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Granulomatous Diseases
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Albert Alhatem, Robert A. Schwartz, Muriel W. Lambert, W. Clark Lambert
Cutaneous sarcoid granulomata and chronic facial lesions are associated with a poor prognosis due to the pulmonary fibrosis and uveitis. Lupus pernio is often associated with advanced lung fibrosis, bone cysts, and eye disease.
Lupus Pernio
Published in K. Gupta, P. Carmichael, A. Zumla, 100 Short Cases for the MRCP, 2020
K. Gupta, P. Carmichael, A. Zumla
This patient has typical swollen violaceous 4-5 cm diameter plaques on her nose and left cheek. They are indurated in nature with scattered overlying varicosities. This is the typical lesion of lupus pernio and the underlying systemic condition is most likely to be sarcoidosis.
Granulomatous Conditions of the Nose
Published in John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie, Basic Sciences Endocrine Surgery Rhinology, 2018
Joanne Rimmer, Valerie J. Lund
Sarcoidosis has a variable clinical course that often correlates with the mode of presentation; an acute onset with arthritis and erythema nodosum is usually self-limiting, whilst a more insidious onset with lupus pernio and sinonasal involvement tends to be more chronic.18 Up to two-thirds of patients will spontaneously remit, generally those with stage I and II pulmonary disease, while 10–30% follow a chronic course with relapses despite systemic therapy.19 Some patients require no treatment at all, but international guidelines suggest systemic or intra-lesional steroid treatment for life-threatening disease or if critical organs are involved.20 Despite a lack of controlled studies, steroids remain the mainstay of treatment for severe disease, with the addition of agents such as methotrexate to reduce the steroid dose if required. Hydroxychloroquine has been used for cutaneous and sinonasal sarcoidosis, although the response rate is less than 50% and there is a risk of ocular toxicity.21 Lupus pernio may respond dramatically to medical treatment.22 There has been recent interest in TNF-alpha inhibitors such as infliximab and etanercept.23
Emerging and potential treatment options for sarcoidosis
Published in Expert Opinion on Orphan Drugs, 2018
Debabrata Bandyopadhyay, Marc A. Judson
Sleep disorders – Sarcoidosis patients have rates of sleep disorders that exceed the rates in the general population and may reach more than 50% [155,161]. Specific reasons for the high rate of sleep disorders in sarcoidosis include (a) weight gain from corticosteroid use that increases the risk for developing obstructive sleep apnea (OSA); (b) the presence of sarcoidosis of the upper respiratory tract (SURT) that may increase upper airway resistance leading to OSA or other sleep disorders; (c) the presence of interstitial lung disease that is associated with a greater risk of OSA (incidentally, in the specific case of sarcoidosis, parenchymal involvement has been associated with worse severity of sleep disordered breathing that in those without parenchymal involvement); and (d) the presence of lupus pernio, disfiguring facial sarcoidosis, although we suspect that this association relates to the fact that lupus pernio is closely associated with SURT [162–165].
A case of chronic sarcoidosis presenting with lupus pernio
Published in Postgraduate Medicine, 2020
Lupus pernio is one of the specific skin lesions in sarcoidosis. It was first described by Ernest Besnier in 1889 as a chronic lesion typically found in the nose, cheeks, ears, and fingers [9]. It is a feature of a multisystem disease and is often associated with severe organ involvement mostly including the lungs and upper respiratory tract [1314–15]. Moreover, LP may precede the chronic course of sarcoidosis. Therefore, in the management of sarcoidosis, we recommend patients diagnosed with LP in dermatology clinics to be referred to pulmonary diseases clinics for investigation of lung involvement.
Infliximab for relapsing neurosarcoidosis recurring after kidney transplantation: a case report
Published in Acta Clinica Belgica, 2021
Nicolas Hanset, Mawufemo Yawovi Tsevi, Thierry Duprez, Adrian Ivanoiu, Arnaud Devresse, Nathalie Demoulin, Nada Kanaan
Physical examination was unremarkable except for nasal and malar reddish rash. Laboratory tests showed normal C-reactive protein, creatinine, albumin and calcemia levels. Serum PTH level, 1,25(OH)2vitaminD3, TSH, T4, serum angiotensin-converting enzyme (ACE) and lysozyme levels were in the normal range. Urinalysis showed an increased calcium/creatinine ratio (0.82 mM/mM (N < 0.4)), with normal proteinuria and no hematuria. Tacrolimus trough level was in the range (5.2 ng/mL). Serum PCR for CMV, EBV and HIV were negative. Chest radiography was normal. Gadolinium-enhanced brain magnetic resonance imaging (MRI) demonstrated multiple, ubiquitous microlesions revealed by brain-blood-barrier breakdown together with en plaque meningeal thickening of the tentorium (Figure 1(a–c)). Cerebrospinal fluid (CSF) analysis displayed increased white blood cells count (25/µl; 98% lymphocytes), with elevated proteins at 58 mg/dL (normal range: 15–45), normal glucose level, and negative microbiological tests, including bacterial culture and polymerase chain reaction (PCR) for Mycobacterium tuberculosis. Biological and radiological work-up allowed the exclusion of an infectious or malignant disease occurring in an immunosuppressed-transplanted patient, and led to the diagnosis of sterile lymphocytic meningitis associated with parenchymal involvement secondary to neurosarcoidosis (NS). Cutaneous lesions were retrospectively suggestive of lupus pernio. Oral MP was started (32 mg daily for four weeks, tapered by 4 mg every 2 weeks), with initially good clinical and radiological response (Figure 1(d–f)). He experienced a first radiological relapse under 12 mg of MP/day. Despite increasing MMF doses to 2g/day and resuming MP at 32 mg/day for 4 weeks, with tapering by 4 mg/3 weeks, new brain lesions appeared under MP 8 mg/day, leading to resume doses to 32 mg/day for one month. Slower tapering of MP (4 mg/month) was applied until reaching 4 mg/day- his maintenance dose for kidney transplantation. Cerebral MRI performed 3 months after reaching maintenance CS was normal. Unfortunately, 2 months later, he complained of extreme nervousness and instability when walking. Relapse was confirmed on cerebral MRI. Infliximab (Remicade R), a monoclonal antibody against tumor necrosis factor-α, was obtained from the pharmaceutical company as part of a medical need program for off-label indications. The patient underwent chest X-ray and intradermo to rule latent tuberculosis, and was vaccinated against influenza virus and pneumococcus. He received an intravenous infusion of Remicade (5 mg/kg) at day 0, 15, 45 then once every 2 months. Treatment was well tolerated and to date, 7 months later, the patient is clinically very well and cerebral MRI confirmed the favourable evolution with complete regression of cerebral inflammatory images. The treatment is intended to be pursued for 2 years.