Author: Amanda Oakley, Dermatologist, Hamilton, New Zealand, 2001. Updated in January 2016.
Lupus erythematosus (LE) is a connective tissue autoimmune 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.
Cutaneous LE comprises several chronic and relapsing LE-specific and LE-nonspecific inflammatory conditions. There can be some overlap.
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:
LE is classified as an autoimmune disorder, 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:
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 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:
About 15% of patients with cutaneous LE have subacute cutaneous LE. One-third of the 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.
More than 100 drugs have been associated with the onset of subacute cutaneous LE. They include:
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 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 LE accounts for 80% of presentations with cutaneous LE. About 25% of patients with chronic cutaneous LE also have systemic 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 is a variant of discoid LE in which there are thickened and warty plaques resembling viral warts or skin cancers. Hypertrophic LE can occur on palms and/or soles. This is also called palmoplantar LE and is a form of acquired keratoderma.
Mucosal LE presents with plaques, ulcers and scaling. Mucosal lesions may predispose to squamous cell carcinoma. Common sites are:
Lupus profundus affects subcutaneous tissue. Other names for lupus profundus are lupus panniculitis and subcutaneous LE.
LE nonspecific cutaneous features are most often associated with SLE. They include:
Chronic cutaneous LE causes facial deformity and scarring. The active and burned-out disease can lead to social isolation and depression.
A 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.
Note: All women with positive anti-Ro or anti-La antibodies should be advised that if they become pregnant there may be a risk to their infant of developing neonatal lupus and congenital heart block. Women with these antibodies should be referred to an obstetrician and consideration may be given to prophylactic administration of hydroxychloroquine or low dose prednisone to prevent heart block in the fetus.
The aim of treatment for cutaneous LE is to prevent flares, improve appearance and to prevent scarring.
Treatment for cutaneous and systemic LE may include:
Severe disease may require more aggressive treatment:
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 the severity is lessened by strict sun protection and avoidance of nicotine.
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