Connective Tissue Disease Flashcards
What are the three major forms of cutaneous lupus?
chronic cutaneous lupus erythematosis - discoid lupus erythematosus (DLE)
subacute cutaneous lupus erythematosis (SCLE)
acute cutaneous lupus erythematosus (ACLE)
discoid lupus erythematosus
more common in females and AA
confined to skin
head and upper body scaly, pink brown plaques
scalp lesions result in alopecia
subacute cutaneous lupus erythematosus
photosensitive
lateral face, upper trunk, extensor upper extremities
scaling, annular pink papules and plaques
no scarring
srong association with anti-Ro (SSA) antibodies
acute lupus erythematosus
classic malar rash
spares nasolabial folds
transient eruption
triggered by sun exposure
often associated with antibodies (anti-double stranded DNA)
must evaluate for systemic disease
neonatal lupus erythematosus (NLE)
infants whos mothers have anti-Ro antibodies
similar to SCLE - annular, pink scaly plaques
sun exposure NOT needed
dyspigmentation persists
internal manifestations of NLE
congenital heart block - almost always present at birth and 2/3 require pacemakers
mortality roughly 20%
hepatobiliary disease
thrombocytopenia
dermatomyositis
women more common
proximal extensor inflammatory myopathy
heliotrop eruption - violaceous and periorbital edema
Gottron’s papules
naile changes
poidiloderma
shawl sign
Gottron’s Papules
extensor joints - MCP, IP, elbows
pink to volaceous
papules and plaques
often scaling
poikiloderma
hyperpigmentation
hypopigmentation
telangiectasia
epidermal atrophy
** have to have all of these
shawl sign
anti mi2 antibodies
photodistributed poikiloderma - V of neck and upper back
What is the internal involvement of dermatomyositis?
myopathy
pulmonary disease
cardiac disease
calcinosis
malignancy
What are skin findings of RA?
deforming arthritis
rheumatoid nodules - specific finding
non-specific cutaneous lesions - vasculitis, bywater’s lesions, neutrophilic dermatoses
relapsing polychonrditis
inflammation of cartilaginous structures
anti-body mediated and treate with immunosuppression
What are the clinical features of RP?
inflammation of ear, nose, respiratory tract, joints
death is possible
Sjogren’s Syndrome
affects secretory glands
auto-antibodies (anti-fodrin, anti-SSA, anti-SSB)
variable cutaneous features
most common features are xerostomia, xerophthalmia, arthritis
serous complications - extranodal lymphomas, peripheral neuropathy, hearing loss
cutaneous features of Sjogren’s Syndrome
xerosis (dry skin)
petechiae
purpura and vasculitis
annular erythema
urticarial vasculitis
What are the two major clinical subtypes of systemic sclerosis?
limited cutaneous and diffuse cutaneous
vascular dysfunction, immune activation, tissue fibrosis
diffuse scleroderma
anti Scl70 antibodies
Raynaud’s phenomenon
starts with hands
spreads to forearms, arms, trunk, face, lower extremities
limited cutaneous scleroderma
anti-centromere antibodies
fibrotic changes limited to fingers, hands, face
includes CREST syndrome:
- calcinosis cutis
- raynaud’s phenomenon
- esophageal dysmotility
- sclerodactyly
- telangiectasia
sclerodactyly
fibrosis of the skin on the hands
taught and tight skin
results in contractures
loss of digital pulp
tapering of the digits
nail changes
facial changes of lcSSC
micro-otia
difficulty opening mouth
loss of rhytides (wrinkles)
morphea
distinct from SSc
no Raynaud’s
no fibrosis of internal organs
erythematous to voliaceous expanding indurated plaques
centrally more sclerotic - can lead to loss of motion and can be disfiguring
en coup de sabre
can see depressed indurated plaque with some hyperpigmentation on the forehead
linea morphea
contracture corssing joint space
want to treat more aggressively
