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Lupus Erythematosus (LE)

  • Spectrum with three major forms: systemic LE (SLE), subacute LE (SCLE) and chronic cutaneous LE (CCLE)
  • Chronic cutaneous LE (discoid LE) (Figure 3.32A–C)
    • Chronic cutaneous form with involvement typically of face/scalp in young women; ↑ incidence in African American patients
    • Presents with indurated erythematous thin papules with adherent scale and follicular plugging on face, scalp and/or ears (concha bowl), ‘carpet tack’ sign (follicular plugs with removal of scale); subsequent atrophy, telangiectasias, dyschromia, scarring (SCC may develop in scars); ¼ with oral involvement; no systemic symptoms or extracutaneous involvement; rarely may see widespread discoid involvement (↑ serologic abnormalities have ↑ likelihood to develop SLE)
    • Variants
      • Tumid lupus: deeper, more nodular lesions with erythema and induration mainly involving face and trunk, no scaling or follicular plugging; dermis with mucin and intense inflammatory infiltrate
      • Lupus panniculitis or profundus: tender subcutaneous nodules with typical DLE surface changes involving buttocks, chest, shoulder, face; heals with deep atrophy; 10–15% meet SLE criteria
      • Hypertrophic or verrucous LE: hyperkeratotic or verrucous papules/plaques over extensor arms, ± face/trunk, may appear similar to warts
      • Chillblain LE: acral dusky purple papules and plaques associated with acrocyanosis, minimal atrophy/scarring
    • Histology: atrophic epidermis with plugged follicles, vacuolar degeneration of basal layer, thickened basement membrane, melanin incontinence, perivascular/periadnexal lymphocytic infiltrate, ↑ mucin between collagen bundles; 90% of lesional biopsies show diffuse irregular band of IgG/C3 at BMZ (lupus band)
    • Labs: ¼ with + ANA (low titer); 5 –10% with DLE may develop SLE over time
    • Treatment: sun avoidance, topical/IL corticosteroid, oral antimalarial, topical calcineurin inhibitor

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    Of note, C2 defiency increases susceptibility to autoimmune conditions, especially lupus (DLE, SCLE, SLE)
    		 
       

  • Subacute cutaneous LE (SCLE) (Figure 3.33A, B)
    • Overlap between DLE and SLE with photosensitivity
    • Presents with papulosquamous or annular/polycyclic erythematous scaly patches/plaques on shoulders, trunk, extensor arms; face typically spared; heal without scarring but telangiectasias common; fatigue and arthralgias common but limited organ involvement
    • Histology: atrophic epidermis, some vacuolar change of BMZ, sparse inflammatory infiltrate
    • Labs: 60–80% with + ANA, anti-Ro (60–90%)
    • Course: persistent with intermittent flares, up to 50% will eventually meet SLE criteria (but milder disease)
    • Treatment: sun protection, antimalarial, oral corticosteroid, dapsone or other immunosuppressive agent (topical corticosteroids rarely sufficient alone)
    • Associations: HLA-B8 (strongest), HLA-DR3, HLA-DRw52, HLA-DQ1

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    Drug-induced SCLE: hydrochlorothiazide, terbinafine, diltiazem, ACEI, NSAIDs, griseofulvin, antihistamines, IFN, PUVA, TNFα
    		 
       

  • Systemic LE (SLE) (Figure 3.33C)
    • Systemic multi-organ involvement typically in young adults; ↑ incidence in African American patients
    • Need 4 out of 11 criteria for diagnosis (see below)
    • Cutaneous lesions include bilateral malar erythema following sun exposure, discoid lesions, oral ulcerations, photosensitivity, patchy to diffuse nonscarring alopecia with lupus hairs (along frontal scalp line), Raynaud’s phenomenon, livedo reticularis, acrocyanosis, cutaneous signs of antiphospholipid syndrome, urticarial vasculitis, red lunulae, multiple dermatofibromas
    • Histology: modest vacuolar change, perivascular and periadnexal lymphocytic infiltrate, ↑ dermal mucin
    • Labs: >95% with + ANA, anti-dsDNA and anti-Sm are both highly specific for SLE, see Table 3.18 for details
    • Treatment: antimalarial, oral/topical corticosteroid, steroid-sparing immunosuppressive agent
    • Associations: HLA-DR2, HLA-DR3

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    Drug-induced SLE: hydralazine, procainamide, chlorpromazine, INH, quinidine, practolol, d-penicillamine, PUVA, minocycline
    		 
       

  • Criteria (need 4 out of 11):
       
     
     
    Malar erythema
    Discoid LE
    + ANA
    Arthritis
     
    Hematologic
    disorder
    Immunologic
    (dsDNA, Sm)
    Neurologic
    disorder
    Serositis
     
    Photosensitivity
    		 
    Nephropathy
    		 
    Oral ulcers
    		  
    		 
       
       
     
    MD SOAP BRAIN: Malar rash, Discoid rash, Serositis, Oral ulcers, Arthritis, Photosensitivity, Blood (heme), Renal, ANA, Immuno abnormality, Neurologic
    		 
       
Figure 3.33 A: SCLE* B: SCLE* C: SLE, malar erythema* * Courtesy of Dr. Iris K. Aronson
Figure 3.33
A: SCLE*
B: SCLE*
C: SLE, malar erythema*
* Courtesy of Dr. Iris K.
Aronson
Figure 3.32 A: Discoid LE* B: Discoid LE* C: Discoid LE* * Courtesy of Dr. Paul Getz
Figure 3.32
A: Discoid LE*
B: Discoid LE*
C: Discoid LE*
* Courtesy of Dr. Paul Getz

 

 

 

 

 

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