Last updated: February 2024
What is cutaneous lupus erythematosus?
Cutaneous lupus erythematosus (CLE) is a form of lupus that affects the skin. It can either occur in isolation or with systemic lupus erythematosus (SLE), where the internal organs or joints may be involved. There are four main subtypes of CLE.
- Acute CLE (ACLE) almost always occurs with SLE. It is most seen as a ‘butterfly rash’ on the face, although many other forms occur.
- Subacute CLE (SCLE) occurs in approximately 10-15% of SLE cases.
- Intermittent (lupus tumidis)
- Chronic CLE (CCLE) (e.g., discoid lupus erythematosus (DLE), lupus profundus, chilblain lupus)
In some cases, there may be overlap between the different forms of CLE present.
The most common forms of isolated CLE are SCLE and DLE. These subtypes will remain the focus of this information.
Who gets cutaneous lupus erythematosus?
CLE is more common in females and amongst certain racial groups. It is not hereditary or contagious.
What causes cutaneous lupus erythematosus?
CLE is thought to be an “autoimmune” condition where specific antibodies are produced and cause damage to the skin resulting in the appearance of a rash.
The exact cause is unknown. However, genetic and environmental factors are thought to play a role, and sunlight, hormonal factors and certain medications may trigger or exacerbate the condition.
What does cutaneous lupus erythematosus look like?
The appearance of CLE varies greatly depending on the subtype.
Subacute cutaneous lupus erythematosus (SCLE)
- The rash usually appears as red, non-itchy patches on sun-exposed areas such as the upper chest, back and arms.
- There are two main forms of SCLE:
- Papulosquamous SCLE occurs in approximately two thirds of cases and consists of red scaly patches.
- Annular (or polycylic) SCLE occurs in approximately one third of cases and causes red rings or an “arcuate shaped” rash.
- SCLE heals without scarring, although the rash may fade with some skin colour changes.
- Rarely there may be mild symptoms such as tiredness, weakness or joint aches.
Discoid lupus erythematosus (DLE)
- DLE occurs most commonly on sun-exposed sites, in particular the face and scalp.
- It appears as red scaly patches and bumps which can be thickened (‘indurated’) or warty.
- If DLE affects hairy sites, then the inflammation can affect the hair follicles and result in patches of hair loss (alopecia) which may be permanent.
- DLE may occasionally be uncomfortable or itchy.
- DLE heals with scarring and/or skin colour changes. The scarring can sometimes be quite disfiguring and its treatment is often unsuccessful.
How is cutaneous lupus erythematosus diagnosed?
A skin biopsy is usually required for routine histopathology (viewing under microscope) and immunofluorescence (specific stains) to confirm diagnosis of CLE.
Blood tests are also usually taken to look for markers of inflammation and autoantibodies (such as ANA and dsDNA), the latter often being negative in CLE.
How is cutaneous lupus erythematosus treated?
Treatment options will vary depending on the individual and their needs.
There is no cure for CLE. The main goal of treatment is to reduce symptoms and the appearance of the rash, as well as prevent long-term complications such as scarring and hair loss.
General measures are important in the treatment of all forms of CLE, and may include:
- Sun protection and avoidance of direct sunlight (i.e., by seeking out shade, wearing tightly woven longer sleeve clothing, broad brimmed hats and sunglasses) and wearing a SPF 50+ broad spectrum sunscreen, ideally containing a physical blocker such as zinc oxide or iron oxide.
- Vitamin D supplement
- Avoiding soaps which can further dry and irritate the skin and using a good moisturiser regularly.
- Smoking cessation
Topical treatments for CLE may include topical steroids and non-steroid based anti-inflammatory ointments (e.g., pimecrolimus or tacrolimus). In DLE, local steroid injections may be useful in selected cases.
Systemic medications (oral or injections) may also be needed to treat CLE, such as:
- Hydroxychloroquine
- Methotrexate, mycophenolate mofetil, azathioprine
- Retinoids (acitretin or isotretinoin), dispone or cyclosporine are less commonly used.
- Targeted ‘biologic’ treatments (i.e., rituximab) may be used in severe treatment resistant cases, and clinical trials assessing new treatment options are underway
What is the likely outcome of cutaneous lupus erythematosus?
SCLE uncomplicated by systemic disease often has a chronic course with intermittent flare-ups. It often flares during sunnier weather. Sometimes there is spontaneous remission. The skin usually heals without scarring or permanent marks, and can be managed with treatment.
In DLE there may be permanent loss of pigmentation, scarring of the skin and/or hair loss leading to disfigurement and psychological distress. Early successful treatment can reduce the risk of scarring.
Very rarely, some chronic severe and untreated DLE skin lesions may turn into skin cancer (such as squamous cell carcinoma or basal cell carcinoma).
| A/Prof Amanda Saracino | February 2024 |
| A/Prof Amanda Saracino | June 2017 |