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Peripheral Autonomic Neuropathies
Published in David Robertson, Italo Biaggioni, Disorders of the Autonomic Nervous System, 2019
Some patients develop swelling of the legs, oedema and severe pain on movement after a considerable interval following back surgery. The oedema is often responsive to diuretics and there may be no trophic changes in the affected limb. On occasion, Raynaud phenomenon develops in the hands. The exact mechanism of this has not been determined, but it could result from an abnormal firing of the whole of the postganglionic sympathetic nervous system induced by the initial trauma to the back. Lumbar sympathectomy in such patients is also effective in removing the symptoms if carried out soon after the onset.
Sjögren syndrome and mixed connective tissue disease
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Common cutaneous features include Raynaud phenomenon, periungual telangiectasias, acrosclerosis or sclerodactyly, swollen inflamed digits (“sausage fingers”), and symmetrically swollen hands (“puffy hands”). Raynaud phenomenon is the earliest skin manifestation and is present in almost all patients. Patients can develop painful digital ulcers, and even digital infarcts/gangrene in severe cases. Acute lupus-like features such as photosensitivity and malar rash can develop during disease flares [33–38]. Patients with MCTD often develop glandular features of Sjögren syndrome such as dry eyes and dry mouth (sicca complex) [39]. There may be well-defined sclerodermoid (unlike the diffuse binding down of skin in systemic sclerosis) or poikilodermatous (areas of mottled or reticulate dyspigmentation, telangiectasias, atrophy) lesions on the upper trunk and proximal limbs; however, other dermatomyositis-specific changes are usually absent. Oral ulcers and nasal septal perforation have also been described.
Erythemas
Published in Aimilios Lallas, Enzo Errichetti, Dimitrios Ioannides, Dermoscopy in General Dermatology, 2018
Nicola di Meo, Paola Corneli, Iris Zalaudek
Raynaud phenomenon consists of episodic digital ischemia occurring in response to cold or emotional stimuli, which results from a disturbance in vascular homeostasis leading to uncontrolled vasoconstriction.6 It is typically classified into a primary form (also called Raynaud disease), which is idiopathic and occurs as an isolated innocuous disorder, and a secondary form, which occurs in association with underlying diseases (especially systemic sclerosis) or is caused by physical factors or drugs.6
Angiotensin II receptor blockers in dermatology: a narrative review
Published in Journal of Dermatological Treatment, 2022
Raynaud phenomenon (RP) is characterized by decreased blood flow to the digits because of cold-induced vasoconstriction (27). CCBs constitute the first-line treatment option for RP (28), and phosphodiesterase-5 inhibitors and prostanoid derivatives are possible treatment choices. ACEIs and ARBs have been administered for RP treatment due to their vasodilation properties. Several ACEIs have been used in randomized double-blind trials for RP treatment (29,30), including captopril and enalapril. However, the results reported from such trials are inconsistent, and evidence supporting ACEI use for RP is limited (31). Regarding ARBs, one randomized controlled trial compared the efficacy of losartan and nifedipine in primary and secondary RP treatment (32), and treatment with 50 mg losartan daily or 40 mg nifedipine daily was provided over a period of 12 weeks. Notably, in terms of the reduction of RP severity and episode frequency, losartan demonstrated superior efficacy to nifedipine during the study period (32). Furthermore, another study revealed losartan exhibited superior efficacy to picotamide in primary RP (33). Although evidence regarding the efficacy of ARBs for RP treatment is limited, the role of ARBs in the treatment of RP warrants additional studies.
A case of linear scleroderma “en coup de sabre” with strabismus fixus in a child
Published in Strabismus, 2019
Pradhnya Sen, Easha Ramawat, Amit Mohan, Chintan Shah
Zannin et al.3 reported the features of significant ocular involvement in 24 children with JLS, of these ECDS was seen in 66.7% of patients. Ocular features such as adnexal abnormalities were reported in 10 patients, anterior segment ischemia in 7 patients, 3 with central nervous system-related abnormalities, 4 patients presented with single findings such as paralytic squint (1), pseudopapillodema (1), and refractive errors (2). They found a concomitant involvement of other internal organs in 10 patients. Six had neurological involvement, consisting of epilepsy (2), peripheral neuropathy (1), MRI abnormalities (2), and pseudo tumor cerebri (1). Arthritis was present in two patients, aortic insufficiency, abnormal pulmonary function tests, and Raynaud phenomenon were present in three other individual cases, respectively. In our case, there are no other systemic abnormalities, only extraocular movements were restricted with features of strabismus fixus. As far as serological features, ANA was found to be positive in 12 patients (50.0%) but, there was no significant association with a particular ophthalmological finding.
Concurrent onset of acute lupus myocarditis, pulmonary arterial hypertension and digital gangrene in a lupus patient: a possible role of vasculitis to the rare disorders
Published in Modern Rheumatology Case Reports, 2020
Takayasu Ando, Yoshioki Yamasaki, Yukiko Takakuwa, Harunobu Iida, Yusa Asari, Kanako Suzuki, Marina Uchida, Nozomi Kotoku, Yasuhiro Tanabe, Motohiro Chosokabe, Masahide Takahashi, Kengo Suzuki, Yoshihiro J. Akashi, Hidehiro Yamada, Kimito Kawahata
A 41-year-old woman experienced Raynaud phenomenon in X-1 month and visited her local clinic. Since her blood tests were positive for anti-nuclear antibody (ANA) and anti-RNP antibodies, mixed connective tissue disease (MCTD) was suspected. Upon routine screening for infections, she was found to be a carrier of the Hepatitis B virus. Entecavir was started 3 weeks (X-3 weeks) prior to the administration of 10 mg of PSL (X-1 day). The day after PSL administration (X), she visited her local clinic because she developed acute and progressive dyspnoea. Additionally, she developed ischaemic changes in all her fingers, which resulted in digital gangrene. She was transferred to our hospital on X and was admitted for more intensive management.