10 - 61 - LUPUS ERYTHEMATOSUS - HIGH YIELD

Question 1

Question 2

ACLE-SPECIFIC SKIN DISEASE

Answer 2

Question 3

SCLE-specific skin disease

Answer 3

Question 4

CCLE-specific skin disease

Answer 4

Question 5

LE-nonspecific skin disease

Answer 5

Question 6

most frequent clinical manifestation of LE

Answer 6

Joint inflammation

Question 7

second most frequent clinical manifestation of LE

Answer 7

Skin disease

Question 8

Question 9

most common form of CCLE

Answer 9

classic DLE skin lesion

Question 11

classic DLE skin lesion is present in how many percent of SLE populations?

Answer 11

15% to 30%

Question 12

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

Answer 12

5%

Question 13

Extracutaneous Manifestations of Systemic Lupus Erythematosus

Answer 13

Question 14

most upregulated gene pathway identified in microarray studies in SLE patients

Answer 14

type 1 IFN

Question 15

most important environmental factor in the induction phase of SLE, especially of LE-specific skin disease

Answer 15

UV radiation

Question 16

Causes of Drug-Induced Lupus

Answer 16

Question 17

finding of what circulating autoantibodies strongly supports a diagnosis of SCLE?

Answer 17

autoantibodies to the Ro/SS-A ribonucleoprotein particle

Question 18

risk factors for the development of SLE in patients with SCLE

Answer 18

• papulosquamous type of SCLE,
• leukopenia,
• high titer of antinuclear antibody (ANA) (>1:640),
• anti–double-stranded DNA (dsDNA) antibodies

Question 18

True of False.

SCLE lesions tend to be LESS transient than ACLE lesions and heal with MORE pigmentary change.

Answer 18

TRUE

Question 19

Keratotic plugs accumulate in dilated follicles that soon become devoid of hair. When the adherent scale is lifted from more advanced lesions, keratotic spikes similar in appearance to carpet tacks can be seen to project from the undersurface of the scale. What do you call this sign

Answer 19

“carpet tack” sign

Question 20

True or false.

Patients with generalized DLE (ie, lesions both above and below the neck) have somewhat higher rates of immunologic abnormalities, a higher risk for progressing to SLE, and a higher risk for developing more severe manifestations of SLE than patients with localized DLE

Answer 20

True

Question 21

rare variant of CCLE in which the hyperkeratosis normally found in classic DLE lesions is greatly exaggerated

Answer 21

HYPERTROPHIC DISCOID LUPUS ERYTHEMATOSUS

Question 22

areas of predilection of HYPERTROPHIC DISCOID LUPUS ERYTHEMATOSUS

Answer 22

extensor aspects of the arms, the upper back, and the face

Question 23

T/F. Patients with hypertrophic DLE probably do not have a greater risk for developing SLE than do patients with classic DLE lesions.

Answer 23

True

Question 24

Mucosal DLE occurs in approximately how many % of patients with CCLE.

Answer 24

25%

Question 25

most commonly affected in mucosal DLE

Answer 25

oral mucosa

Question 26

in mucosal DLE, what is the most commonly involved area in the mouth?

Answer 26

Buccal mucosal surfaces Less frequent sites: palate, alveolar processes, and tongue

Question 27

Chilblain LE appears to be associated with what antibody

Answer 27

anti-Ro/ SS-A antibodies

Question 28

variant of CCLE in which the dermal findings of DLE, namely, excessive mucin deposition and superficial perivascular and periadnexal inflammation, are found on histologic evaluation. The characteristic epidermal histologic changes of LE-specific skin disease are only minimally expressed, if at all.

Answer 28

LUPUS ERYTHEMATOSUS TUMIDUS

Question 29

most photosensitive subtype of cutaneous lupus, and typically demonstrates a good response to antimalarials.

Answer 29

LUPUS ERYTHEMATOSUS TUMIDUS

Question 30

laboratory markers for SCLE

Answer 30

anti-Ro/SS-A (70% to 90%) anti-La/SS-B (30% to 50%)

Question 31

ANA are present in how many % of patients with SCLE

Answer 31

60% to 80%

Question 32

LE-specific skin disease histopathology

Answer 32

- hyperkeratosis - epidermal atrophy, - vacuolar basal cell degeneration - dermal–epidermal junction basement membrane thickening, - dermal edema, - dermal mucin deposition, and - mononuclear cell infiltration of the dermal–epidermal junction and dermis, focused in a perivascular and periappendageal distribution

