Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature

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Term Occurence Count Dictionary
methotrexate 1 rheumatologydiseasesdrugs
myositis 2 rheumatologydiseases
panniculitis 2 rheumatologydiseases
systemic lupus erythematosus 6 rheumatologydiseases
vasculitis 15 rheumatologydiseases
mycophenolate mofetil 1 rheumatologydiseasesdrugs
petechia 1 rheumatologydiseases
chloroquine 4 rheumatologydiseasesdrugs
cutaneous lupus erythematosus 3 rheumatologydiseases
dermatomyositis 2 rheumatologydiseases
hydroxychloroquine 2 rheumatologydiseasesdrugs
triamcinolone 1 rheumatologydiseasesdrugs
arthritis 1 rheumatologydiseases
azathioprine 1 rheumatologydiseasesdrugs
tacrolimus 2 rheumatologydiseasesdrugs

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Select Drug Character Offset Drug Term Instance
azathioprine 23107 well-tolerated [[79]–[81]]. Steroid sparing immunosuppressive agents in JSLE patients (e.g. methotrexate, azathioprine and mycophenolate mofetil), are usually initiated along with hydroxychloroquine [[74]].ConclusionPatients
chloroquine 18313 there is systemic involvement, short courses of systemic corticosteroids with concurrent use of hydroxy chloroquine and/or immunosuppressive therapies are indicated. Table 2 summarizes the commonly used treatments
chloroquine 19648 skin lesions or systemic disease flare upOsteoporosis, cushing syndrome and growth retardationHydroxy chloroquine - Children5 mg/kg ideal body weight per dayCombination with systemic steroidsOcular toxicity, gastrointestinal
chloroquine 22064 should be carefully considered, particularly in JSLE patients.Aminoquinolone antimalarial drugsHydroxy chloroquine demonstrates good efficacy with mucocutaneous lupus lesions and is therefore the first line treatment
chloroquine 23188 (e.g. methotrexate, azathioprine and mycophenolate mofetil), are usually initiated along with hydroxy chloroquine [[74]].ConclusionPatients with JSLE commonly present with mucocutaneous manifestations, and it is therefore
hydroxychloroquine 18306 where there is systemic involvement, short courses of systemic corticosteroids with concurrent use of hydroxychloroquine and/or immunosuppressive therapies are indicated. Table 2 summarizes the commonly used treatments
hydroxychloroquine 23181 patients (e.g. methotrexate, azathioprine and mycophenolate mofetil), are usually initiated along with hydroxychloroquine [[74]].ConclusionPatients with JSLE commonly present with mucocutaneous manifestations, and it is therefore
methotrexate 23093 and are well-tolerated [[79]–[81]]. Steroid sparing immunosuppressive agents in JSLE patients (e.g. methotrexate , azathioprine and mycophenolate mofetil), are usually initiated along with hydroxychloroquine [[74]].ConclusionPatients
mycophenolate mofetil 23124 well-tolerated [[79]–[81]]. Steroid sparing immunosuppressive agents in JSLE patients (e.g. methotrexate, azathioprine and mycophenolate mofetil ), are usually initiated along with hydroxychloroquine [[74]].ConclusionPatients with JSLE commonly present
tacrolimus 19417 pimecrolimusEyelids, face and intertriginous areas (steroid-sparing effects)Burning sensation and infection0.03%, 0.1% tacrolimus 2. SystemicSystemic Steroids0.5-2 mg/kg ideal body weight per day between 2–4 weeks, followed by tapering
tacrolimus 22798 important [[78]]. Routine eye check-ups is recommended.Other medicationsTopical calcineurin inhibitors, such as tacrolimus and primecrolimus, are useful for lesions that are particularly sensitive to topical corticosteroids
triamcinolone 19012 areasHypopigmentation, skin atrophy, increased hair growth and telangiectasia;- Moderate potency0.1% triamcinolone acetonideScalp and body0.1% mometasone furoate- High potency0.05% clobetasone propionateScalp, palms
Select Disease Character Offset Disease Term Instance
arthritis 17303 diseases such as henoch-schönlein purpura, juvenile dermatomyositis and systemic juvenile idiopathic arthritis [[71]].Immunofluorescence findingsImmunofluorescence is not routinely used for the diagnosis in JSLE
cutaneous lupus erythematosus 6148 between the two age groups.Specific mucocutaneous lesionsThese lesions are categorized into 3 forms.Acute cutaneous lupus erythematosus (ACLE) ACLE presents with localized or generalized lesions and they are very sensitive to ultraviolet
cutaneous lupus erythematosus 7317 changes affecting the ear; and (D) Generalized ACLE or maculopapular lupus rash at both knees.Subacute cutaneous lupus erythematosus (SCLE) SCLE is extremely rare in JSLE patients [[32]], but more common in adult SLE patients. There
cutaneous lupus erythematosus 8313 lesions, any suspected drugs should be discontinued but these are seldom used in childhood.Chronic cutaneous lupus erythematosus (CCLE) In adults, it is common to see discoid lupus on the skin without underlying systemic involvement.
