02-14 Immunologic Skin Disorders Flashcards
Compare and contrast morphea and systemic sclerosis in terms of clinical features and prognosis. Compare dermatomyositis in children vs. adults in terms of clinical features and relationship to underlying malignancy. Describe the cutaneous features of dermatomyositis. Describe the three types of cutaneous lupus and compare and contrast the typical cutaneous findings on physical exam. Discuss the approach to treatment of dermatomyositis. List and describe the feature
<p>
Auto-immune disorders can affect the skin directly, indirectly or both. —Give an example disease for each scenario and a brief explanation of the mechanism.</p>
<p>
<strong>INDIRECTLY</strong> - Contact Dermatitis</p>
<p>
—Skin is innocent bystander in an acquired immune response to an external allergen</p>
<p>
<strong>DIRECTLY</strong> - Pemphigus foliaceous</p>
<p>
—Auto-immune bullous diseases result from Abs formed against keratinocyte Ags</p>
<p>
<strong>COMBINED</strong> - Neonatal lupus</p>
<p>
—In rheumatic disease the skin can either by a bystander, directly affected or both.</p>
<p>
Lupus 101</p>
<p>
Lupus is a systemic disease caused by:</p>
<ul>
<li>
auto-immune anti-nuclear antibodies</li>
<li>
that form immune complexes</li>
<li>
which trigger complement/damage</li>
<li>
affecting various organs</li>
<li>
including oftentimes the skin</li>
</ul>
<p>
Diagnostic Criteria for Lupus</p>
<p>
Must have 4+ of the following (incl 1+ clinical & 1+ immuno finding)</p>
<p>
CLINICAL FINDINGS:</p>
<ul>
<li>
<strong>--skin--</strong></li>
<li>
1. malar (acute cutaneous) lupus</li>
<li>
2. discoid (chronic cutaneous) lupus</li>
<li>
<em>^^both often photosensitive^^</em></li>
<li>
<strong>--head/face--</strong></li>
<li>
3. Oral or nasal ulcers</li>
<li>
4. Non-scarring alopecia</li>
<li>
5. Neuro dz</li>
<li>
<strong>--other organs--</strong></li>
<li>
6. Arthritis</li>
<li>
7. Serositis (e.g. pleuritis, pericarditis)</li>
<li>
8. Renal dz</li>
<li>
<strong>--blood--</strong></li>
<li>
9. Hemolytic anemia</li>
<li>
10. Thrombocytopenia</li>
<li>
11. Leukocytopenia</li>
</ul>
<p>
IMMUNOLOGIC FINDINGS:</p>
<ol>
<li>
ANA+</li>
<li>
Anti-DNA+</li>
<li>
Anti-Sm+</li>
<li>
Anti-Phospholipid+</li>
<li>
Low complement (C3, C4, CH50-total comp)</li>
<li>
Direct Coomb's test+ (in absence of hemolytic anemia)</li>
</ol>
<p>
Acute Cutaneous Lupus</p>
<ul>
<li>
Clinical presentation?</li>
<li>
% w/ systemic dz?</li>
</ul>
<p>
CLINICAL PRESENTATION</p>
<ul>
<li>
classically facial malar rash; can be other places, too</li>
<li>
often photosensitive</li>
<li>
may be anti-dsDNA+</li>
</ul>
<p>
% W/ SYSTEMIC DZ</p>
<ul>
<li>
90%</li>
</ul>
<p>Subactue Cutaneous Lupus (SCLE)</p>
<ul>
<li>Clinical presentation?</li>
<li>% w/ systemic dz?</li>
</ul>
<ul>
<li>Widespread, red scaly rash on the arms, chest, upper back, and occasionally face</li>
<li>Very photosensitive</li>
<li>Usually ANA negative, but anti-Ro (+)* and anti-La (+)
<ul>
<li>*cross placenta→ neonatal lupus</li>
</ul>
</li>
<li>Sometimes caused by medications (HCTZ, NSAIDS, terbinafine, diltiazem)</li>
<li>50% of the time see underlying systemic lupus</li>
<li>Increased risk of mother transmitting Abs to fetus and causingj neonatal lupus</li>
</ul>
<p>Neonatal Lupus</p>
<ul>
<li>risk for children of mothers w/ SCLE, anti-Ro abs (abs cross the placenta)</li>
<li>transient skin rash on face, around eyes, trunk</li>
<li><u>risk of complete <strong>heart block</strong></u>
<ul>
<li>may require pacemaker</li>
</ul>
</li>
<li>may have associated hepatobiliary disease, thrombocytopenia</li>
</ul>
<p>
Chronic Cutaneous Lupus</p>
<ul>
<li>
A.K.A.?</li>
<li>
Presentation</li>
<li>
Frequency of Systemic Disease</li>
<li>
</li>
</ul>
<ul>
<li>
old terminology: “discoid lupus”</li>
<li>
discoid lesions that are photosensitive</li>
<li>
can be seen in setting of:
<ul>
<li>
systemic lupus (~10%)</li>
<li>
purely cutaneous disease: CCLE</li>
</ul>
</li>
<li>
low risk of progression to SLE
<ul>
<li>
5-10%</li>
<li>
should rule out at initial diagnosis</li>
</ul>
</li>
<li>
can cause significant scarring
<ul>
<li>
esp since often favors face/scalp.</li>
</ul>
</li>
</ul>
<p>
Treatment options for cutaneous lupus</p>
<ul>
<li>
sunprotection
<ul>
<li>
High SPF sunscreen with physical blockers</li>
</ul>
</li>
<li>
topical and intralesional corticosteroids</li>
<li>
antimalarials (hydroxychloroquine)
<ul>
<li>
Usual choice for SCLE</li>
<li>
Risk of retinal toxicity</li>
</ul>
</li>
