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cutaneous lupus erythematosus

Etiology: - subacute cutaneous lupus - drug-induced lupus erythematosus Pathology: 1) blood vessel-based inflammatory reactions (vasculitis) 2) lymphocyte-mediated inflammation of the dermal-epidermal junction (interface dermatitis) Clinical manifestations: 1) lupus-specific skin manifestations [2] a) acute cutaneous lupus - photosensitivity rash - malar rash (butterfly rash), forehead, chin & other sun-exposed areas - poikiloderma - spares nasolabial folds - symptoms for up to >= one month [2] - all patients have systemic lupus erythematosus [2] b) subacute cutaneous lupus - may be considered a separate entity from SLE - disease is less severe than systemic lupus erythematosus - 10% of patients will develop internal disease - renal, central nervous system, serositis - upper torso in a photodistribution pattern - association with HLA DR3 & Ro/SSA & La/SSB antibody - lesions are annular scaly patches with crusted margins & central hypopigmentation or papular & non scarring - psoriaform patches [2] - telangiectasias common - no malar rash [2] distinguishes from acute cutaneous lupus c) chronic cutaneous lupus (see discoid lupus) - face, neck, scalp & external ears - raised, well marginated borders - central scarring with atrophy - psoriaform scale - follicular plugging - irreversible alopecia in the scalp d) chilblain lupus erythematosus 2) non-specific cutaneous manifestations [2] a) urticaria b) oral ulcerations - on tongue & hard palate - generally painless c) alopecia d) lupus panniculitis (lupus profundus) - presents with painful nodules e) vasculitis f) livedo reticularis * images [5] Laboratory: - centromere protein B Ab in serum Complications: - malar rash associated with high risk for lupus nephritis & other manifestations of systemic lupus erythematosus - coexisting autoimmune conditions [18%) [7] - autoimmune thyroiditis with highest frequency (5%) - Sjogren syndrome (3%) - rheumatoid arthritis (2%) - alopecia areata (2%) [7] - chronic cutaneous lupus (see discoid lupus) - 5-15% risk of systemic lupus erythematosus Management: 1) sunscreen, protective clothing 2) topical corticosteroids 3) methotrexate most effective in achieving complete clinical response [8] 4) antimalarial agents - hydroxychloroquine most effective in reducing number of clinical flares [8] - chloroquine appears noninferior to methotrexate 5) other options - mycophenolate - cyclosporine - dapsone

Specific

chilblain lupus erythematosus discoid lupus (chronic cutaneous lupus erythematosus) lupus miliaris disseminatus faciei; facial idiopathic granulomas with regressive evolution (LMDF, FIGURE) subacute cutaneous lupus erythematosus

General

systemic lupus erythematosus

References

  1. Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 350-57
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018, 2022.
  3. Walling HW, Sontheimer RD. Cutaneous lupus erythematosus: issues in diagnosis and treatment. Am J Clin Dermatol. 2009;10(6):365-81 PMID: 19824738
  4. Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol. 2013 Jun;27(3):391-404 PMID: 24238695
  5. DermNet NZ. Cutaneous lupus erythematosus (images) http://www.dermnetnz.org/immune/cutaneous-lupus.html
  6. Ocampo V, Haider S Images in Clinical Medicine: Cutaneous Lupus - "The Pimple That Never Went Away" N Engl J Med 2016; 375:1566. October 20, 2016 PMID: 27797305 Free full text http://www.nejm.org/doi/full/10.1056/NEJMicm1407057
  7. Kunzler E, Hynan LS, Chong BF. Autoimmune Diseases in Patients With Cutaneous Lupus Erythematosus. JAMA Dermatol. Published online May 2, 2018. PMID: 29801110 https://jamanetwork.com/journals/jamadermatology/fullarticle/2679045
  8. Sprow G, Afarideh M, Werth VP JAMA Dermatology Clinical Evidence Synopsis. Interventions for Cutaneous Disease in Systemic Lupus Erythematosus. Summary of a Cochrane Review. JAMA Dermatol. Published online January 12, 2022 PMID: 35019939 https://jamanetwork.com/journals/jamadermatology/fullarticle/2787883