Cutaneous SLE & Small Vessel Vasculitis Flashcards
autoimmunity - defined
*complex interplay of genetic predisposition coupled with an unknown environmental trigger causes a person’s immune system to develop antibodies against a normal part of their body/immune system
*the type of antibody formed dictates to a large degree what disease manifestations a patient will experience
*lab tests are commonly used to help pinpoint the exact type of autoantibodies and aid in diagnosis
autoimmune connective tissue diseases
*lupus, sarcoidosis, dermatomyositis, and scleroderma are all considered autoimmune connective tissue diseases
*these auto-antibodies target components of tissue found in skin plus other organ systems such as joints, lungs, brain, kidneys, heart, etc
*most have a B-cell mediated pathogenesis
many different types of lupus that involve the skin +/- other systemic involvement
*chronic cutaneous lupus
*acute cutaneous lupus
*subacute cutaneous lupus
*systemic lupus erythematosus
*discoid lupus
*lupus profundus/panniculitis
*drug induced lupus
*neonatal lupus
*chillblain lupus
*bullous lupus
cutaneous lupus as predictor of internal involvement
*all types of cutaneous lupus have an associated % of pts who will either concurrently have internal lupus or who will progress to interval involvement
*in order from LEAST to MOST likely to have internal lupus: discoid < chronic cutaneous < chillblain < subacute cutaneous < lupus profundus < bullous < neonatal < drug induced < acute cutaneous
most common skin findings in systemic lupus
- malar rash
- oral ulcers
- photosensitivity
malar rash in SLE
*most commonly associated with acute cutaneous lupus / systemic lupus
*spares the nasolabial fold
acute cutaneous lupus (ACLE)
*classically presents with malar rash (spares nasolabial fold)
*usually ANA positive
*most highly associated with internal / systemic lupus
subacute cutaneous lupus erythematosus (SCLE)
*photodistributed annular plaques with raised borders and central clearing
*almost always thought to be drug-related
*POLYCYCLIC psoriasiform rash
*about 50% meet criteria for SLE
chronic cutaneous lupus erythematosus (CCLE)
*often presents as discoid lupus erythematosus; only progresses to systemic lupus ~5% of time
*often on the head, neck, scalp, ears but can be anywhere
*initially erythematous but can lead to scarring plaques with atrophy and hypopigmentation centrally
lupus profundus
*panniculitis (skin lupus goes all the way down into the fat lobules)
neonatal lupus
*usually from maternal transfer of an antibody (esp. anti-SSA, anti-SSB)
*concern for congenital heart block
bullous lupus
chillblain lupus
*red papules on distal fingertips
*usually provoked by the cold
treatment of cutaneous lupus
*PHOTOPROTECTION
*topical anti-inflammatory medications (topical corticosteroids, topical calcineurin inhibitors)
*hydroxychloroquine (systemic anti-malarial)
*other systemic therapy
sarcoidosis (overview)
*granuloma formation in multiple organ systems; granulomas consist of collections of mixed inflammatory cells
*organ involvement: LUNGS (hilar lymphadenopathy), SKIN, eyes, brain, heart, liver
forms of cutaneous sarcoidosis
*papular forms on face and extremities
*lupus pernio → plaques confined to nose & face; more likely to be associated with lung disease
*subcutaneous nodules
*Koebner phenomenon: lesions within pre-existing scars or tattoos
*Lofgren Syndrome: erythema nodosum, arthralgias, and pulmonary lymphadenopathy
lupus pernio
*a form of cutaneous SARCOIDOSIS
*refer to ENT & pulmonary if seen (high risk of lung involvement)
Koebner phenomenon
*seen in SARCOIDOSIS, psoriasis, etc
*aka isomorphic response
*new lesion of existing disease due to trauma (ex. tattoo, bumping elbow or knee)
erythema nodosum in sarcoidosis
*classically associated with Lofgren syndrome (erythema nodosum + arthralgias + hilar lymphadenopathy)
*a type of panniculitis (inflammation of fat)
treatment of sarcoidosis
*depends on extent of involvement
*cutaneous disease: potent topical steroids, intralesional steroid injections
*systemic disease + widespread cutaneous disease:
-oral steroids
-hydroxychloroquine
-methotrexate
-thalidomide
-TNF alpha inhibitors
-JAK inhibitors
dermatomyositis (overview)
*inflammatory, photosensitive disease affecting both the skin and muscle