<li>
systemic immunosuppressants</li>
<li>
retinoids</li>
<li>
thalidomide</li>
</ul>
<p>
Clinical Features of Dermatomyositis</p>
<div>
May be combined (dermatomyositis), only derm (amyopathic dermatomyositis) or only myopathic (polymyositis)</div>
<div>
<em>NOTE:severity of skin and muscle involvement do not necessarily correlate</em></div>
<div>
</div>
<div>
SKIN FINDINGS</div>
<ul>
<li>
Heliotrope rash: violaceous scaly rash on eyelids and face [IMAGE HERE]</li>
<li>
Gottron’s papules: flat red papules on dorsal surface of hands, usually over joints</li>
<li>
Gottron's sign:symmetric, scaly, erythematous rash over the extensor surfaces of the hands (usually over joints)</li>
<li>
Peri-ungual telangiectasias and ragged cuticles on hands</li>
<li>
"Mechanic's Hands" (Erythema and hyperkeratosis)</li>
<li>
Photosensitive, red, scaly rash: on face, dorsal arms, chest, upper back</li>
<li>
Calcinosis cutis (in children): ectopic Ca deposits in tissue, can be painful and debilitating</li>
</ul>
<p>
MUSCLE FINDINGS</p>
<ul>
<li>
Proximal muscle weakness: difficulty rising from chair or combing hair</li>
<li>
Elevated muscle enzymes (CPK) and abnormal electromyogram</li>
</ul>
<p>
SYSTEMIC FINDINGS</p>
<ul>
<li>
May have cardiac and pulmonary involvement</li>
</ul>
<p>
Who gets dermatomyositis?</p>
<ul>
<li>
affects both children and adults but has bimodal distribution:
<ul>
<li>
average age onset • 7.6 yrs children • 52 yrs adults</li>
</ul>
</li>
<li>
female preponderance in adults</li>
<li>
children more likely to get calcinosis cutis</li>
</ul>
<p>
Dermatomyositis and malignancy</p>
<ul>
<li>
associated with malignancy in adults: 10-50%
<ul>
<li>
may precede onset of symptoms</li>
<li>
most common types of cancer: lung, breast, ovaries, GI tract</li>
<li>
vigilant screening mandated</li>
<li>
resurgence of skin disease sometimes a marker for recurrence</li>
</ul>
</li>
<li>
malignancy may be associated with:
<ul>
<li>
cutaneous vasculitis</li>
<li>
“malignant erythema”</li>
<li>
ulcerations</li>
<li>
skin disease refractory to treatment</li>
</ul>
</li>
<li>
NOT typically associated with malignancy in children</li>
</ul>
<p>
Dermatomyositis: Dx</p>
<ul>
<li>
skin biopsy may be helpful</li>
<li>
evaluate for muscle involvement
<ul>
<li>
muscle enzymes</li>
<li>
MRI</li>
<li>
muscle biopsy</li>
<li>
EMGs</li>
</ul>
</li>
<li>
ANA may be negative; may have anti-synthetase antibodies (inc. Jo-1)
<ul>
<li>
associated with pulmonary disease</li>
</ul>
</li>
</ul>
<p>
Dermatomyositis Tx</p>
<ul>
<li>
skin and muscle disease may respond differently to tx</li>
<li>
Sun protection (photosensitive)</li>
<li>
systemic corticosteroids initial therapy for muscle disease</li>
<li>
steroid-sparing agents (methotrexate, azathioprine) may be added</li>
<li>
skin disease may respond to topical corticosteroids, antimalarials</li>
<li>
Intravenous immunoglobulin (IVIG) for refractory cases</li>
</ul>
<p>
Compare sterotypical facial rash of lupus and dermatomyositis</p>
<p>
see image</p>
<p>
Compare hand lesions: lupus v. dermatomyositis</p>
<p>
Which is which?</p>
<p>
top one is lupus</p>
<p>
bottom one is Gottron's papules in dermatomyositis</p>
<p>
Types of Scleroderma</p>
<p>
Morphea (just cutaneous) --> CREST (limited systemic) --> systemic sclerosis</p>
<p>Morphea</p>
<ul>
<li>Presentation</li>
<li>Tx</li>
<li>Natural Hx</li>
</ul>
<p>Presentation</p>
<ul>
<li>characteristic lesions<br></br>
– inflammatory patches and plaques that expand usu. on trunk/extremities<br></br>
– may initially be violaceous or hyperpigmented<br></br>
– over time, patch becomes sclerotic and may lose pigment to become ivory white
<ul>
<li>Can cause significant disfigurement if present on face</li>
</ul>
</li>
<li>may involve fat with loss of subcutaneous tissue</li>
<li>contractures</li>
<li>
<p>in sum: an inflammatory dz localized to skin and subQ tissues w/o associated systemic dz, sclerodactyly, Raynaud’s,lab abnormalities orfatality</p>
</li>
</ul>
<p>Tx —Challenging:</p>
<ul>
<li>corticosteroids</li>
<li>Phototherapy (PUVA)</li>
<li>vitamin derivatives (Vits A and D)</li>
<li>immunosuppressive agents</li>
</ul>
<p>Natural Hx</p>
<ul>
<li>disease usually progresses for several years, then regresses (“burns out”)</li>
</ul>
<p>
Scleroderma</p>
<ul>
<li>
Subtypes?</li>
<li>
Skin Findings?</li>
<li>
Systemic findings?</li>
<li>
Extent of spread?</li>
<li>
Immuno testing results?</li>
<li>
Treatment?</li>
<li>
Prognosis</li>
</ul>
<ul>
<li>
Subtypes?
