1. Localized ACLE (malar rash, butterfly rash) 2. Generalized ACLE (lupus maculopapular rash, SLE rash, photosensitive lupus dermatitis) a. TEN-like ACLE

1. Annular SCLE 2. Papulosquamous SCLE Drug-induced

1. Classic DCLE a. Localized DLE b. Generalized DLE 2. Hypertrophic/verrucous DLE 3. Lupus profundus/lupus panniculitis 4. Mucosal DLE 5. Oral DLE 6. Conjunctival DLE 7. Lupus tumidus (urticarial plaque phase of LE) 8. Chillblain LE (chillblain lupus, perniotic lupus) 9. Lichenoid DLE (LE/lichen planus overlap, lupus planus)

61 - Lupus Erythematosus Flashcards by dvojnd rivera

The term LE-specific relates to those lesions displaying a/an

Interface dermatitis

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Most frequent clinical manifestation of LE

Joint inflammation

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Second most frequent clinical manifestation of LE

Skin disease

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ACLE

Localized ACLE (malar rash, butterfly rash)
Generalized ACLE (lupus maculopapular rash, SLE rash, photosensitive lupus dermatitis)
a. TEN-like ACLE

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SCLE

Annular SCLE
Papulosquamous SCLE

*Drug-induced

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CCLE

Classic DCLE
a. Localized DLE
b. Generalized DLE
Hypertrophic/verrucous DLE
Lupus profundus/lupus panniculitis
Mucosal DLE
Oral DLE
Conjunctival DLE
Lupus tumidus (urticarial plaque phase of LE)
Chillblain LE (chillblain lupus, perniotic lupus)
Lichenoid DLE (LE/lichen planus overlap, lupus planus)

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Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds

Malar rash

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Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions

Discoid rash

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Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation

Photosensitivity

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Oral ulcers: oral or nasopharyngeal ulceration, usually (painful/painless), observed by a physician

Painless

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Serositis criteria

a. Pleuritis - convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion
or
b. Pericarditis - documented by electrocardiogram or rub or evidence of pericardial effusion

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Renal disorder criteria

a. Persistent proteinuria - >0.5 g/day or greater than 3+ if quantitation not performed
or
b. Cellular casts - may be red cell, hemoglobin, granular, tubular, or mixed

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Neurologic disorder criteria

a. Seizures - in the absence of offending drugs or known metabolic derangements (eg, uremia, ketoacidosis, or electrolyte imbalance)
or
b. Psychosis - in the absence of offending drugs or known metabolic derangements (eg, uremia, ketoacidosis, or electrolyte imbalance)

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Hematologic disorder criteria

a. Hemolytic anemia - with reticulocytosis
or
b. Leukopenia - < 4000 μL total on 2 or more occasions
or
c. Lymphopenia - < 1500/μL on 2 or more occasions
or
d. Thrombocytopenia - < 100,000 μL in the absence of offending drugs

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Immunologic disorder criteria

a. Anti-DNA - antibody to native DNA in abnormal titer
or
b. Anti-Smith antigen - presence of antibody to Smith nuclear antigen
or
c. Positive finding of antiphospholipid antibodies based on (1) an abnormal serum level of igG or igM anticardiolipin antibodies, (2) a positive test result for lupus anticoagulant using a standard method, (3) a false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test

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Antinuclear antibody criteria

An abnormal titer of antinuclear antibody by immunofluorescence of an equivalent assay at any point in time and in the absence of drugs known to be associated with “drug-induced lupus” syndrome

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1982 Revised Criteria for Classification of SLE: a person shall be said to have SLE if any _____ or more of the _____ criteria present

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_____ SCLE patients are somewhat older at disease onset

Drug-induced

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Most common form of CCLE

Classic DLE

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Approximately _____% of patients presenting with isolated localized DLE subsequently develop SLE

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Most upregulated gene pathway identified in microarray studies in SLE patients

Type 1 IFN

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Produce large amounts of type 1 IFN in response to DNA and RNA stimulation through toll-like receptors 7 and 9

Plasmacytoid dendritic cells

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Probably the most important environmental factor in the induction phase of SLE

UV radiation

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Y/N: Smokers are at a greater risk of developing SLE than are nonsmoker and former smokers

Yes

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Patients with treatment-resistance CLE were much more likely to

Smoke

Patients with LE-specific skin disease who smoke are less responsive to

Anti-malarial treatment

Seroconversion to _____ virus among patients with SLE is nearly universal

Epstein-Barr virus

Y/N: The designations acute, subacute, and chronic, in regard to CLE, refer to how long individual lesions have been present.

No - Refer to the pace and severity of any associated SLE and are not necessarily related to how long individual lesions have been present

Most commonly imputed drug classes causing drug-induced lupus

Antacids Antifungals Biologics Chemotherapeutic

Localized ACLE has commonly been referred to as

Classic butterfly rash or | Malar rash of SLE

Characteristically spared in malar rash

Nasolabial folds

Generalized ACLE presents as a widespread morbilliform or exanthematous eruption often focused over the extensor aspects of the arms and hands and characteristically sparing the

Knuckles

Perivascular nail fold erythema and telangiectasia are considerably more common and occur in more exaggerated forms of

Dermatomyositis | Systemic sclerosis

An extremely acute form of ACLE is rarely seen that can simulate

Toxic epidermal necrolysis

ACLE can be differentiated from true TEN because it

Occurs on predominantly sun-exposed skin | Has a more insidious onset

A finding of circulating autoantibodies to the _____ strongly supports a diagnosis of SCLE

Ro/SS-A ribonucleoprotein particle

Erythema multiforme-like lesions occurring in patients with SLE in the presence of La/SS-B autoantibodies

