous rash, with oral desquamation, tongue swelling, vaginal desquamation, and rash of the palms and soles within 24 h, which suggested the possibility of SJS. A skin biopsy was obtained, and pathology confirmed this suspicion. She was treated with parenteral antibiotics, corticosteroids, and supportive care, and after 10 days was discharged from the hospital. She had a complete recovery in 30 days. CONCLUSIONS The clinical course of SJS induced by the ophthalmic application of medication can be just as severe as the oral or parenteral routes. This is, to the best of our knowledge, the first documented case of SJS being triggered by topical ofloxacin.

Unusual Case of Ludwig Angina Caused by Clostridium sporogenes in an Immunocompromised HIV-Positive Patient with Alcoholism and Dental Abscess.

BACKGROUND Ludwig angina is a cellulitis of the soft tissues of the neck and floor of the mouth. It is most commonly caused by Viridans streptococcal species, but other bacterial species have been shown to lead to this severe infection. Clostridium sporogenes is an anaerobic gram-positive, spore-producing bacillus found in soil and the human gastrointestinal tract. This report is of a case of a 49-year-old HIV-positive man with alcoholism and poor dental hygiene leading to a molar abscess who presented with Ludwig angina due to C. sporogenes. CASE REPORT A 49-year-old man presented with severe left molar pain, fever, and worsening neck swelling for 5 days. His medical history was significant for AIDS; he was not on antiretroviral therapy. Computed tomography of the neck was positive for extensive subcutaneous emphysema of the left sublingual space. Ludwig angina was diagnosed, and he was taken urgently for incision and drainage of the bilateral neck fascial space. On day 6 of hospitalization, 1 of 2 blood cultures grew C. sporogenes. He left the hospital on day 13 and was readmitted 6 days later with progression of the disease and alcohol withdrawal. CONCLUSIONS This case illustrates the need for rapid diagnosis and treatment of Ludwig angina and the importance of considering commonly pathogenic and rarely pathogenic bacteria when considering the underlying bacterial cause of an infection in an immunocompromised patient. To the best of our knowledge, this is the first case of Ludwig angina caused by C. sporogenes reported in the medical literature.

Pseudo-Thrombotic Microangiopathy Secondary to Vitamin B12 Deficiency.

Background: Clinical B12 deficiency with hematological or neurological manifestations is rare. An unusual manifestation of B12 deficiency is pseudo-thrombotic microangiopathy (TMA), which is characterized by hemolytic anemia, thrombocytopenia, and schistocytosis and only occurs in 2.5% of those with B12 deficiency. Pseudo-TMA is misdiagnosed as thrombotic thrombocytopenic purpura (TTP) in 40% of cases, resulting in misguided treatment including plasmapheresis. Case: A 44-year-old Hispanic presented with 3 weeks of progressively worsening non-radiating chest pain, fatigue, and shortness of breath (SOB). Laboratory findings revealed severe pancytopenia and macrocytosis with a hemoglobin of 5.4 g/dL, mean corpuscular volume of 116.3 fL, and vitamin B12 low at 149 pg/mL. She was diagnosed with pseudo-TMA and after starting 1000 micrograms of parenteral vitamin B12 injections daily and discontinuing plasmapheresis and steroid administration, she improved. Conclusion: Failure to recognize pseudo-TMA often results in unnecessary treatment with plasmapheresis and delays appropriate treatment with vitamin B12 supplementation. It is therefore extremely important to consider pseudo-TMA as a differential diagnosis in patients that present with hemolytic anemia, thrombocytopenia, and schistocytosis.

A 24-Year-Old Man Presenting with Lung Metastases from a Primary Retroperitoneal Extragonadal Choriocarcinoma.