dermatomyositis 11554 they correlate with systemic disease. Photosensitivity with malar rash is commonly found in juvenile dermatomyositis ; thus, other lupus features are needed in order to differentiate between the two conditions [[47]].Oral
dermatomyositis 17254 found in juvenile patients with other systemic diseases such as henoch-schönlein purpura, juvenile dermatomyositis and systemic juvenile idiopathic arthritis [[71]].Immunofluorescence findingsImmunofluorescence is not
myositis 11561 correlate with systemic disease. Photosensitivity with malar rash is commonly found in juvenile dermato myositis ; thus, other lupus features are needed in order to differentiate between the two conditions [[47]].Oral
myositis 17261 in juvenile patients with other systemic diseases such as henoch-schönlein purpura, juvenile dermato myositis and systemic juvenile idiopathic arthritis [[71]].Immunofluorescence findingsImmunofluorescence is not
panniculitis 5001 [4], [18], [28], [29]]20-50% [[5], [36]]Generalized DLE10-37% [[31], [34]–[36]]40-49% [[36]]Lupus panniculitis / profundus<1% [[34], [37]]1-3% [[10]]2.LE nonspecific skin lesionsCutaneous vasculitis16-45% [[11],
panniculitis 9466 with a worse prognosis [[28], [33]–[35]].Other forms of CCLE are rare in children, such as lupus panniculitis and lupus profundus [[33], [36]], mucosal LE and chilblain LE [[37]–[40]], and tumid LE [[41]–[43]].
petechia 10331 usually affects small blood vessels (leukocytoclastic vasculitis). The lesions are characterized as petechia e or palpable purpura (Figure 2A), and may occasionally blister. They are commonly found on the face,
systemic lupus erythematosus 91 Title: Pediatric Rheumatology Online JournalMucocutaneous manifestations in juvenile-onset systemic lupus erythematosus : a review of literatureDirekrit ChiewchengcholRuth MurphySteven W EdwardsMichael W BeresfordPublication
systemic lupus erythematosus 364 (pmc-release): 1/2015Publication date (collection): /2015AbstractPatients diagnosed with juvenile-onset systemic lupus erythematosus (JSLE) often have skin and oral lesions as part of their presentation. These mucocutaneous lesions,
systemic lupus erythematosus 1855 controlled and their reappearance is often the first indicator of a disease flare.IntroductionJuvenile-onset systemic lupus erythematosus (JSLE) is one of the most common systemic autoimmune connective tissue disorders in children. The disease
systemic lupus erythematosus 4573 manifestations appear more frequently [[29]].Table 1Comparative frequency of mucocutaneous lesions in juvenile systemic lupus erythematosus (JSLE) and adult systemic lupus erythematosus (adult SLE)Mucocutaneous lesionsJSLEAdult SLE1.LE specific
systemic lupus erythematosus 4619 1Comparative frequency of mucocutaneous lesions in juvenile systemic lupus erythematosus (JSLE) and adult systemic lupus erythematosus (adult SLE)Mucocutaneous lesionsJSLEAdult SLE1.LE specific skin lesionsMalar rash44-85% [[2], [5], [6]]40-52% [[5],
systemic lupus erythematosus 18582 JSLE patients.Table 2Summary of commonly used treatment in mucocutaneous lupus lesions in juvenile systemic lupus erythematosus (JSLE) [[74]–[81]]TreatmentsDoseIndicationsCommon/serious side effects1. TopicalSunscreen2 mg/cm2,
vasculitis 1477 disease activity. The most commonly associated skin lesions without classical lupus changes are cutaneous vasculitis , oral ulcers and diffuse non-scarring alopecia. These signs frequently relate to disease activity. An