*presents with characteristic skin findings in addition to proximal muscle weakness
*dermatomyositis is associated with increased risk of multiple malignancies, especially OVARIAN CANCER
*can develop pulmonary fibrosis (interstitial lung disease, ILD) as well
heliotrope sign
*skin finding seen in dermatomyositis
*periocular rash, can present with a lot of edema, hugs the nose medially
shawl sign
*skin finding seen in dermatomyositis
*rash in the shape of where someone would wear a shawl (posterior neck)
characteristic skin findings associated with dermatomyositis
*heliotrope sign
*shawl sign
*V sign
*Gottron’s sign (purplish papules over knuckles)
workup of dermatomyositis
*need 2 confirmatory muscle inflammation tests: CK, triceps muscle biopsy, triceps MRI, EMG of triceps muscle
*screen for malignancy: colon, lung, OVARIAN, prostate, breast
*lung involvement: PFTs, looking for a restrictive pattern
*myositis panel
-common antibody: Jo-1 antisynthetase with mechanics hands and ILD
treatment of dermatomyositis
*prednisone +/- other systemic immune meds like methotrexate, mycophenolate mofetil
*topical steroids
*strict sun avoidance
*early and aggressive PT/OT to battle weakness and falls
scleroderma / systemic sclerosis (overview)
*autoimmune disease affecting mostly women in 30s-40s
*antibodies to Scl-70 (nuclear antibody to topoisomerase I)
*thought to be endothelial cell injury which leads to overproliferation of fibroblasts, causing extensive fibrosis in multiple different organs:
-skin, retinopathy, joint pain and contractures, esophageal reflux, pulmonary fibrosis, cardiac arrhythmia and heart failure, renal disease
scleroderma / systemic sclerosis - characteristic skin findings
* decreased oral aperture (taut shiny skin, particularly perioral skin, with restricted mouth opening)
* sclerodactyly: finger skin thickened, shiny with resorption of distal digits
* Raynaud’s phenomenon (blanching of skin as well as dusky violaceous discoloration when exposed to cold; painful rewarming)
* telangiectasias on face, palms, mucosa
* salt and pepper skin
limited scleroderma (CREST)
*common subtype of scleroderma which is considered to be a more limited systemic involvement
*associated with anti-centromere antibodies
*CREST:
-Calcinosis cutis
-Raynaud’s phenomenon
-Esophageal dysmotility
-Sclerodactyly
-Telangiectasias
treatment of scleroderma
*Raynaud’s: topic nitroglycerin, oral CCBs, prostaglandins
*reflux: PPIs, esophageal dilation if needed
*kidney disease (scleroderma renal crisis): ACE inhibitors
*cardiac: anti-arrhythmics
*pulmonary disease: serial PFTs
*AVOID PREDNISONE IN SCLERODERMA as can precipitate renal crisis
cryoglobulinemia (overview)
*precipitation of cryoglobulins in cooler temperatures causes clots to form in smaller blood vessels, resulting in “retiform purpura”
*3 subtypes:
-type 1 associated with hematologic malignancy
-types 2 and 3 associated with connective tissue disease
retiform purpura
*associated with cryoglobulinemia
vasculitis vs. vasculopathy
*vasculopathy: occlusion of vessels starves tissue (occlusion can be due to bacteria, clots, immunoglobulins)
*vasculitis: immune complex deposition in vessel walls triggers neutrophil recruitment and destruction of vessels (triggers include drugs, viral infections, bacteria infections, connective tissue diseases)
levamisole-induced vasculitis/vasculopathy
*levamisole is an adulterant found in cocaine
*if you see vasculitis/vasculopathy of ear especially, ask about cocaine use
cutaneous vasculitis pathogenesis
*circulating immune complexes can lodge in the walls of endothelial cells, activating complement
*part of the complement cascade (C5a) attracts neutrophils which chew up the immune complexes, but also release damaging enzymes which can injure endothelial cells and damage blood vessels
cutaneous leukocytoclastic vasculitis - characteristic skin findings
*palpable purpura
*vasculitis of ankles/feet
*little red dots on lower extremities
IgA vasculitis (overview)
*sometimes called Henoch Schoenlein Purpura (HSP)
*variant of cutaneous leukocytoclastic vasculitis, affecting kids > adults
*constellation of findings: cutaneous LCV, arthritis, abdominal pain, kidney damage (hematuria, proteinuria, elevated Cr)
*check for IgA deposits around vessels on direct immunofluorescence of skin biopsy