BACKGROUND Primary retroperitoneal choriocarcinoma is a rare form of extragonadal germ cell tumor that is highly aggressive and responds poorly to chemoradiation. Extragonadal choriocarcinomas are notoriously challenging to diagnose, and have often progressed to advanced disease by the time of diagnosis. The survival rate for extragonadal choriocarcinoma is approximately 30%, which is much lower than that of extragonadal non-seminomatous germ cell tumors (GCT) in general. CASE REPORT A 24-year-old man with no significant past medical history presented with left-sided, pleuritic chest pain and back pain radiating down his left leg, of 1-year duration. Computed tomography (CT) of the chest revealed multiple bilateral pulmonary nodules and a CT of the abdomen and pelvis showed a large heterogeneous soft tissue mass measuring 9.3×8×10.5 cm. A CT-guided core needle biopsy of a lung nodule was performed and the findings were consistent with the diagnosis of metastatic choriocarcinoma. Magnetic resonance imaging (MRI) of the brain was negative for metastatic disease. Tumor markers were significant for a markedly elevated beta human chorionic gonadotropin (B-hCG) of 104 712 mIU/mL. He was diagnosed with a stage IIIC germ cell tumor, further classified as a primary retroperitoneal choriocarcinoma with lung metastasis, and was started on urgent inpatient chemotherapy. CONCLUSIONS Due to the poor outcomes associated with extragonadal choriocarcinoma, it is important to promptly and correctly identify this malignancy in order to initiate treatment in a timely manner. The following case report explores the histopathologic characterization of this malignancy and describes the clinical course and outcomes from treatment for this patient.

Systemic Lupus Erythematous and Neuromyelitis Optica Causing Hypercapnic Respiratory Failure.

BACKGROUND Neuromyelitis optica (NMO) is a rare neurological disease characterized by attacks of transverse myelitis and optic neuritis, contiguous spinal cord lesions on more than 3 vertebral segments on magnetic resonance imaging (MRI), and seropositivity for AQP-4 Ab. The tissue destruction from NMO is immune mediated and results in demyelination and axonal damage. Optic and spinal nerve involvement can eventually lead to blindness, weakness, and altered consciousness, and bladder and bowel involvement in some cases. CASE REPORT A 54-year-old Black woman presented with chest pain, dysphagia, generalized weakness, diplopia, and paresthesias in her bilateral feet. A brain MRI revealed an area of hyperintensity in the cervical medullary junction. A diagnosis of NMO was made after the treatment response was poor for systemic lupus erythematous (SLE) myelitis. She eventually developed acute hypercapnic respiratory failure, became encephalopathic, and was emergently intubated. She was extubated but had poor recovery and was eventually discharged home. CONCLUSIONS NMO is a rare immune-mediated disease that is often delayed in diagnosis and treatment. Clinical suspicion is important since there is a tendency for the disease to overlap concomitant autoimmune diseases in 25% of cases. Progressive and permanent tissue damage can occur despite the use of high-dose steroids, long-term immunosuppressant agents, immunomodulators, exchange transfusions, and even autologous hematopoietic stem cell bone marrow transplantation.

Far migration of an intrauterine contraceptive device from the uterus to the small bowel.

A sexually active, asymptomatic 44-year-old presented for Intrauterine device (IUD) removal that had been in place for 13 years. IUD removal was unsuccessful as the strings could not be located. Imaging revealed an extrauterine IUD and at surgical removal of the abdominal IUD a small bowel perforation requiring bowel resection was required. Uterine perforation is a rare complication of IUD use occurring in approximately 1-1.3 in 1000. Risk factors for perforation include provider inexperience, retroverted uterus, immobile uterus, and myometrial defect from a previous cesarean delivery or myomectomy.

Left Ventricular Pseudoaneurysm and Left Ventricular Thrombus in a Patient Presenting with an Acute ST-Elevation Myocardial Infarction.

