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Small vessel vasculitis
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Renu George, Ankan Gupta, Aswin M. Nair
The predominant cutaneous symptom is a long-lasting wheal associated with a burning sensation or tenderness that resolves with pigmentation. Other lesions seen include angioedema, purpura, erythema multiforme–like eruptions, bullous eruptions, Raynaud phenomenon, and/or livedo reticularis [33]. The systemic associations include musculoskeletal system, eye, lungs, and kidneys. Migratory, transient arthralgias and arthritis are the most common systemic manifestations. Jaccoud arthropathy is rarely seen and may be associated with aortic and mitral valvulopathy, requiring echocardiographic evaluation [34,35]. The renal involvement of UV tends to be more severe in children [36]. Rare complications include aseptic meningitis and cranial nerve palsies.
Case 17: Painful Hands
Published in Iqbal Khan, Medical Histories for the MRCP and Final MB, 2018
Symptoms of SLE can be extremely diverse, affecting many different systems. Hence the condition may present under different specialists, perhaps masquerading as another illness. Systems affected include the following.Musculoskeletal: arthralgia and arthritis, but pain is worse than the physical signs. A deforming arthropathy (described as Jaccoud’s arthropathy) may occur.Renal: disease can range in severity from a benign, focal glomerulitis to a potentially fatal, diffuse membranoproliferative glomerulonephritis.Dermatological: the butterfly rash over the cheeks is well recognised. It may be photosensitive. Other lesions include discoid lupus, scarring alopecia, Raynaud’s disease, livedo reticularis and mucosal (e.g. oral, vaginal) ulcers.Cardiovascular: hypertension, pericarditis, Libman-Sacks endocarditis.Respiratory: cough, dyspnoea, pleuritic chest pain and haemoptysis may all occur.Gastrointestinal: serositis can lead to pain. Impaired motility causes nausea, vomiting and even pseudo-obstruction. Patients may complain of anorexia and weight loss.Neurological: long-standing headaches and memory problems are common. Patients may have a history of seizures. Occasionally, there may be associated psychosis. There may be problems with the vision associated with retinal vasculitis. The most devastating problem is that of strokes.Haematological: Coomb’s positive haemolysis, leucopoenia, thrombocytopaenia.Systemic: fever, lethargy, generalised lymphadenopathy, splenomegaly.
Functional reconstruction of a hand that was severely deformed due to Jaccoud’s arthropathy
Published in Modern Rheumatology Case Reports, 2021
Kei Funamura, Hajime Ishikawa, Rika Kakutani, Asami Abe, Hiroshi Otani, Kiyoshi Nakazono, Akira Murasawa
Santiago reported that Jaccoud’s arthropathy was categorised to the other non-inflammatory musculoskeletal conditions and was generally observed in not only the hand but also the foot [5], knee [6], and shoulder [7]. The most common joint deformity was ulnar deviation, swan-neck deformity and boutonniere deformity of the fingers, Z deformity of the thumb, and hallux valgus deformity [8]. Jaccoud’s arthropathy is characterised by "reversible" joint deformity, unlike rheumatoid arthritis (RA), which causes irreversible deformity with joint destruction. The deformity is caused by a loss of mechanical balance following not synovitis in the joint but weakening, elongation, and disappearance of the supporting soft tissue structures, such as the ligaments, tendons, and joint capsules [8].
Arthroplasty for severe deforming arthropathy in overlap syndrome of systemic lupus erythematosus and anti-Jo-1 antibody positive dermatomyositis
Published in Modern Rheumatology Case Reports, 2018
Kenjiro Fujimura, Hiromichi Mitsuyasu, Motoko Ishida, Koji Sakuraba, Masataka Nakamura, Tomoya Miyamura, Satoshi Kamura, Eiichi Suematsu, Hisaaki Miyahara
Deforming arthropathy is rare in connective tissue diseases, while arthralgia occurs frequently. One of the most well-known types of deforming arthropathy is Jaccoud’s arthropathy (JA), which mainly occurs in patients with systemic lupus erythematosus (SLE) [1]. Deforming arthropathy is also found in patients with polymyositis/dermatomyositis (PM/DM), which is reportedly associated with the presence of anti-Jo-1 antibody [2–7].