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A 40-year-old man presented with headaches and blurred vision for 5 weeks. His blood pressure was very high at 210/130. Examination found his vision was severely impaired at 20/200 in both eyes. Further workup revealed hypertensive retinopathy with cotton-wool spots and hemorrhages in both eyes. The patient was diagnosed with severe hypertension and hypertensive retinopathy causing vision loss. Treatment to lower his blood pressure was started.
Osce
- 2. Osce 1 • A 66-year-old man presented to the clinic with a 4-week history of painful, edematous, and scaly ears, a condition that was later accompanied by itchy, hyperkeratotic plaques on the palms and soles with surrounding erythema (Panels A, B, and C). The skin lesions were noted to be symmetric. Previous treatment with a 0.05% formulation of clobetasol cream was unsuccessful.
- 4. QUS • What is the most likely diagnosis associated with symptoms of pruritic skin changes seen in these images? • Malignancy • Psoriasis • Inflammatory bowel disease • Acute hepatitis C infection • Cutaneous sarcoidosis
- 5. • Acrokeratosis paraneoplastica presents with skin changes in the hands, feet, nose, and ears. Periungal tissues may also be involved. It is associated with malignancies in the upper respiratory or gastrointestinal tracts. Cutaneous changes typically involve psoriasiform dermatitis with surrounding erythema.
- 6. BAZEX syndrome • Acrokeratosis paraneoplastica of Bazex (also known as "Acrokeratosis neoplastica," and "Bazex syndrome") is a cutaneous condition characterized by psoriasiform changes of hands, feet, ears, and nose, with involvement of the nails and periungual tissues. • The condition is associated with carcinomas of the upper aerodigestive tract • The pathophysiology of acrokeratosis paraneoplastica is not understood. Proposed mechanisms include cross-reactivity between skin and tumor antigens, the action of tumor-produced growth factors, and even zinc deficiency.
- 7. • Approximately 140 cases of acrokeratosis paraneoplastica have been reported in the literature. • Morbidity and mortality in acrokeratosis paraneoplastica are related directly to the underlying neoplasm. The skin lesions of acrokeratosis paraneoplastica rarely improve without successful treatment of the underlying malignancy. • Most acrokeratosis paraneoplastica cases are associated with squamous cell carcinoma (SCC) of the upper one third of the respiratory or GI tracts (ie, oropharynx/larynx, lungs, esophagus) • Treatment of acrokeratosis paraneoplastica depends on the type and stage of the underlying neoplasm. Successful treatment of the underlying malignancy typically improves the skin lesions.
- 8. • Improvement has been reported with the use of systemic retinoids, topical and oral corticosteroids, salicylic acid, topical vitamin D analogues, and psoralen plus UVA (PUVA) • One report suggests a role for zinc supplementation in patients with acrokeratosis paraneoplastica
- 9. Osce 2 • Which of the following is the most likely cause of a painless swelling seen in this image? • Coronary angiography • Median nerve compression • Gout flare • Rheumatoid arthritis • Bullous pemphigoid
- 11. Pseudoaneurysms • Pseudoaneurysms occur infrequently as a complication of transradial coronary angiography. • Predisposing factors include the use of a large catheter sheath, multiple punctures at the site, the use of antiplatelet and antithrombotic agents, inadequate hemostasis or postprocedure compression, and vascular-site infection. The patient underwent surgical excision of the pseudoaneurysm and suture of the radial artery (next slide). • She remained well at follow-up visits both 1 week and 2 months after the procedure, with preserved radial arterial pulse and no residual swelling.
- 14. Osce 3 • What is the name for this finding that is reducible on physical exam? • Heberden’s nodes • Rheumatoid nodules • Jaccoud’s arthropathy • Pseudogout tophi • Erosive arthropathy of rheumatoid arthritis
- 15. • In Jaccoud’s arthropathy, the deformities are reducible and result mainly from soft-tissue abnormalities, such as laxity of ligaments, fibrosis of the capsule, and muscular imbalance, rather than from destruction of the bone of joints, as occurs in rheumatoid arthritis. • the condition characterised clinically by 'reversible' joint deformities such as swan neck, thumb subluxation, ulnar deviation, along with an absence of articular erosions on a plain radiograph. • JA was initially described in patients with rheumatic fever (RF), but as this disorder has become rare the main clinical entity associated to JA at present is systemic lupus erythematosus (SLE). JA has also been described in other connective tissue diseases, infections and neoplasia. In general, its prevalence in either SLE or RF is around 5%.
- 16. • The etiopathogenic mechanisms of JA are not known, but some authors have suggested an association with hypermobility syndrome. • Presently, the therapy for JA is conservative and based on the use of non-hormonal anti-inflammatory drugs, low doses of corticosteroids, methotrexate and antimalarials. • The role of surgery through either the realignment of soft tissue around the joint--or more aggressive procedures such as arthrodesis, silastic implant and arthroplasty--needs to be proven.
