cutaneous lupus erythematosus
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Etiology
Pathology
- blood vessel-based inflammatory reactions (vasculitis)
- lymphocyte-mediated inflammation of the dermal-epidermal junction (interface dermatitis)
Clinical manifestations
- lupus-specific skin manifestations[2]
- 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]
- photosensitivity rash
- 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
- 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
- chilblain lupus erythematosus
- acute cutaneous lupus
- non-specific cutaneous manifestations[2]
- urticaria
- oral ulcerations
- on tongue & hard palate
- generally painless
- alopecia
- lupus panniculitis (lupus profundus)
- presents with painful nodules
- vasculitis
- livedo reticularis
* images[5]
Laboratory
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
- sunscreen, protective clothing
- topical corticosteroids
- methotrexate most effective in achieving complete clinical response[8]
- antimalarial agents
- hydroxychloroquine most effective in reducing number of clinical flares[8]
- chloroquine appears noninferior to methotrexate
- other options
More general terms
More specific terms
- 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
References
- ↑ Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 350-57
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018, 2022.
- ↑ Walling HW, Sontheimer RD. Cutaneous lupus erythematosus: issues in diagnosis and treatment. Am J Clin Dermatol. 2009;10(6):365-81 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19824738
- ↑ Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol. 2013 Jun;27(3):391-404 PMID: https://www.ncbi.nlm.nih.gov/pubmed/24238695
- ↑ 5.0 5.1 DermNet NZ. Cutaneous lupus erythematosus (images) http://www.dermnetnz.org/immune/cutaneous-lupus.html
- ↑ 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 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/27797305 Free full text <Internet> http://www.nejm.org/doi/full/10.1056/NEJMicm1407057
- ↑ 7.0 7.1 7.2 Kunzler E, Hynan LS, Chong BF. Autoimmune Diseases in Patients With Cutaneous Lupus Erythematosus. JAMA Dermatol. Published online May 2, 2018. PMID: https://www.ncbi.nlm.nih.gov/pubmed/29801110 https://jamanetwork.com/journals/jamadermatology/fullarticle/2679045
- ↑ 8.0 8.1 8.2 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: https://www.ncbi.nlm.nih.gov/pubmed/35019939 https://jamanetwork.com/journals/jamadermatology/fullarticle/2787883