Question 33

ACLE histopath findings

Answer 33

- cell-poor interface dermatitis - sparse lymphohistiocytic cellular infiltrate - mild degree of focal vacuolar alteration of basal keratinocytes - telangiectases and extravasation of erythrocytes - may see individually necrotic keratinocytes - upper dermis: usually shows pronounced mucinosis - UNCOMMON to see basement membrane zone thickening, follicular plugging, or alteration of epidermal thickness - some noted an increase in the number of neutrophils in the infiltrate especially in recent onset

Question 34

SCLE histopath findings

Answer 34

- interface dermatitis, with foci of vacuolar alteration of basal keratinocytes alternating with areas of lichenoid dermatitis - Pronounced epidermal atrophy is often present - Dermal changes: edema, prominent mucin deposition, and sparse mononuclear cell infiltration usually limited to areas around blood vessels and periadnexal structures in the upper one-third of the dermis - Difference from DLE: Lesser degrees of hyperkeratosis, follicular plugging, mononuclear cell infiltration of adnexal structures, and dermal melanophages

Question 35

CCLE histopath findings

Answer 35

- epidermal changes: hyperkeratosis, variable atrophy, and interface changes similar to those described for SCLE (interface dermatitis, with foci of vacuolar alteration of basal keratinocytes alternating with areas of lichenoid dermatitis) - epidermal basement membrane is markedly thickened - Dermal changes: dense mononuclear cell infiltrate composed primarily of CD4 T lymphocytes and macrophages predominantly in the periappendageal and perivascular areas (extends well into the deeper reticular dermis and/ or subcutis - distinguishing feature from ACLE and SCLE), melanophages, and dermal mucin deposition - chronic scarring DLE lesions: decreased inflammatory cell infiltrate; replaced by dermal fibroplasia

Question 36

Question 37

almost always associated with underlying visceral involvement

Answer 37

Acute cutaneous lupus erythematosus (malar rash)

Question 38

most often have skin-only or skin-predominant disease.

Answer 38

Chronic cutaneous lupus (classic discoid lupus erythematosus, lupus panniculitis, chilblain lupus, and tumid lupus erythematosus)

Question 39

reversible, nonscarring alopecia that patients with SLE often develop during periods of systemic disease activity

Answer 39

lupus hair ## Footnote may represent telogen effluvium occurring as the result of flaring systemic disease

Question 40

patients who have both LE **panniculitis** and **DLE** lesions

Answer 40

LE profundus

Question 41

refer to those having only **subcutaneous** involvement (without DLE lesions)

Answer 41

LE panniculitis

Question 42

The overlying skin often becomes attached to the subcutaneous nodules and is drawn inward to produce deep, saucerized depressions The head, proximal upper arms, chest, back, breasts, buttocks, and thighs are the sites frequently affected

Answer 42

LE profundus/LE panniculitis (Kaposi-Irrgang disease)

Question 43

Roughly how many % of patients with LE profundus/ panniculitis have evidence of SLE?

Answer 43

50% ## Footnote * systemic features of patients with LE panniculitis/ profundus tend to be less severe, similar to those of patients with SLE who have DLE skin lesions

Question 43

purple-red patches, papules, and plaques on the toes, fingers, and face, which are precipitated by cold, damp climates and are clinically and histologically similar to idiopathic chilblains (pernio)

Answer 43

Chilblain LE ## Footnote * As they evolve, these lesions usually assume the appearance of scarred atrophic plaques with associated telangiectases. * They may resemble old lesions of DLE or may mimic acral lesions of small vessel vasculitis. * Patients with chilblain LE often have typical DLE lesions on the face and head. * It is possible that chilblain LE begins as a classic acral, cold-induced lesion that then Koebnerizes DLE lesions, thus explaining the spectrum of clinicohistologic findings, which seem to vary based on when, in the course of the lesion, the biopsy sample is taken

Question 44

Approximately how many % of patients presenting with chilblain LE later develop SLE?

Answer 44

20%

Question 44

it is likely that it is one of the most common causes of digital lesions in patients with LE

Answer 44

Chilblain LE

Question 45

succulent, edematous, urticaria-like plaques with little surface change

Answer 45

Lupus erythematosus tumidus

Question 46

the presence of this test correlates positively with risk for developing LE nephritis

Answer 46

nonlesional lupus band test

Question 47

Therapeutic Options for Lupus Erythematosus–Specific Skin Disease

Answer 47

Question 48

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

Answer 48

Quinacrine

Question 49

can produce significant hemolysis in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency (this adverse effect has also been reported to occur rarely with hydroxychloroquine and chloroquine)

Answer 49

Quinacrine

Question 50

breakthrough of CLE activity has been a problem with the long-term use of what medication?

Answer 50

retinoids

Question 51

* FDA approved for SLE * fully human monoclonal antibody directed against B-lymphocyte stimulator (BLyS)

Answer 51

belimumab [Benlysta]