Rowell syndrome

When the face is involved with SCLE, it is most often the _____, with sparing of the _____

Lateral face | Central, malar regions

A consistent clinical difference is that DLE lesions are characteristically _____, whereas SCLE lesions are not

Indurated

Risk factors for the development of SLE in a patient presenting with SCLE lesions

Papulosquamous type of SCLE Leukopenia High titer of ANA (>1:640) anti-dsDNA antibodies

When the adherent scale is lifted from more advanced DLE lesions, keratotic spikes similar in appearance to _____ can be seen to project from the undersurface of the scale

Carpet tacks

May represent telogen effluvium occurring as the result of flaring systemic disease

Lupus hair

Y/N: LE skin disease activity can be precipitated by any form of cutaneous trauma

Yes - Koebner phenomenom or isomorphic effect

Perforation of the nasal septum is more often associated with _____ than DLE

SLE

Kaposi-Irrgang disease

LE profundus/LE panniculitis

Chillblain LE appears to be associated with _____ antibodies

anti-Ro/SS-A

Helps to distinguish chillblain LE from idiopathic chillblains

Persistence of lesions beyond the cold months Positive ANA Presence of one of the other ACR criteria for SLE

Y/N: The characteristic epidermal histologic changes of LE-specific skin disease are only minimally expressed, if at all in lupus erythematosus tumidus

Yes

Most photosensitive subtype of cutaneous lupus

Lupus erythematosus tumidus

Y/N: Lupus erythematosus tumidus typically demonstrates a poor response to antimalarials

No - good response

Y/N: Lupus erythematosus tumidus lesions tend to resolve completely without either scarring or atrophy

Yes

The presence of LE-nonspecific skin changes, especially when seen in conjunction with LE-specific rashes, corresponds with

Higher systemic disease activity

The laboratory markers for SCLE are the presence of _____ (70% - 90%) and, less commonly, _____ (30% - 50%) autoantibodies

Anti-Ro/SS-A | Anti-La/SS-B

Approximately _____% of the patients with SCLE develop active SLE

Evidence suggests that overlap occurs between SCLE and

Sjogren syndrome

First line treatment for LE-specific skin disease

Topical glucocorticoids, topical calcineurin inhibitor | Intralesional triamcinolone acetonide

Second line treatment for LE-specific skin disease

Hydroxychloroquine Chloroquine Quinacine If monotherapy fails, add quinacrine to either hydroxychloroquine or chloroquine

Short course treatment (2-16 weeks only) for LE-specific skin disease

Prednisone | Thalidomide

Third line treatment for LE-specific skin disease

Azathioprine Mycophenolate mofetil Methotrexate

Maximum dose of hydroxychloroquine

6.5 mg/kg/day based on ideal body weight

Maximum dose of chloroquine

3-4 mg /kg/day

Approximately _____ are required to reach equilibrium blood levels of hydroxychloroquine

6 weeks

If no response is seen after _____ of hydroxychloroquine, quinacrine hydrochloride 100 mg/day can be added

8-12 weeks

Antimalarial that does not cause retinopathy

Quinacrine

Doses of _____ may need to be adjusted for patients with decreased renal or hepatic function

Quinacrine

Chloroquine is generally felt to be more efficacious than hydroxychloroquine in treating CLE, perhaps because of the

Earlier therapeutic responds that might occur as a result of the shorter time period required to reach steady-state blood levels

Should not be used simultaneously because of the enhanced risk of retinal toxicity

Hydroxychloroquine | Chloroquine

(Hydroxychloroquine/Chloroquine) may be more retinotoxic than (Hydroxychloroquine/Chloroquine)

Chloroquine | Hydroxychloroquine

Y/N: Modern hydroxychloroquine regimen can be used safely in children and in women who are pregnant

Yes

Antimalarial associated with higher incidence of side effects

Quinacrine

Commonly produces a yellow discoloration of the entire skin and sclera in fair-skinned individuals, which is completely reversible when the dose of the drug is reduced or discontinued altogether

Quinacrine

Can produce significant hemolysis in patients with G6PD deficiency

Quinacrine

Can produce bone marrow suppression, including aplastic anemia, although this effect is exceedingly rare with the current dosage regimens

Each of the aminoquinolone antimalarials

Significant dose-related and/or methemoglobinemia can result from the use of

Dapsone

Sensory neuropathy is another toxicity associated with _____, and 25% to 75% of patients with CLE develop peripheral neuropathy while taking this drug

Thalidomide

Thromboembolism is a serious adverse event that may occur in patients with a preexisting hypercoagulable state

Thalidomide

Thalidomide analog | US FDA approved for the treatment of multiple myeloma

Lenalidomide

Safety data to date indicates a much lower rate of peripheral neuropathy with (thalidomide/lenalidomide) compared to (thalidomide/lenalidomide); however it has a similar rate of thromboembolism (especially when combined with glucocorticoids) and leukopenia compared to (thalidomide/lenalidomide)

Lenolidomide Thalidomide Thalidomide

Because steroid-induced bone loss occurs most rapidly in the first _____ of use, all patients who do not have contraindications should begin agents to prevent osteoporosis with the initiation of steroid therapy

6 months

(Prednisone/Prednisolone) rather than (Prednisone/Prednisolone) should be used in patients who have significant underlying liver disease, because (Prednisone/Prednisolone) requires hydroxylation in the liver to become biologically active

Prednisolone Prednisone Prednisone

Any amount of prednisone given as a single oral dose in the morning has (more/less) adrenal-suppressing activity than the same amount given in divided doses throughout the day

Less

Any given amount of prednisone, taken in divided doses, has a (greater/lesser) LE-suppressing activity than does the same amount of drug given as a single morning dose

Greater