<ul>
<li>
CREST Syndrome</li>
<li>
Systemic Sclerosis</li>
</ul>
</li>
<li>
Skin Findings?
<ul>
<li>
thickening and contraction of the skin</li>
<li>
hyperpigmentation</li>
<li>
telangiectasias</li>
<li>
digital ulcers</li>
<li>
other cutaneous ulcers</li>
<li>
sclerodactyly</li>
</ul>
</li>
<li>
Systemic findings are variable and include:
<ul>
<li>
Raynaud’s phenomenon</li>
<li>
arthralgias</li>
<li>
esophageal/GI</li>
<li>
lung</li>
<li>
kidney and</li>
<li>
heart involvement</li>
<li>
sicca sx (dry eyes, mouth)</li>
</ul>
</li>
<li>
Extent of spread
<ul>
<li>
Involved can be local or diffuse; diffuse carries poorer prognosis</li>
</ul>
</li>
<li>
Immuno testing results
<ul>
<li>
Largely a clinical dx</li>
<li>
Skin biopsy can confirm cutaneous lesions</li>
<li>
95% ANA+</li>
<li>
pulm fibrosis assoc'd w/ Anti-Topoisomerase I (Scl 70) Abs</li>
<li>
CREST syndrome assoc'd w/ Anti-centrosome Abs</li>
</ul>
</li>
<li>
Treatment is tough</li>
<li>
<ul>
<li>
Skin sx often unresponsive to treatment</li>
<li>
Put out fires:
<ul>
<li>
calcium channel blockers for Raynaud’s (or IV alprostadil (PGE1) if refractory Reynaud's)</li>
<li>
prostacyclin infusion for pulm HTN</li>
<li>
ACE inhibitors for renal disease
<ul>
<li>
used to be the killer pre-ACEIs, now lung dz is killer</li>
</ul>
</li>
</ul>
</li>
<li>
Systemic immunomodulators: e.g. MTX, azathioprine, cyclophosphamide, steroids, UV light, thalidomide etc.</li>
</ul>
</li>
<li>
Prognosis
<ul>
<li>
CREST syndrome has better prognosis</li>
<li>
Systemic disease often fatal, esp w/ signif cardiac, lung, or renal involv.</li>
</ul>
</li>
</ul>
<p>
CREST Syndrome includes?</p>
<ul>
<li>
<u>C</u>alcinosis</li>
<li>
<u>R</u>aynaud’sphenomenon</li>
<li>
<u>E</u>sophageal dysmotility</li>
<li>
<u>S</u>clerodactyly</li>
<li>
<u>T</u>elangiectasias</li>
</ul>
<p>
Dx?</p>
<p>
Acute cutaneous lupus</p>
<p>
What are dxs here?</p>
<p>
L = malar rash of SLE</p>
<p>
R = helitropic lesion of dermatomyositis</p>
<p>
What is the dx?</p>
<p>
Morphea</p>
<p>
What are these lesions called? What are they commonly caused by?</p>
<p>
Digital uclers caused by Systemic Scleroderma</p>
<p>
What are these lesions called? What are they caused by?</p>
<p>
Periungal telangiectasias associated w/ Dermatomyositis</p>
<p>
What is this kiddo suffering from? Etiology?</p>
<p>
Neonatal systemic lupus erythmatosus - caused by Anti-Ro IgG from mother with Subacute Cutaneous Lupus</p>
<p>
What is this?</p>
<p>
morphea, again</p>
<p>
Dx?</p>
<p>
Discoid lesions of Chronic Cutaneous Lupus, re-call that these lesions can cause significant scarring (unlike the more acute lesions which are less likely to cause scarring.</p>