BACKGROUND ST-elevation myocardial infarction (STEMI), when associated with acute left ventricular (LV) free-wall rupture, is often a lethal complication, and if not followed by sudden death, the rupture may be contained by the parietal pericardium and a local thrombus, leading to the formation of a left ventricular (LV) pseudoaneurysm. The incidence of LV pseudoaneurysm after STEMI is ~ 0.3%. CASE REPORT A 73-year-old man who presented with an acute syncopal episode and intermittent chest pain for 7 days was found to have an anterolateral myocardial infarction (MI) with lateral wall rupture and pseudoaneurysm formation. He had an LV thrombosis in the LV aneurysm. While this increased his risk of thromboembolic events, it likely stopped the evolution of the rupture and stabilized the pericardial effusion size. The patient underwent coronary artery bypass grafting (CABG), thrombectomy, and lateral wall repair. CONCLUSIONS Left ventricular pseudoaneurysm and left ventricular thrombus in a patient presenting with an acute ST-elevation myocardial infarction is a rare complication of myocardial infraction, with an incidence of <1%. It is often a lethal complication and requires stabilization and repair if not followed by sudden death.

Extensive catastrophic thromboses from elevation of factor VIII.

Catastrophic thrombotic syndrome, otherwise known as thrombotic storm (TS) is an extreme prothrombotic clinical syndrome that presents as rapid onset of multiple thromboembolic events affecting a large variety of vasculature. In recent studies, there has been a correlation of high plasma levels of factor VIII with thrombotic events. We present the case of a young man who exhibited multi-organ failure due to thrombotic storm. A 38-year-old male presented to the emergency department for progressive dyspnea and was diagnosed to have pulmonary embolism. The patient developed respiratory distress requiring intubation and was diagnosed with both an ST-elevation myocardial infarction and right cerebral infarction during the hospital course. The patient expired and autopsy revealed the cause of death to be myocardial, cerebral and renal infarction from widespread vascular thrombosis. Autopsy revealed cause of death to be elevated factor VIII associated thrombotic coagulopathy. Factor VIII level upon autopsy was 375% (55-200%). Although TS is rare, it can be lifethreatening if not recognized early. Survival depends on the prompt initiation and duration of anticoagulation.

Hypercalcemia Resulting from Necrotizing Leiomyoma in a Pregnant Female.

BACKGROUND Hypercalcemic crisis is defined as a serum calcium level >14 mg/dL in a symptomatic patient. While severe hypercalcemia during pregnancy is rare, it poses a life-threatening risk to both mother and fetus. Hypercalcemia in association with a benign tumor such as a leiomyoma is exceedingly rare. CASE REPORT A 38-year-old primagravida at 31.2 week's gestation conceived by in vitro fertilization presented to the emergency department for complaints of nausea, vomiting, and epigastric abdominal pain. Her fetal monitor strip was reassuring. A complete metabolic panel on admission was significant for severely elevated calcium of 15.9 mg/dL (8.6-10.3 mg/dL) and an elevated lipase of 1457U/L (11-82 U/L). She was started on aggressive intravenous fluid resuscitation, but became confused and lethargic, unarousable to verbal stimuli, as a result of hypercalcemia. Computed tomography (CT) scan of the abdomen and pelvis revealed a heterogeneously enhancing, placental-appearing soft tissue mass extending posteriorly and to the right that measured 2414 cm. The patient subsequently underwent planned low transverse cesarean delivery and exploratory laparotomy for myomectomy with removal of a 2834-g benign leiomyoma measuring 19.018.514.0 cm. Her serum parathyroid hormone-related protein (PTHrP) was elevated to 9.6 pmol/L (<4.2 pmol/L). The patient's calcium normalized to 9.8 mg/dL (8.6-10.3mg/dL) immediately following surgery. CONCLUSIONS Leiomyoma as a cause of hypercalcemia should be included in the differential diagnosis because surgical removal of leiomyoma is curative. Particularly in pregnant patients, for whom medical therapies for hypercalcemia are limited and those available can result in complications, early identification and surgical resection can be life saving.