- 17. A 1-year-old girl presented with a history of growth failure, mild anemia, hypophosphatemia, hypovitaminosis D, glucosuria, and proteinuria. She underwent diagnostic bone marrow aspiration,
- 18. Cystinosis • Bone marrow revealed an increased proportion of macrophages containing polygonal crystals (20%; normal range, 2 to 5%), which are pathognomonic for cystinosis • Cystinosis is a lysosomal storage disease characterized by the abnormal accumulation of the amino acid cystine. • It typically follows an autosomal recessive inheritance pattern. Cystinosis is the most common cause of Fanconi syndrome in the pediatric age group.
- 19. • There (are) three distinct types of cystinosis each with slightly different symptoms: nephropathic cystinosis, intermediate cystinosis, and non- nephropathic or ocular cystinosis. • The signs and symptoms of intermediate cystinosis are the same as nephropathic cystinosis, but they occur at a later age. Intermediate cystinosis typically begins to affect individuals around age twelve to fifteen. Malfunctioning kidneys and corneal crystals are the main initial features of this disorder. If intermediate cystinosis is left untreated, complete kidney failure will occur. • People with non-nephropathic or ocular cystinosis do not usually experience growth impairment or kidney malfunction. The only symptom is photophobia due to cystine crystals in the cornea.
- 20. • Cystinosis is normally treated with a drug called cysteamine (brand name Cystagon). • The administration of cysteamine can reduce the intracellular cystine content. Cysteamine concentrates inside the lysosomes and reacts with cystine to form both cysteine and a cysteine-cysteamine complex, which are able to leave the lysosomes, When administered regularly. • Cysteamine eye drops remove the cystine crystals in the cornea that can cause photophobia if left unchecked. • Patients with cystinosis are also often given sodium citrate to treat the blood acidosis, as well as potassium and phosphorus supplements..
- 21. Case 5 • A 70-year-old woman who presented to the clinic for a routine examination was found to have a 6-cm mass on her hard palate. She reported that the mass had been present since birth.
- 23. She reported that the mass had been present since birth. Occasionally, she found that the area was hard to clean and that entrapped food gave her halitosis, but otherwise she was asymptomatic. Palatal tori are seen in approximately 20 to 30% of the population and are more common in females than in males. Frequently, these bony outgrowths are incidental findings on routine oral examinations, and there is often considerable variation in clinical appearance. The lesion in this patient was unchanged over a 2-year period of follow-up. Surgical correction was offered, but the patient declined because the lesion was asymptomatic.
- 24. Case 6 • A 9-year-old boy with a history of a lung lesion presented to the emergency department with fever and chest pain. ..
- 25. A chest radiograph (Panel A) showed a large opacity in the right lung, abutting the minor fissure and displacing it downward
- 26. Computed tomography of the chest revealed a large heterogeneous mass with scattered areas of low attenuation, suggesting mucous impaction, and a single large systemic artery (Panel B, arrow) providing vascular supply from the descending aorta. No air bronchograms were evident within the mass.
- 27. PULMONARY SEQUESTRATION • Pulmonary sequestration is a congenital disorder characterized by anomalous lung tissue that lacks normal communication with the tracheobronchial tree, which increases the likelihood of infection. • The patient underwent a right upper lobectomy. • The patient recovered without complications and since the operation has had no further pulmonary problems.
- 28. Case 7 • An 83-year-old man was evaluated for a 1- year history of a pruritic, progressively worsening migratory rash, with associated weakness and a 5-kg weight loss. He had a 30-pack-year history of smoking; he had stopped smoking 45 years earlier. …
- 29. Erythema gyratum repens On examination, he had erythematous skin lesions that appeared in concentric, raised, serpiginous bands, with desquamation (Panel A). The rash affected mainly the trunk and proximal extremities.
- 30. A computed tomographic scan revealed a pulmonary mass measuring 59 by 43 mm (Panel B). Bronchoscopy with biopsy revealed squamous-cell carcinoma of the lung
- 31. Erythema gyratum repens is a rare syndrome typically associated with an underlying malignant condition. It occurs most frequently in conjunction with lung cancer and next most frequently with esophageal and breast cancers. It may regress with treatment of the cancer. Treatment with gemcitabine was initiated for this patient, but he died 3 months after diagnosis, after only one infusion; the rash had not resolved.
- 32. Case 8 • A 40-year-old man presented with a 5- week history of headaches and blurred vision in both eyes, with no other medical history. • He reported having no chest pain or shortness of breath. • A physical examination was notable for a blood pressure of 210/130, and the visual acuity was 20/200 in both eyes.
- 33. Retinopathy in malignant hypertension Fundus examination revealed optic-disk hyperemia and blurring (long arrow), retinal hemorrhage (short arrow), a macular “star” with lipid deposits (arrowhead), and venous congestion, in both the right eye (Panel A) and the left eye (Panel B)
- 35. The patient was admitted to the hospital and given urgent care. Patients with malignant hypertension, such as our patient, have a diastolic blood pressure of 130 mm Hg or higher in association with ocular pathologic features. These ocular findings can also be caused by blood dyscrasias, systemic lupus erythematosus, neuroretinitis, sarcoidosis, or eclampsia. Changes in the retina may result in permanent loss of vision. This patient was given atenolol, amlodipine, and enalapril for the high blood pressure. One month later, the fundus showed clinically significant improvement Two months after presentation, the patient's blood pressure was normal (128/80 mm Hg), and his visual acuity had improved to 20/60.