Case for diagnosis. Infiltrated areas on the trunk

Abstract Leprosy is an infectious disease with chronic evolution, caused by Mycobacterium leprae, an acid-fast bacillus that mainly affects the skin and peripheral nervous tissue. Many of the clinical manifestations of leprosy can mimic connective tissue diseases. The authors present the case of a 49-year-old woman who had been treated for four years for systemic lupus erythematosus in a rheumatological service. Skin biopsy of a plaque on the inguinal region was compatible with borderline lepromatous leprosy associated with a type 1 lepra reaction. The patient is undergoing treatment with multibacillary multidrug therapy, showing clinical improvement.

Case for diagnosis. Infiltrated areas on the trunk.

Leprosy is an infectious disease with chronic evolution, caused by Mycobacterium leprae, an acid-fast bacillus that mainly affects the skin and peripheral nervous tissue. Many of the clinical manifestations of leprosy can mimic connective tissue diseases. The authors present the case of a 49-year-old woman who had been treated for four years for systemic lupus erythematosus in a rheumatological service. Skin biopsy of a plaque on the inguinal region was compatible with borderline lepromatous leprosy associated with a type 1 lepra reaction. The patient is undergoing treatment with multibacillary multidrug therapy, showing clinical improvement.

Erasmus syndrome: silicosis and systemic sclerosis.

The silicosis is the pneumoconiosis more frequent, resulting from the inhalation of silica or silicates containing mineral dust, mainly characterized by irreversible lung fibrosis. It is associated with the development of other diseases, including pulmonary tuberculosis, lung cancer and autoimmune diseases. The connective tissue disease after exposure to silica occurs usually after 15 years of initial exposure.The Erasmus syndrome describes the association of systemic sclerosis following exposure to silica with or without silicosis. The authors report two cases of patients with diagnosis of silicosis who developed systemic sclerosis.

Síndrome de Erasmus: silicose e esclerose sistêmica / Erasmus syndrome: silicosis and systemic sclerosis

A silicose é a pneumoconiose mais frequente, decorrente da inalação de sílica ou de poeiras minerais contendo silicatos, caracterizada principalmente pela fibrose pulmonar de caráter irreversível. Está associada com o desenvolvimento de outras doenças, incluindo tuberculose pulmonar, câncer pulmonar e doenças autoimunes. A doença do tecido conjuntivo posterior à exposição à sílica ocorre geralmente após 15 anos do início da exposição. A síndrome de Erasmus refere-se ao desenvolvimento de esclerose sistêmica em indivíduos previamente expostos à sílica e que apresentavam ou não silicose. Os autores relatam dois casos de pacientes com diagnóstico de silicose que desenvolveram esclerose sistêmica.

The silicosis is the pneumoconiosis more frequent, resulting from the inhalation of silica or silicates containing mineral dust, mainly characterized by irreversible lung fi brosis. It is associated with the development of other diseases, including pulmonary tuberculosis, lung cancer and autoimmune diseases. The connective tissue disease after exposure to silica occurs usually after 15 years of initial exposure.The Erasmus syndrome describes the association of systemic sclerosis following exposure to silica with or without silicosis. The authors report two cases of patients with diagnosis of silicosis who developed systemic sclerosis.

Paget's disease of bone and its complications due to delay in diagnosis.

Paget's disease of bone is an osteometabolic focal disease characterized by defects in bone remodeling. It may be asymptomatic, but often is associated with bone pain, deformity, pathological fracture, secondary osteoarthrosis and deafness. The diagnosis is usually made by radiological and laboratory findings. This report describes a male patient, 74 years old, native of Amazon, without European ancestry, with polyostotic Paget's disease, with clinical, radiological and laboratory diagnosis after 30 years of disease. The authors emphasize several complications of Paget's disease due to delayed diagnosis and the rarity of the disease in this population group.

[Acute pancreatitis and spontaneous rupture of pancreatic pseudocyst in systemic lupus erythematosus]. / Pancreatite aguda e rutura espontânea de pseudocisto pancreático em paciente com lúpus eritematoso sistêmico.

Systemic Lupus Erythematosus (SLE) is an autoimmune disease, with multisystemic involvement. Gastrointestinal symptoms are common, like nausea, vomiting and dyspepsia. Acute pancreatitis is an unusual manifestation of SLE, being an important differential diagnosis in evaluation of abdominal pain. The patients usually presents with pain of variable intensity, some occasions simulating acute abdomen. Several factors have been implicated in the pathogenesis of this condition, such as vasculitis, drugs and antiphospholipid antibodies. The role of corticosteroids as etiologic factor remains controversial. Due to the rarity of SLE associated to pancreatitis, we report two cases of patients with severe inflammatory process. In one case, it was used corticosteroids in high doses during treatment, with good outcome. In another, the patient died because of pancreatic pseudocyst rupture and its postoperative hemodynamic complications. In the reported cases, predisposing factors for acute pancreatitis were not verified, so it was considered a primary manifestation of SLE activity.

[Bilateral femoral fracture secondary to a severe osteomalacia in a patient with renal tubular acidosis type II]. / Fratura femoral bilateral secundária à osteomalácia grave em paciente com acidose tubular renal tipo II.

Renal tubular acidosis is a rare disease that can present in a primary, resulting from genetic defects in transport mechanisms of the renal tubules, or secondary, consequent to systemic diseases or drugs. The authors report a case of a patient with renal tubular acidosis type II who developed bilateral femoral fracture secondary to severe osteomalacia, with the intention of highlighting the importance of understanding this disease since the late diagnosis and treatment may generate serious repercussions for the patient.

Devastating skeletal effects of delayed diagnosis of complicated primary hyperparathyroidism because of ectopic adenoma.

Primary hyperparathyroidism is a disease caused by exaggerated secretion of the parathyroid gland hormone, produced by an adenoma in 80% of cases. Ectopic adenomas occur in a small proportion of cases. Herein, the authors report a 72-year-old woman with a delayed diagnosis of primary hyperparathyroidism, produced by an intrathoracic adenoma, with a longstanding course, presenting with severe osteoporosis, multiple fractures, bone deformities, and neurologic impairments. Persistent hypercalcemia, high levels of alkaline phosphatase, and parathyroid hormone were documented and a paratracheal mass was found on a helicoidal tomography of the thorax. After surgical removal, the histopathological examination confirmed an ectopic adenoma of the parathyroid gland and the patient achieved some improvement in her clinical picture.