Lupus: what are its different forms?

Systemic lupus erythematosus (SLE).

The most common form of lupus is systemic lupus, also known as systemic lupus erythematosus (SLE). It is described as systemic or disseminated because it can affect several organs simultaneously. It is driven by the production of autoantibodies, particularly double‑stranded DNA (dsDNA) antibodies, which give rise to widespread inflammation. Its clinical manifestations are highly diverse. Beyond profound fatigue, fever or joint pain, it may involve the mucosa with oral lupus (lesions or ulcers in the mouth), or present as rarer cutaneous forms such as bullous lupus, characterised by the appearance of blisters. These symptoms vary markedly from one person to another, ranging from very mild signs to more complex organ involvement, making each care pathway unique.

The severity of SLE depends primarily on internal organ involvement, particularly when it progresses to lupus nephritis through a direct attack on the renal filtration structures. This severity also increases in neurological forms of lupus, where involvement of the nervous system can disrupt cognitive or motor functions.

Lupus érythémateux systémique. — Systemic lupus erythematosus.
Source: ARNAUD L. Systemic lupus erythematosus – Antiphospholipid syndrome (2020).

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≈ 70%

some individuals with lupus exhibit a systemic form.

30 to 50%

some patients develop renal impairment.

25 to 75%

some patients present neurological deficits.

30 to 40%

develop cardiovascular and pulmonary disorders.

Cutaneous lupus erythematosus (CLE).

Unlike systemic lupus, cutaneous lupus is mainly confined to the skin. It accounts for a substantial proportion of the cases seen in dermatology.

Discoid lupus erythematosus (DLE), a scarring form.

Discoid lupus is the most common form of chronic cutaneous lupus. It presents as well-defined red patches with a scaly surface, which often appear on sun-exposed areas such as the face, scalp or ears. Over time, these lesions can leave permanent scars and may even lead to irreversible hair loss if the scalp is affected. The chronic inflammation of discoid lupus does not merely attack the hair; it destroys the hair follicle in depth. This inflammatory process leads to fibrosis (the transformation of tissues into rigid scar tissue), which permanently replaces the hair roots and thus prevents any new growth.

In some rarer cases, the condition takes on an unusual form known as verrucous lupus, in which the plaques become very thick, firm and rough in appearance, resembling warts. Although this form is striking because of how raised the lesions are, it remains confined to the skin and does not necessarily mean that internal organs are affected, even though regular monitoring is essential to ensure that the disease does not spread to the rest of the body.

Lupus érythémateux discoïde. — Discoid lupus erythematosus.
Source: ARNAUD L. Systemic lupus erythematosus – Antiphospholipid syndrome (2020).

Subacute cutaneous lupus, a highly photosensitive form.

Subacute cutaneous lupus is characterised by marked sensitivity to sunlight. It presents with ring-shaped lesions and red plaques that resemble psoriasis. However, in contrast to psoriasis, it appears less dry, with fine scales localised on sun-exposed areas. These lesions occur mainly on the trunk and arms, and often spare the face, as this is generally better protected in daily life (sun care, hats, avoidance behaviours). These areas of the body also react differently to UV light; their cells are more sensitive and more readily trigger the lupus-related immune response. Unlike discoid lupus, they do not leave scars but can cause pigmentary disorders due to inflammation, which stimulates melanin production (post-inflammatory hyperpigmentation).

Lupus cutané subaigu. — Subacute cutaneous lupus.
Source: ARNAUD L. Systemic lupus erythematosus – Antiphospholipid syndrome (2020).

Drug-induced lupus.

Drug-induced lupus is a particular form of the disease, triggered by certain treatments. Some molecules modify the expression of DNA in cells, which disrupts immune recognition. The immune system then regards these cells as foreign. Among these molecules are isoniazid, used in the treatment of tuberculosis, and hydralazine, recommended for severe arterial hypertension. Likewise, biotherapies such as TNF-α inhibitors, often used in the treatment of Crohn’s disease, rheumatoid arthritis or psoriasis, can occasionally cause lupus-like symptoms. The most typical sign of this form is the presence of anti-histone antibodies (90% of cases). Clinically, symptoms such as fever, joint pain and fatigue are observed. However, in contrast to systemic lupus, severe involvement (kidney, brain) is very rare.

In most cases, the manifestations disappear after discontinuation of the treatment responsible for this form of lupus.

Neonatal lupus erythematosus (NLE).

Neonatal lupus is a particular form that is linked to the systematic transfer of maternal antibodies (anti-SSA/Ro and anti-SSB/La) to the foetus during pregnancy, but the disease manifests itself only in 1 to 2% of cases. The difference lies in the genetic susceptibility of the foetus and in local environmental factors, such as inflammation or cellular stress. These elements all need to be present for the antibodies to genuinely “attack” the baby’s tissues instead of simply circulating.

This form of lupus presents in the newborn with transient cutaneous lesions and, more rarely, cardiac disorders. The most serious complication is atrioventricular block, resulting from involvement of the heart’s electrical conduction system. In most cases, the skin manifestations resolve spontaneously within a few months, as the maternal antibodies are cleared. However, nearly 5% of patients with NLE may go on to develop systemic lupus erythematosus (SLE) during adolescence or early adulthood.

Whether it manifests only at the level of the skin or more widely affects the body as a whole, lupus shows marked variability from one individual to another. It is precisely this variability that can make the initial diagnosis challenging.