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Cutaneous lupus erythematosus

Author: Amanda Oakley, Dermatologist, Hamilton, New Zealand, 2001. Updated in January 2016.

What is lupus erythematosus?

Lupus erythematosus (LE) is an autoimmune connective tissue disorder that can affect one or several organs. Circulating autoantibodies and immune complexes are due to loss of normal immune tolerance and are pathogenic. Clinical features of LE are highly variable. LE nearly always affects the skin to some degree.

What is cutaneous lupus erythematosus?

Cutaneous LE comprises several chronic and relapsing LE-specific and LE-nonspecific inflammatory conditions. There can be some overlap.

Who gets cutaneous lupus erythematosus?

Cutaneous LE most often affects young to middle-aged adult women (aged 20–50 years) but children, the elderly, and males may be affected.

Important predisposing factors for cutaneous LE include:

What causes lupus erythematosus?

LE is classified as autoimmune, as it is associated with pathogenic antibodies directed against components of cell nuclei in various tissues. UVB irradiation causes keratinocyte necrosis, immune system activation and antibody formation.

Factors that aggravate LE include:

What are the specific features of cutaneous lupus erythematosus?

There are various types of cutaneous LE, classified as acute, subacute, intermittent and chronic cutaneous LE. The revised Cutaneous Lupus Erythematosus Disease Area and Severity Index (RCLASI) can be used to assess disease activity and damage.

Acute cutaneous lupus erythematosus

Acute cutaneous LE affects at least 50% of patients with systemic lupus erythematosus (SLE). Many are sick, young, fair-skinned females.

Specific features of acute cutaneous LE may include:

SLE may also affect joints, kidneys, lungs, heart, liver, brain, blood vessels (vasculitis) and blood cells. It may be accompanied by antiphospholipid syndrome.

More images of cutaneous lupus erythematosus and systemic lupus erythematosus...

Subacute cutaneous lupus erythematosus

About 15% of patients with cutaneous LE have subacute cutaneous LE. One-third of cases are due to previous drug exposure.

Features of subacute cutaneous LE include:

Up to 50% of patients with subacute cutaneous LE may also have a mild form of SLE, resulting in arthralgia (painful joints) or arthritis (joint disease) and low blood counts. Severe SLE is rare in patients with subacute cutaneous LE.

Drug-induced subacute cutaneous LE

More than 100 drugs have been associated with the onset of subacute cutaneous LE. They include:

Neonatal cutaneous lupus erythematosus

Neonatal cutaneous LE arises within 2 months of birth to mothers with known or subclinical subacute cutaneous LE.

Features of neonatal cutaneous LE may include:

A paediatrician should assess all babies born to mothers with subacute LE (or carrying anti-Ro/La) at birth. Mortality in babies with heart block is up to 20%, despite pacemaker implantation.

Intermittent cutaneous lupus erythematosus

Intermittent cutaneous LE, more often known as lupus tumidus, is a dermal form of lupus.

Features of lupus tumidus include:

Lupus tumidus is similar to Jessner lymphocytic infiltrate, in which diagnostic criteria for lupus are absent.

Chronic cutaneous lupus erythematosus

Chronic cutaneous LE accounts for 80% of presentations with cutaneous LE. About 25% of patients with chronic cutaneous LE also have systemic LE.

Discoid LE

Discoid LE is the most common form of chronic cutaneous LE. It is more prevalent in patients with skin of colour, who are at greater risk of postinflammatory hyperpigmentation and hypertrophic scarring.

Hypertrophic LE

Hypertrophic LE is a variant of discoid LE in which there are thickened and warty plaques resembling viral warts or skin cancers. Hypertophic LE can occur on palms and/or soles. This is also called palmoplantar LE, and is a form of acquired keratoderma.

Mucosal LE

Mucosal LE presents with plaques, ulcers and scaling. Mucosal lesions may predispose to squamous cell carcinoma.

More images of cutaneous lupus erythematosus ...

Lupus profundus

Lupus profundus affects subcutaneous tissue and may also be called lupus panniculitis.

What are the nonspecific cutaneous features of lupus erythematosus?

LE nonspecific cutaneous features are most often associated with SLE. They include:

Complications of cutaneous lupus erythematosus

Chronic cutaneous LE causes facial deformity and scarring. Active and burned-out disease can lead to social isolation and depression.

Systemic LE may involve heart, lung and brain with significant morbidity and mortality. Vasculitis and antiphospholipid syndrome involving internal organs can be serious.

How is cutaneous lupus erythematosus diagnosed?

Skin biopsy may be diagnostic, showing a lichenoid tissue reaction and features specific to the kind of cutaneous LE. Direct immunofluorescence tests may show positive antibody deposition along the basement membrane (lupus band test).

Diagnostic features on biopsy are more likely to be found in LE-specific skin lesions than in LE-nonspecific cutaneous LE.

How can cutaneous lupus erythematosus be prevented?

What is the treatment for cutaneous lupus erythematosus?

The aim of treatment for cutaneous LE is to prevent flares, improve appearance and to prevent scarring.

Local therapy

Systemic therapy

Treatment for cutaneous and systemic LE may include:

Severe disease may require more aggressive treatment:

Procedures

What is the outlook for cutaneous lupus erythematosus?

The prognosis for cutaneous LE is variable.

The skin involvement in SLE tends to mirror systemic involvement.

Drug-induced SCLE clears within a few weeks of withdrawal of the responsible drug.

Untreated chronic cutaneous LE tends to persist, but severity is lessened by strict sun protection and avoidance of nicotine.

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