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Guideline for the diagnosis, treatment and long-term management of cutaneous lupus erythematosus

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eMediNexus    04 March 2022

Cutaneous lupus erythematosus (CLE) presents as an inflammatory, autoimmune disease containing a broad spectrum of subtypes including acute, subacute, chronic and intermittent CLE. 

Further, chronic CLE can be categorized into various subclasses of lupus erythematosus (LE) like discoid LE, verrucous LE, LE profundus, chilblain LE and Blaschko linear LE. 

An evidence- and consensus-based guideline provides all dermatologists and rheumatologists with a practical guideline for the diagnosis, treatment and long-term management of CLE. This guideline was developed based on the checklist specified by the international Reporting Items for Practice Guidelines in Healthcare (RIGHT) Working Group and was registered at the International Practice Guideline Registry Platform. 

25 dermatologists, 7 rheumatologists, one research scientist on lupus and 2 methodologists, from 16 countries/regions in Asia, America and Europe, with the joint efforts of the Asian Dermatological Association (ADA), the Asian Academy of Dermatology and Venereology (AADV) and the Lupus Erythematosus Research Center of Chinese Society of Dermatology (CSD), came together to develop this guideline. 

All recommendations saw at least 80% agreement of the 32 voting physicians. The consensus was established on- diagnosing CLE chiefly based on the evaluation of clinical and histopathological manifestations, with the exclusion of SLE by assessment of systemic involvement. Localized CLE lesions should be treated with topical corticosteroids and topical calcineurin inhibitors as the first-line treatment. The addition of systemic treatment including antimalarial and (or) short-term corticosteroids can be considered for widespread or severe CLE lesions and (or) cases resistant to topical treatment. Antimalarials should be used as the first-line systemic treatment for all types of CLE, (even in pregnant patients and pediatric patients). Second-line choices include thalidomide, retinoids, dapsone and MTX, while MMF is third-line treatment. Ultimately, pulsed-dye laser or surgery can be counted as a fourth-line treatment for localized, refractory lesions of CCLE in cosmetically unacceptable areas, while belimumab may be utilized as a fourth-line treatment for widespread CLE lesions in patients with active SLE, or recurrence of ACLE during corticosteroids tapering. 

For the management of the disease, patient education and a long-term follow-up are essential. Disease activity, damage of skin and other organs, quality of life, comorbidities and possible adverse events should be assessed in every follow-up visit, when appropriate.

SOURCE- J Autoimmun. 2021;123:102707. doi: 10.1016/j.jaut.2021.102707. Epub 2021 Aug 5. PMID: 34364171.

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