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cutaneous lupus erythematosus

Etiology: - subacute cutaneous lupus - drug-induced lupus erythematosus - hydrochlorothiazide, calcium channel blockers, ACE inhibitors, terbinafine 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 - 10-50% of patients will develop systemic disease - renal, central nervous system, serositis (arthralgias) - only form of cutaneous lupus to manifest systemic symptoms [2] - 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 - anti-Ro/SSA Ab in serum (positive in majority of patients with subacute cutaneous lupus 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 tumid 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. - Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025
  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
  9. Vale ECSD, Garcia LC. Cutaneous lupus erythematosus: a review of etiopathogenic, clinical, diagnostic and therapeutic aspects. An Bras Dermatol. 2023;98:355-372. PMID: 36868923