vasculitis 3924 (60-90%) [[22], [23]]. The most common mucocutaneous lesions in JSLE are: malar rash, photosensitivity, cutaneous vasculitis and oral or nasal ulcers. Others include generalized lupus rash, non-scarring alopecia, livedo reticularis
vasculitis 5090 [34]–[36]]40-49% [[36]]Lupus panniculitis/ profundus<1% [[34], [37]]1-3% [[10]]2.LE nonspecific skin lesionsCutaneous vasculitis 16-45% [[11], [21], [47]]11-70% [[5], [7], [12], [13]]Photosensitivity35-50% [[2], [4], [5]]63% [[7]]Oral
vasculitis 10036 lesions commonly found in children and adults are similar and both affect vasculature (e.g. cutaneous vasculitis , livedo reticularis and Raynaud’s phenomenon). The other common lesions are photosensitivity, oral
vasculitis 10199 other common lesions are photosensitivity, oral ulcers and diffuse non-scarring alopecia.Cutaneous vasculitis Cutaneous vasculitis usually affects small blood vessels (leukocytoclastic vasculitis). The lesions
vasculitis 10220 are photosensitivity, oral ulcers and diffuse non-scarring alopecia.Cutaneous vasculitis Cutaneous vasculitis usually affects small blood vessels (leukocytoclastic vasculitis). The lesions are characterized as
vasculitis 10285 alopecia.Cutaneous vasculitis Cutaneous vasculitis usually affects small blood vessels (leukocytoclastic vasculitis ). The lesions are characterized as petechiae or palpable purpura (Figure 2A), and may occasionally
vasculitis 10741 systemic disease activity [[46]]. Other clinical presentations include: punctate lesions and urticarial vasculitis (Figure 2C).Figure 2LE nonspecific skin lesions: (A) Vasculitic purpura at left palm; (B) Cutaneous
vasculitis 10854 (Figure 2C).Figure 2LE nonspecific skin lesions: (A) Vasculitic purpura at left palm; (B) Cutaneous vasculitis at right cheek, eyelid and nose; (C) Cutaneous urticarial vasculitis at right palm; (D) Oral discoid
vasculitis 10923 purpura at left palm; (B) Cutaneous vasculitis at right cheek, eyelid and nose; (C) Cutaneous urticarial vasculitis at right palm; (D) Oral discoid lesion on the lateral border of the tongue; and (E) Oral ulceration
vasculitis 14986 lesions Other lesions include: calcinosis cutis, acanthosis nigricans, hypocomplementaemic urticarial vasculitis (very rare in JSLE patients) [[62]–[64]], whilst rheumatoid nodules, anetoderma and erythromelalgia
vasculitis 16142 and skin appendages with perivascular lymphocytic and neutrophil infiltrates called leukocytoclastic vasculitis [[68]–[70]]. Abundant extracellular deposition of mucin is markedly observed in the dermis between
vasculitis 16937 depending on the type of lesions and are not pathognomonic of the condition. For example, cutaneous vasculitis in JSLE shows small vessel leukocytoclastic vasculitis including endothelial cell damage, fibrin deposition,
vasculitis 16992 pathognomonic of the condition. For example, cutaneous vasculitis in JSLE shows small vessel leukocytoclastic vasculitis including endothelial cell damage, fibrin deposition, inflammatory cell infiltrate (predominantly neutrophils)
vasculitis 21696 of induce remission of severe mucocutaneous lesions (e.g. multiple oral ulcers, or severe cutaneous vasculitis with ulceration and necrosis). However, these should be avoided for prolonged periods due to serious

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