Cutaneous SLE & Small Vessel Vasculitis

Question 1

autoimmunity - defined

Answer 1

*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

Question 2

autoimmune connective tissue diseases

Answer 2

*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

Question 3

many different types of lupus that involve the skin +/- other systemic involvement

Answer 3

*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

Question 4

cutaneous lupus as predictor of internal involvement

Answer 4

*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

Question 5

most common skin findings in systemic lupus

Answer 5

1. malar rash
2. oral ulcers
3. photosensitivity

Question 6

malar rash in SLE

Answer 6

*most commonly associated with acute cutaneous lupus / systemic lupus
*spares the nasolabial fold

Question 7

acute cutaneous lupus (ACLE)

Answer 7

*classically presents with malar rash (spares nasolabial fold)
*usually ANA positive
*most highly associated with internal / systemic lupus

Question 8

subacute cutaneous lupus erythematosus (SCLE)

Answer 8

*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

Question 9

chronic cutaneous lupus erythematosus (CCLE)

Answer 9

*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

Question 10

lupus profundus

Answer 10

*panniculitis (skin lupus goes all the way down into the fat lobules)

Question 11

neonatal lupus

Answer 11

*usually from maternal transfer of an antibody (esp. anti-SSA, anti-SSB)
*concern for congenital heart block

Question 12

bullous lupus

Answer 12

Question 13

chillblain lupus

Answer 13

*red papules on distal fingertips
*usually provoked by the cold

Question 14

treatment of cutaneous lupus

Answer 14

*PHOTOPROTECTION
*topical anti-inflammatory medications (topical corticosteroids, topical calcineurin inhibitors)
*hydroxychloroquine (systemic anti-malarial)
*other systemic therapy

Question 15

sarcoidosis (overview)

Answer 15

*granuloma formation in multiple organ systems; granulomas consist of collections of mixed inflammatory cells
*organ involvement: LUNGS (hilar lymphadenopathy), SKIN, eyes, brain, heart, liver

Question 16

forms of cutaneous sarcoidosis

Answer 16

*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

Question 17

lupus pernio

Answer 17

*a form of cutaneous SARCOIDOSIS
*refer to ENT & pulmonary if seen (high risk of lung involvement)

Question 18

Koebner phenomenon

Answer 18

*seen in SARCOIDOSIS, psoriasis, etc
*aka isomorphic response
*new lesion of existing disease due to trauma (ex. tattoo, bumping elbow or knee)

Question 19

erythema nodosum in sarcoidosis

Answer 19

*classically associated with Lofgren syndrome (erythema nodosum + arthralgias + hilar lymphadenopathy)
*a type of panniculitis (inflammation of fat)

Question 20

treatment of sarcoidosis

Answer 20

*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

Question 21

dermatomyositis (overview)

Answer 21

*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

Question 22

heliotrope sign

Answer 22

*skin finding seen in dermatomyositis
*periocular rash, can present with a lot of edema, hugs the nose medially

Question 23

shawl sign

Answer 23

*skin finding seen in dermatomyositis
*rash in the shape of where someone would wear a shawl (posterior neck)

Question 24

characteristic skin findings associated with dermatomyositis

Answer 24

*heliotrope sign
*shawl sign
*V sign
*Gottron’s sign (purplish papules over knuckles)

Question 25

workup of dermatomyositis

Answer 25

*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

Question 26

treatment of dermatomyositis

Answer 26

*prednisone +/- other systemic immune meds like methotrexate, mycophenolate mofetil
*topical steroids
*strict sun avoidance
*early and aggressive PT/OT to battle weakness and falls

Question 27

scleroderma / systemic sclerosis (overview)

Answer 27

*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

Question 28

scleroderma / systemic sclerosis - characteristic skin findings

Answer 28

* 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

Question 29

limited scleroderma (CREST)

Answer 29

*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

Question 30

treatment of scleroderma

Answer 30

*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

Question 31

cryoglobulinemia (overview)

Answer 31

*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

Question 32

retiform purpura

Answer 32

*associated with cryoglobulinemia

Question 33

vasculitis vs. vasculopathy

Answer 33

*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)

Question 34

levamisole-induced vasculitis/vasculopathy

Answer 34

*levamisole is an adulterant found in cocaine
*if you see vasculitis/vasculopathy of ear especially, ask about cocaine use

Question 35

cutaneous vasculitis pathogenesis

Answer 35

*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

Question 36

cutaneous leukocytoclastic vasculitis - characteristic skin findings

Answer 36

*palpable purpura
*vasculitis of ankles/feet
*little red dots on lower extremities

Question 37

IgA vasculitis (overview)

Answer 37

*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