Cutaneous Lupus

Question 1

How is Cutaneous Lupus classified ?

Answer 1

Cutaneous Lupus is divided into specific and not specific skin lesions based upon whether the histopathology demonstrated an interface dermatitis or not, respectively. Within the category of specific cutaneous lesions, it is divided into 1) acute cutaneous LE, 2) subacute cutaneous LE, and 3) chronic cutaneous LE.

Question 2

which type has the potential for atrophy or scarring

Answer 2

chronic cutaneous lupus

Question 3

what is the Predominant locations of inflammatory infiltrates in subsets of cutaneous lupus erythematosus.

Answer 3

superficial dermis, ACLE and SCLE

DLE; superficial plus deep dermis and periadnexal,

superficial and deep dermis, LET;

subcutaneous fat, LEP.

The final diagnosis requires clinicopathologic correlation.

Question 4

What are the main areas of involvement of discoid lupus ?

Answer 4

face, scalp and ears (Fig. 41.4),

but may be present in a more widespread distribution (Fig. 41.5).

It is unusual for discoid lesions to be present below the neck without lesions also being present above the neck.

Question 5

Can discoid lupus invlove mucosal surfaces ?

Answer 5

Occasionally, discoid lesions develop on mucosal surfaces, including the lips, nasal mucosa, conjunctivae, and genital mucosa.

Question 6

Does discoid lupus show a photosensitive distribution

Answer 6

Sometimes it does

other times appears on sun protected areas

Question 7

Describe features of discoid lupus

Answer 7

active lesions typically feel thicker and firmer than surrounding uninvolved skin.

The adnexa are prominently involved, with follicular plugging and scarring alopecia commonly observed.

Dyspigmentation is a common sequela noted in longstanding lesions, typically with hypopigmentation in the central area and hyperpigmentation at the periphery (Fig. 41.6), but sometimes with vitiligo-like depigmentation.

Question 8

which type of cancer can appear on long standing DLE lesions ?

Answer 8

Rarely, squamous cell carcinoma develops in a longstanding discoid lesion.

Question 9

Is there an unsual variant for DLE ?

Answer 9

An unusual variant of DLE is hypertrophic DLE, characterized by thick scaling overlying the discoid lesion or occurring at the periphery of the discoid lesion.

The intensely hyperkeratotic lesions are often prominent on the extensor arms (see Fig. 41.5H), but the face and upper trunk may also be involved.

Frequently, there are typical discoid lesions present in other locations.

Question 10

Is subacute lupus photosensitive

Answer 10

true

Question 11

which part of the body is usually spared in subacute lupus?

Answer 11

midface

Question 12

what are the common areas of involvement in subacute lupus ?

Answer 12

sides of the face, upper trunk and extensor aspects of the upper extremities are commonly involved

Question 13

Describe the two presentations of aubacute lupus

Answer 13

annular configuration, with raised pink–red borders and central clearing (Fig. 41.8),

or a papulosquamous presentation with a chronic psoriasiform or eczematous appearance.

Question 14

when taking history for a patient presenting with subacute lupus, what is important to ask about and why ?

Answer 14

DRUGS

In ~20–30% of patients with SCLE, the disease is linked to medications

Question 15

What are the most common medications linked to subacute lupus ?

Answer 15

Terbinafine

Thiazide diuretics (e.g. hydrochlorothiazide)

TNF-α inhibitors

Proton pump inhibitors (e.g. lansoprazole, pantoprazole, omeprazole)

Calcium channel blockers (e.g. diltiazem, nifedipine, verapamil)

Anti-epileptics (e.g. carbamazepine)

Taxanes (e.g. docetaxel, paclitaxel)

Thrombocyte inhibitors (e.g. ticlopidine

Question 16

What type of antibodies are associated with subacute LE what precentage are positive ? ?

Answer 16

anti-SSA/Ro autoantibodies

it is likely that a substantial majority of patients with this condition (~70% in a large series, reported range of 60–100%) have anti-SSA/Ro antibodies.

Question 17

what is another disease that also associated with the same antibodies as subacute cutaneous LE ?

Answer 17

Sjögren syndrome as well as SCLE, it is not surprising that some patients have features of both conditions, and some may have serious internal manifestations of Sjögren syndrome such as pulmonary or neurologic disease.

Question 18

what serologic and clinical disease has been associated with malar rash ?

Answer 18

An association with anti-dsDNA antibodies and lupus nephritis has been proposed and is plausible, although some patients with a malar rash have neither anti-dsDNA antibodies nor lupus nephritis.

Question 19

what is the typical rash of acute LE ?

Answer 19

bilateral malar rash

The morphology of the lesions ranges from mild erythema to intense edema

lesions tend to be transient, follow sun exposure, and resolve without scarring (but sometimes with dyspigmentation).

The duration may range from a few hours to several weeks.

Question 20

what is the differential diagnosis of malar rash of acute lupus ?

Answer 20

seborrheic dermatitis and the erythematotelangiectatic type of rosacea

In malar rash there is there is often sparing of the nasolabial fold

but also in rosacea ???

Question 21

TRUE OR FALSE

acute lupus only presents on the cheeks

Answer 21

false.

sometimes lesions may be more widespread in distribution (Fig. 41.10; see Fig. 41.4).

When lesions occur on the hands, the knuckles are typically spared. It is not unusual for patients with ACLE also to have oral ulcerations.

also remember in subacute LE if it presents in the hands there is sparing of the proximal interphalangeal joints.

Question 22

what is Rowell syndrome ?

Answer 22

The presence of erythema multiforme-like lesions in lupus patients has been termed Rowell syndrome51. Rarely, patients develop an acute eruption clinically similar to toxic epidermal necrolysis or erythema multiforme major (see below). These lesions may represent a severe variant of ACLE or, in some cases, SCLE.

Question 23

what is the differential diagnosis of Lupus erythematosus tumidus

Answer 23

1. urticarial plaques” described in lupus patients.
2. urticarial vasculitis
3. plaque form polymorphic light eruption
4. Lymphocytic infiltrate of Jessner
5. (cutaneous lymphoid hyperplasia (pseudolymphoma)
6. reticular erythematous mucinosis,
7. cutaneous lymphoma

Subacute and chronic cutaneous LE are distinguished by the presence of secondary changes, including scale, follicular plugging and central hypopigmentation, along with interface changes histologically.

Because interface changes are sparse, if present at all, in LE tumidus, distinction from LIJ may prove impossible.

Reticular erythematous mucinosis, which is considered by some as synonymous with LE tumidus, has abundant dermal mucin

Cutaneous lymphomas can have significant clinical and histopathologic overlap with LIJ. If the dermal lymphocytic infiltrate is extensive, immunophenotypic analyses may help to distinguish between the two entities

Question 24

What distinguishes lupus erythematous tumidus ?

Answer 24

lacks scale or follicular plugging because of uninvolved epidermis

Question 25

what are the main extracutaneous manifestations of neonatal lupus

what is the timing of appearance ?

Answer 25

The major extracutaneous findings are congenital heart block (with or without cardiomyopathy), hepatobiliary disease and cytopenias, in particular thrombocytopenia.

The heart block is almost always present by birth, but on rare occasions has developed after birth

Hepatobiliary disease and cytopenias, especially thrombocytopenia, may be present at birth, or they may develop within the first few months of life57