Connective Tissue Disease

Question 1

What are the three major forms of cutaneous lupus?

Answer 1

chronic cutaneous lupus erythematosis - discoid lupus erythematosus (DLE)

subacute cutaneous lupus erythematosis (SCLE)

acute cutaneous lupus erythematosus (ACLE)

Question 2

discoid lupus erythematosus

Answer 2

more common in females and AA

confined to skin

head and upper body scaly, pink brown plaques

scalp lesions result in alopecia

Question 3

subacute cutaneous lupus erythematosus

Answer 3

photosensitive

lateral face, upper trunk, extensor upper extremities

scaling, annular pink papules and plaques

no scarring

srong association with anti-Ro (SSA) antibodies

Question 4

acute lupus erythematosus

Answer 4

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

Question 5

neonatal lupus erythematosus (NLE)

Answer 5

infants whos mothers have anti-Ro antibodies

similar to SCLE - annular, pink scaly plaques

sun exposure NOT needed

dyspigmentation persists

Question 6

internal manifestations of NLE

Answer 6

congenital heart block - almost always present at birth and 2/3 require pacemakers

mortality roughly 20%

hepatobiliary disease

thrombocytopenia

Question 7

dermatomyositis

Answer 7

women more common

proximal extensor inflammatory myopathy

heliotrop eruption - violaceous and periorbital edema

Gottron’s papules

naile changes

poidiloderma

shawl sign

Question 8

Gottron’s Papules

Answer 8

extensor joints - MCP, IP, elbows

pink to volaceous

papules and plaques

often scaling

Question 9

poikiloderma

Answer 9

hyperpigmentation

hypopigmentation

telangiectasia

epidermal atrophy

** have to have all of these

Question 10

shawl sign

Answer 10

anti mi2 antibodies

photodistributed poikiloderma - V of neck and upper back

Question 11

What is the internal involvement of dermatomyositis?

Answer 11

myopathy

pulmonary disease

cardiac disease

calcinosis

malignancy

Question 12

What are skin findings of RA?

Answer 12

deforming arthritis

rheumatoid nodules - specific finding

non-specific cutaneous lesions - vasculitis, bywater’s lesions, neutrophilic dermatoses

Question 13

relapsing polychonrditis

Answer 13

inflammation of cartilaginous structures

anti-body mediated and treate with immunosuppression

Question 14

What are the clinical features of RP?

Answer 14

inflammation of ear, nose, respiratory tract, joints

death is possible

Question 15

Sjogren’s Syndrome

Answer 15

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

Question 16

cutaneous features of Sjogren’s Syndrome

Answer 16

xerosis (dry skin)

petechiae

purpura and vasculitis

annular erythema

urticarial vasculitis

Question 17

What are the two major clinical subtypes of systemic sclerosis?

Answer 17

limited cutaneous and diffuse cutaneous

vascular dysfunction, immune activation, tissue fibrosis

Question 18

diffuse scleroderma

Answer 18

anti Scl70 antibodies

Raynaud’s phenomenon

starts with hands

spreads to forearms, arms, trunk, face, lower extremities

Question 19

limited cutaneous scleroderma

Answer 19

anti-centromere antibodies

fibrotic changes limited to fingers, hands, face

includes CREST syndrome:

• calcinosis cutis
• raynaud’s phenomenon
• esophageal dysmotility
• sclerodactyly
• telangiectasia

Question 20

sclerodactyly

Answer 20

fibrosis of the skin on the hands

taught and tight skin

results in contractures

loss of digital pulp

tapering of the digits

nail changes

Question 21

facial changes of lcSSC

Answer 21

micro-otia

difficulty opening mouth

loss of rhytides (wrinkles)

Question 22

morphea

Answer 22

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

Question 23

en coup de sabre

Answer 23

can see depressed indurated plaque with some hyperpigmentation on the forehead

Question 24

linea morphea

Answer 24

contracture corssing joint space

want to treat more aggressively

Question 25

leukoclastic vasculitis

Answer 25

crop of palpable purpuric papules

may be necrotic

maay have constitutional symptoms

suspicion for systemic involvement - GI, GU, neuro symptoms

Question 26

sarcoidosis

Answer 26

multisystem condition

all have non-caseating granulomas