10 - 61 - LUPUS ERYTHEMATOSUS Flashcards
ACLE-SPECIFIC SKIN DISEASE
SCLE-specific skin disease
CCLE-specific skin disease
LE-nonspecific skin disease
most frequent clinical manifestation of LE
Joint inflammation
second most frequent clinical manifestation of LE
Skin disease
most common form of CCLE
classic DLE skin lesion
classic DLE skin lesion is present in how many percent of SLE populations?
15% to 30%
Approximately how many % of patients presenting with isolated localized DLE subsequently develop SLE
5%
Extracutaneous Manifestations of Systemic Lupus Erythematosus
most upregulated gene pathway identified in microarray studies in SLE patients
type 1 IFN
most important environmental factor in the induction phase of SLE, especially of LE-specific skin disease
UV radiation
Causes of Drug-Induced Lupus
finding of what circulating autoantibodies strongly supports a diagnosis of SCLE?
autoantibodies to the Ro/SS-A ribonucleoprotein particle
risk factors for the development of SLE in patients with SCLE
- papulosquamous type of SCLE,
- leukopenia,
- high titer of antinuclear antibody (ANA) (>1:640),
- anti–double-stranded DNA (dsDNA) antibodies
True of False.
SCLE lesions tend to be LESS transient than ACLE lesions and heal with MORE pigmentary change.
TRUE
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
“carpet tack” sign
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
True
rare variant of CCLE in which the hyperkeratosis normally found in classic DLE lesions is greatly exaggerated
HYPERTROPHIC DISCOID LUPUS ERYTHEMATOSUS
areas of predilection of HYPERTROPHIC DISCOID LUPUS ERYTHEMATOSUS
extensor aspects of the arms, the upper back, and the face
T/F. Patients with hypertrophic DLE probably do not have a greater risk for developing SLE than do patients with classic DLE lesions.
True
Mucosal DLE occurs in approximately how many % of patients with CCLE.
25%
most commonly affected in mucosal DLE
oral mucosa
in mucosal DLE, what is the most commonly involved area in the mouth?
Buccal mucosal surfaces
Less frequent sites: palate, alveolar processes, and tongue
Chilblain LE appears to be associated with what antibody
anti-Ro/ SS-A antibodies
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.
LUPUS ERYTHEMATOSUS TUMIDUS
most photosensitive subtype of cutaneous lupus, and typically demonstrates a good response to antimalarials.
LUPUS ERYTHEMATOSUS TUMIDUS
laboratory markers for SCLE
anti-Ro/SS-A (70% to 90%)
anti-La/SS-B (30% to 50%)
ANA are present in how many % of patients with SCLE
60% to 80%
LE-specific skin disease histopathology
- 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
ACLE histopath findings
- 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
SCLE histopath findings
- 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
CCLE histopath findings
- 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
almost always associated with underlying visceral involvement
Acute cutaneous lupus erythematosus (malar rash)
most often have skin-only or skin-predominant disease.
Chronic cutaneous lupus (classic discoid lupus erythematosus, lupus panniculitis, chilblain lupus, and tumid lupus erythematosus)
reversible, nonscarring alopecia that patients with SLE often develop during periods of systemic disease activity
lupus hair
may represent telogen effluvium occurring as the result of flaring systemic disease
patients who have both LE panniculitis and DLE lesions
LE profundus
refer to those having only subcutaneous involvement (without DLE lesions)
LE panniculitis
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
LE profundus/LE panniculitis (Kaposi-Irrgang disease)
Roughly how many % of patients with LE profundus/ panniculitis have evidence of SLE?
50%
- 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
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)
Chilblain LE
- 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
Approximately how many % of patients presenting with chilblain LE later develop SLE?
20%
it is likely that it is one of the most common causes of digital lesions in patients with LE
Chilblain LE
succulent, edematous, urticaria-like plaques with little surface change
Lupus erythematosus tumidus
the presence of this test correlates positively with risk for developing LE nephritis
nonlesional lupus band test
Therapeutic Options for Lupus Erythematosus–Specific Skin Disease
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 glucose-6-phosphate dehydrogenase (G6PD) deficiency (this adverse effect has also been reported to occur rarely with hydroxychloroquine and chloroquine)
Quinacrine
breakthrough of CLE activity has been a problem with the long-term use of what medication?
retinoids
- FDA approved for SLE
- fully human monoclonal antibody directed against B-lymphocyte stimulator (BLyS)
belimumab [Benlysta]