Portal vein aneurysm with acute portal vein thrombosis masquerading as a pancreatic mass.

Portal vein aneurysm (PVA) with portal vein thrombosis (PVT) is an exceedingly rare vascular phenomenon with a limited number of reported cases in the medical literature. We describe a case of a 25-year-old man found to have a congenital PVA with PVT initially believed to be a pancreatic mass. While there remains some incongruity amongst clinicians with such a limited number of reported cases, herein, we describe the general consensus of the diagnostic approach and management of this vascular malformation.

Epidural Steroid Injection-Induced Pancreatitis: A Case Report.

BACKGROUND Degenerative disc disease of the lumbar spine can be associated with spinal canal and neuroforaminal stenosis, resulting in severe pain. Conservative approaches to treatment are generally recommended initially, especially in the elderly. Epidural corticosteroid injections can provide significant but temporary pain relief and are a commonly performed procedure in pain management. Pancreatitis caused by corticosteroids is unusual and the prognosis typically is good. CASE REPORT A 73-year-old woman presented with severe intractable back pain 1 week after lumbar epidural steroid injection for symptomatic spinal stenosis. Imaging confirmed severe multi-level degenerative disc disease of the lumbar spine resulting in severe canal and bilateral neuroforaminal stenosis. Because of abdominal pain and nausea, an abdominal CT and labs were performed, revealing evidence of pancreatic inflammation. CONCLUSIONS Lumbar epidural steroid injection may be a risk factor for developing steroid-induced pancreatitis.

T Cell Primary Effusion Lymphoma in an HIV-Negative Man with Liver Cirrhosis.

BACKGROUND Primary effusion lymphoma (PEL) is a rare and aggressive non-Hodgkin lymphoma (NHL) that is responsible for 1% of all lymphomas not related to human immunodeficiency virus (HIV). PEL is characterized by human herpesvirus-8 (HHV-8) positivity in the absence of overt tumor burden that does not exhibit typical B cell or T cell immunophenotype characteristics. The exact mechanism of development is unknown, but it is hypothesized to develop from post-germinal B cell origin. Although it is most common in HIV patients, other immunocompromising comorbidities can be seen in conjunction with PEL, including liver cirrhosis. CASE REPORT We present the case of a 73-year-old HIV-seronegative man with alcohol-induced liver cirrhosis who was found to have T cell PEL of the pleural space diagnosed by thoracentesis. CONCLUSIONS Little is known regarding oncogenesis of T cell PEL, and few studies exist regarding appropriate treatment regimens for PEL as a whole, prompting need for further investigation and discussion to improve survival rates. Even in the absence of active HIV infection, PEL should be considered as a potential cause of pleural effusion in cirrhotic patients in order to prompt earlier treatment for the best chance of survival.

Successful Treatment of Hypokalemic Rhabdomyolysis Caused by a Pancreatic VIPoma: A Case Report.

BACKGROUND VIPomas are rare neuroendocrine tumors typically located in the pancreas. The majority of cases autonomously secret vasoactive intestinal polypeptide (VIP), which can result in profuse, refractory, watery diarrhea. The fluid and electrolyte imbalance can progress to dehydration and profound hypokalemia, resulting in the watery diarrhea, hypokalemia, achlorhydria (WDHA) syndrome. One previous case of a pancreatic VIPoma progressing to hypokalemic rhabdomyolysis has been described. CASE REPORT A 33-year-old woman presented with 3 months of progressive, refractory diarrhea and weakness. Her serum VIP level was elevated and imaging discovered a mass in the region of the pancreatic tail. Laparoscopic partial pancreatic resection was performed and a 3.7-cm diameter, solitary stage T2 N0 M0, well-differentiated carcinoma was removed. CONCLUSIONS A high index of suspicion is important when diagnosing chronic diarrhea. Minimally invasive surgery is an option in the surgical treatment of pancreatic VIPoma.

