The diagnosis of Langerhans Cell Histiocytosis (LCH) is based on histological and immunophenotypic examination of lesional tissue. The main feature is the morphologic identification of the characteristic LCH cells. Additionally, positive staining of the lesional cells with CD1a and/or Langerin (CD207) is required for definitive diagnosis. Continue reading
The Catastrophic Antiphospholipid Syndrome (CAPS) is a rare life-threatening form of Antiphospholipid Syndrome (APS) in which widespread intravascular thrombosis results in multiorgan ischemia and failure. CAPS is the initial presentation of APS in nearly half of patients, while the remaining half has a history of APS. Continue reading
SLICC classification criteria improved the clinical relevance of the ACR criteria, incorporated recent findings on the immunology of SLE, and resolved several problems attributed to the ACR criteria.
Gout, which is characterized by deposition of monosodium urate monohydrate (MSU) in synovial fluid and other tissues, is the most common form of inflammatory arthritis. Continue reading
POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder (PCD). The acronym, which was coined by Bardwick in 1980, refers to several, but not all, of the features of the syndrome: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal PCD, and skin changes. Continue reading
A diagnosis of primary Sjögren’s syndrome is often made on the basis of a classic triad of symptoms: dryness of the mouth and eyes, fatigue, and pain. Systemic complications, which are present in 30 to 40% of patients, may provide the first clues to the disease.
The National Institute on Aging and the Alzheimer’s Association (NIA-AA) charged a workgroup with the task of developing criteria for the symptomatic predementia phase of Alzheimer’s disease (AD), referred to in this article as mild cognitive impairment due to AD. Continue reading
This simple clinical case definition of fibromyalgia correctly classifies 88.1% of cases classified by the ACR classification criteria, and does not require a physical or tender point examination. Continue reading
Perry syndrome is characterised by rapidly progressive parkinsonism often accompanied by depression/apathy, weight loss and central hypoventilation. Continue reading
The 2017 McDonald criteria continue to apply primarily to patients experiencing a typical clinically isolated syndrome, define what is needed to fulfil dissemination in time and space of lesions in the CNS, and stress the need for no better explanation for the presentation. Continue reading