Lupus Erythematosus

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33

Lupus Erythematosus

General

• A multisystem AI-CTD disorder that prominently affects the skin.

• Broadly divided into systemic lupus erythematosus (SLE), cutaneous lupus erythematosus (CLE), and drug-induced lupus erythematosus (DI-LE) (Fig. 33.1).

• CLE is further classified into specific and nonspecific skin lesions, based on the histopathologic presence (specific) or absence (nonspecific, Table 33.1) of an ‘interface dermatitis’; however, this is not a perfect classification scheme because some specific entities (e.g. LE tumidus, lupus panniculitis) do not demonstrate an ‘interface dermatitis’ and other, non-lupus entities may display an ‘interface dermatitis’ on histopathology (e.g. dermatomyositis).

• Classically, the three major forms of specific skin lesions are chronic cutaneous LE (CCLE), subacute cutaneous LE (SCLE), and acute cutaneous LE (ACLE), with CCLE being subdivided into four different entities (see Fig. 33.1).

• Cutaneous lesions may be the sole manifestation of LE or they may be associated with systemic disease (SLE), either concurrently or sequentially.

• For all types of LE: women > men; African Americans or black Africans > other populations; onset typically post-puberty to middle age.

• Histopathologic examination of cutaneous lesions often plays an important role in establishing the diagnosis of CLE; direct immunofluorescence of lesional skin can be helpful in distinguishing CLE from other disorders, in particular lichen planus; accurate subtyping requires clinicopathologic correlation.

• Once a diagnosis of CLE is made, initial and longitudinal evaluation for systemic manifestations of SLE is recommended (Tables 33.2–33.4).

Table 33.2

The American College of Rheumatology 1982 revised criteria for classification of systemic lupus erythematosus.*

Although these criteria are very helpful in distinguishing SLE from other rheumatologic conditions, they are not very helpful in distinguishing other skin diseases from LE. See Appendix for 2012 Systemic Lupus International Collaborating Clinics classification criteria for SLE.

Criterion Basic Definition
 1. Malar rash Fixed erythema, flat or raised over malar eminences
 2. Discoid rash Typical DLE lesions
 3. Photosensitivity Skin rash due to an unusual reaction to sunlight
 4. Oral ulcers Oral or nasal ulceration, observed by a physician
 5. Arthritis Non-erosive, involving ≥2 peripheral joints
 6. Serositis Pleuritis or pericarditis
 7. Renal disorder Persistent proteinuria or cellular casts
 8. Neurologic disorder Seizures or psychosis
 9. Hematologic disorder Hemolytic anemia or leukopenia or thrombocytopenia
10. Immunologic disorder Anti-dsDNA or anti-Sm or antiphospholipid antibodies
11. Antinuclear antibody Abnormal ANA titer, in the absence of drug-induced SLE

* The proposed classification is based on 11 criteria. For the purpose of identifying patients in clinical studies, a person shall be said to have SLE if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation.

DLE, discoid lupus erythematosus.

• Before making a definitive diagnosis of cutaneous lupus, it is necessary to exclude a drug-induced etiology (Table 33.5).

• Treatment options for the various subtypes of CLE are fairly similar (Table 33.6).

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