Unilateral Giant Vulvar Lipoma with Fat Necrosis Not Visualized on Magnetic Resonance Imaging (MRI).

BACKGROUND A subcutaneous lipoma is a benign tumor comprised of mature adipocytes. Clinically it presents as a soft, freely moveable, doughy mass that is typically painless and slow growing. Lipomas containing fat necrosis and corresponding palpable nodular elements are rare and suggest an alternative diagnosis. Lipomas in the vulvar region are rare and giant vulvar lipomas with palpable fat necrosis are unreported. CASE REPORT A 25-year-old patient presented with a 4-year history of an enlarging right vulvar mass with multiple small (<1 cm) firm nodules within the tumor. A pelvic MRI (magnetic resonance imaging) did not visualize the nodules but histopathologic examination revealed a benign lipoma containing fat necrosis and discrete areas of calcium deposition. CONCLUSIONS Fat necrosis can occur in vulvar lipomas and present with intratumor nodularity. MRI imaging, clinical findings and histology may be discordant.

A Case of Thymic Carcinoma with Bone and Cerebral Metastases Treated with Stereotactic Radiosurgery and Chemotherapy.

BACKGROUND Thymic carcinoma is a rare malignant neoplasm. High-grade thymic carcinoma has a high recurrence rate following surgery, and a low 5-year survival rate. Approximately 30% of patients with thymic carcinoma will be asymptomatic at the time of diagnosis. Extrathoracic metastasis on presentation is uncommon. Treatment of the primary tumor includes surgery, chemotherapy, and fractionated radiation. A rare case of thymic carcinoma that presented with bone and cerebral metastases is reported in a patient who responded well to stereotactic radiosurgery and chemotherapy. CASE REPORT A 63-year-old woman presented to the hospital for evaluation of hip pain. She was diagnosed with a lytic bone lesion of the right femur and brain metastasis. Biopsies from the mediastinal mass and right femur showed histological features consistent with carcinoma. Immunohistochemistry showed positive immunostaining of the tumor cells for the c-kit receptor (CD117) and CD5, supporting a diagnosis of stage IVb thymic carcinoma. Treatment included stereotactic radiosurgery, which delivered multiple radiation beams to the tumor tissue from different directions to target the tumor without affecting normal tissues. She was treated as an outpatient with carboplatin and taxol after stereotactic radiosurgery. The patient recovered well following treatment. CONCLUSIONS A case of thymic carcinoma with bone and cerebral metastases was successfully treated with stereotactic radiosurgery and chemotherapy.

Plummer-Vinson Syndrome and Heart Failure: An Unusual Association in an African American Woman.

BACKGROUND Plummer-Vinson syndrome (PVS) is a rare disorder composed of the triad of dysphagia, iron-deficiency anemia (IDA), and esophageal webs. It is most prevalent in middle-aged white women, and the dysphagia often improves when the anemia is treated. It is well established that chronic hypertension can lead to congestive heart failure (CHF). While IDA is frequently found concomitantly with CHF, there have been no reported cases of new-onset CHF with anemia presenting as PVS. CASE REPORT We present the case of a 48-year-old African American woman with symptomatic anemia and new-onset congestive heart failure secondary to hypertension, who presented with the classic symptoms of PVS. CONCLUSIONS CHF with accompanying IDA may be an independent risk factor for the development of PVS. At the very least, there is an association between CHF-induced IDA and PVS. Patients presenting with CHF with symptoms of dysphagia should be considered at risk for the syndrome, and endoscopy may be warranted. Treatment for PVS includes iron replacement, and in some cases requires mechanical dilation.

Glassy Cell Carcinoma of the Endometrium Presenting as an Intracavitary Leiomyoma on Ultrasound.

