Seminarios de la Fundación Espa?ola de Reumatología最新文献

Necrotizing fasciitis is defined as a rapidly progressive infection of the skin and soft tissue that usually involves severe systemic toxicity. The incidence of this infection has increased in the last few decades and is estimated to affect one out of every 100,000 inhabitants in western European countries. This disease is the most serious form of skin and soft tissue infection, due to rapid destruction and necrosis of the fascia and subcutaneous fat, and the development of shock and multiorgan failure in about one third of patients.

Although there are several predisposing factors for the development of the disease, especially for type I, or polymicrobial, necrotizing fasciitis, many patients are young and have no underlying chronic diseases, as is the case for type II, or streptococcal, necrotizing fasciitis. The diagnosis is mainly clinical, and urgent surgical consultation is required as soon as possible once suspicion is high, as the main determinant of mortality is the delay in surgical treatment. Overall mortality remains high, affecting more than 25% of patients. Surgical debridement is the mainstay of treatment, along with hemodynamic support and broad-spectrum antibiotics.

Limbus vertebrae, first described by Schmorl in 1927, is a triangular smooth bone fragment affecting the anterosuperior corner of a single vertebral body and results from herniation of a portion of the nucleus pulposus through the ring apophysis during childhood or adolescence.

The radiological appearance of the limbic vertebra is characteristic and simple x-ray is usually sufficient to complete the diagnosis.

Limbus vertebrae can be mistaken for a fracture, infection or tumor. Clinicians should have sound knowledge of this anomaly so that unnecessary procedures and treatments can be avoided.

This article reviews the association between osteoporosis and pregnancy, as well as the two forms found in clinical practice. This association has been poorly investigated, the information arising from isolated clinical cases or from case series. Two, possibly related, forms are described in pregnancy: systemic and regional osteoporosis. The former mainly affects trabecular bone, while the latter affects load territories, frequently the hip. The pathophysiology is unknown, but probably differs in each case. Nonetheless, in both entities there is a common base of susceptibility, with osteopenia or osteoporosis. The rationale for the systemic form is based on current knowledge of the modifications of bone metabolism in pregnancy, when there is a significant increase in vitamin D, and minor changes in other potent modulators, such as parathyroid hormone.

Regional osteoporosis is similar in semiology and progression to severe diseases, such as avascular necrosis, and a similar mechanism in which regional osteoporosis would only be a stage in progression cannot be discarded. There is a scattered increase of resorption, which creates multiple regions of fragility where microfractures accumulate and bone fragility increases. Diagnosis requires clinical suspicion, which should lead to correct diagnosis in both forms. Treatment is symptomatic and, if possible, is based on the release of load. The use of anti-resorptives, and particularly of bisphosphonates, is limited during pregnancy, since these drugs pass the placenta. In both forms, restitution is progressive and relatively rapid after delivery.

Fibroblastic rheumatism is a rare disease of unknown cause and is characterized by the association of aggressive polyarthritis, sclerodactyly and the presence of cutaneous nodules. The arthritis is rapidly progressive, leading to loss of joint function with flexion contractures of the fingers. The cutaneous lesions are characterized by singular histological findings and consequently their diagnosis is mainly histopathological. Biopsy of a nodule or thickened skin typically reveals increased thickness of collagen fibers and fibroblastic proliferation. The differential diagnosis includes diseases such as rheumatoid arthritis, nodular scleroderma, and multicentric reticulohistiocytosis, among others. Treatment has not yet been consolidated, although most cases have been treated with prednisone and methotrexate with good results. The prognosis is generally good, without functional repercussions, especially if early diagnosis and treatment are provided.

Perniosis consists of the development of inflammatory, erythematous and frequently pruriginous lesions on acral sites after contact with cold. These lesions can occur as an isolated disorder in an otherwise healthy individual or in the context of various disorders such as anorexia nervosa or other diseases associated with low body weight, hematological processes — especially myelodysplastic syndrome — the presence of antiphospholipid antibodies and even some genetic diseases in which there is immune dysregulation, such as Aircadi-Goutières disease. The term idiopathic perniosis is used to designate patients with no underlying systemic disease.

Diagnosis is clinical, although microscopic study of a biopsy sample can be useful in specific circumstances. Perniosis should be distinguished from other, very different processes that may nevertheless cause lesions with a similar appearance. Among these are chilblain lupus, which still lacks a precise definition and is difficult to distinguish from idiopathic perniosis. Treatment is disappointing and is essentially based on topical corticosteroids and oral calcium channel blockers such as nifedipine and felodipine