BACKGROUND Glassy cell carcinoma of the endometrium is an extremely rare variant of adenosquamous carcinoma, and it has a poor prognosis. In postmenopausal women it typically presents as unprovoked, painless uterine bleeding. Tissue sampling is necessary to establish the diagnosis. CASE REPORT A 58-year-old postmenopausal woman on no hormone replacement therapy experienced 2 months of intermittent uterine bleeding. An office transvaginal ultrasound discovered a 1.7-cm intracavitary leiomyoma, but because the endometrial stripe was not visualized, an endometrial biopsy was performed. She was found to have a Stage 1 A endometrial poorly-differentiated adenosquamous carcinoma, glassy cell carcinoma tumor of 1.5 cm in greatest dimension. She underwent a robotic total hysterectomy, bilateral salpingo-oophorectomy, pelvic lymph node mapping, and bilateral pelvic lymphadenectomy. CONCLUSIONS Glassy cell carcinoma of the endometrium can present as an intracavitary leiomyoma in postmenopausal women.

Lymphatic Malformation of the Nasopharynx in a Young Pregnant Female: A Case Report.

BACKGROUND Lymphangiomas represent the focal proliferation of benign, well-differentiated lymphatic tissue. They are most likely congenital, thus more commonly diagnosed at birth and before the age of 2 years. When they are found in adults, they favor the head, neck and axillary region. Rarely do they involve the nasopharynx region or occur in a pregnant patient. CASE REPORT A 21-year-old primagravida in the third trimester of pregnancy developed difficulty swallowing that progressed into difficulty breathing over a 1-month period. Imaging and examination suggested a benign mass in the nasopharynx and the patient underwent surgical removal of the stalk with bipolar cautery. The pathology report revealed a simple 4.5×1.5×0.8 cm lymphangioma. She had no fetal compromise during anesthesia. CONCLUSIONS Surgical removal of a nasopharyngeal lymphangioma during the third trimester of pregnancy is indicated if respiratory obstruction may be a complication.

Idiopathic Thrombocytopenic Purpura Induced by Synthetic Cannabinoid.

: Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder defined as low platelet count with normal bone marrow in the absence of other causes of thrombocytopenia. It is caused by autoantibodies binding to several platelet surface antigens which lead to premature destruction of the platelet by the reticuloendothelial system. ITP can be primary or secondary, and it is known to be associated with various infections and drugs. K2/Spice, or synthetic marijuana, acts on cannabinoid receptors CB1 (cannabinoid type-1) and CB2 (cannabinoid type-2) with increased binding capacity compared to marijuana. A white male was brought to the emergency department with signs of acute drug toxicity. His urine drug test was negative but he admitted to a year long history of synthetic marijuana use. His platelet count was 12,000/mm and he was diagnosed with ITP. After receiving 2 doses of oral dexamethasone his platelets improved. Synthetic cannabinoids are widely available and used psychoactive drugs. Little is known about the complete chemical composition of the synthetic products thus there is relatively little information available on the pharmacodynamic and pharmacokinetic effects. A high index of suspicion is needed to diagnose toxicity to these drugs since there are no readily available on-site lab tests. Currently there exists 1 case report of ITP induced by K2/Spice. Here, we discuss another case of K2/Spice a as a potential cause of immune thrombocytopenia.

Rapidly Progressing Sporadic Creutzfeldt-Jakob Disease Presenting as a Stroke.

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, fatal human prion disease that is characterized by progressive dementia and neurologic degeneration. It can mimic multiple other neurological disorders, and a high index of clinical suspicion is necessary to make a diagnosis. A 74-year-old woman with a 3-month history of a stroke and progressive neurologic deterioration was found to have sCJD. She expired within a week of her diagnosis. Autopsy revealed spongiform encephalopathy consistent with prion disease, and genetic analysis revealed 129 polymorphism and no pathologic mutation, confirming the diagnosis of nonfamilial human prion disease. No pathologic evidence of a stroke was found. Awareness of the disease by clinicians is important not only at the time of initial presentation but also during the following months. Since there is no treatment, invasive medical procedures should be limited to only those that are required for either diagnosis or hospice care.