What are the types of Lupus?

Types of lupus

Lupus is an autoimmune condition with varied systemic manifestations. It is a chronic illness affecting mostly females during their reproductive years. Systemic lupus erythematosus (SLE) is not easy to diagnose or manage. Patients who develop lupus are believed to have inherited or acquired defects of their immune system either through the presence of susceptible genes or by an environmental trigger. The development of antibodies against ones own cells contributes to its main pathology. There are four types of lupus:

 

1. Cutaneous lupus erythematosus

This type is limited to the skin. Cutaneous lupus was the first type of lupus to be diagnosed. This type affects the skin and can cause thick, red, scaly rashes on the face, neck, and scalp that can lead to scarring. There are three types of cutaneous lupus rashes:Acute cutaneous, subacute cutaneous and chronic cutaneous. The cutaneous types of lupus are categorised by clinical morphology, average duration of skin lesions and routine histopathologic examination.The classification of CLE can be difficult and confusing but the improved classification in 1979 by the American dermatologists (Gilliam and Sontheimer) has gained wide acceptance.[1] According to Gilliam and Sontheimer, the cutaneous manifestations of LE can be divided into LE-specific and LE-non-specific skin manifestations based on histopathological findings.[2] The LE-specific skin manifestations show a typical histopathological picture with a lichenoid tissue reaction. LE-specific skin manifestations can be further subdivided into acute CLE (ACLE), subacute CLE (SCLE) and chronic CLE (CCLE) where classic discoid LE (DLE) is the most common form. An alternative classification has also been suggested which includes lupus erythematosus tumidus (LET) as a separate subgroup; the intermittent subtype of CLE (ICLE).[3] The LE-non-specific skin manifestations include a wide range of symptoms with different histopathological pictures. The LE-non-specific skin manifestations are not exclusive to LE disease but are often seen in patients with active SLE but also in several other autoimmune diseases.

Acute cutaneous may be localised (most often as a malar or ‘butterfly’ rash) or generalised. Of the three cutaneous types of lupus, ACLE is often seen in patients with active systemic disease. Flat red patches on the cheeks and nose called a butterfly rash that looks quite like sunburn characterize the most common form of ACLE. Individuals may also present with generalized flat red patches on arms, legs and trunk. These lesions are sensitive to the sun (photosensitive to both sun rays and tanning rays) and therefore commonly appear on sun-exposed areas. While these lesions do not often result in scarring, they may leave dark or light pigment changes. Other lesions associated with ACLE include oral ulcers, hives, and temporary hair loss, which are replaced by new hair once the disease flare is treated. Systemic lupus erythematosus (SLE) can also affect the kidneys heart, lungs, liver, brain, joints, blood cells and blood vessels. Damage to the blood vessels results in small red-purple lesions on the lower legs called vasculitis.

SCLE is highly photosensitive, with predominant distribution on the upper back, shoulders, neck, and anterior chest. SCLE is frequently associated with positive anti-Ro antibodies and may be induced by a variety of medications.

Chronic cutaneous Classic discoid LE is the most common form of CCLE, with indurated scaly plaques on the scalp, face, and ears, with characteristic scarring and pigmentary change (Walling and Sontheimer 2012)

In the most common form, discoid LE, unsightly red scaly patches develop which leave postinflammatory pigmentation and white scars. It may be localised or widespread.

SCLE is often characterised by two forms including papulosquamous lesions and annular lesions. Papulosquamous lesions often appear as red scaly patches that look psoriasiform. Annular lesions are ring-shaped with a small amount of scale on the edge of the lesions. These lesions do not itch and often appear on the chest as well as the upper back and neck, however, may also be seen on the face and arms. SCLE is not often associated with significant systemic disease however it is always important to be evaluated by your physician. Furthermore, it is not uncommon for patients with SCLE to have associated joint disease.

Discoid LE predominantly affects the cheeks, nose and ears, but sometimes involves the upper back, V of neck, and backs of hands. Hypertrophic LE results in thickened and warty skin resembling viral warts or skin cancers. Rarely, discoid LE occurs on the palms and/or soles (palmoplantar LE). If the hair follicles are involved, they are first plugged with adherent scale and then bald areas can develop. If the follicles are destroyed, the bald patches are permanent (scarring alopecia). Discoid LE may affect the lips and inside the mouth, causing ulcers and scaling. These lesions may predispose to squamous cell carcinoma. For advanced reading on Cutaneous Lupus click here.

 

2. Drug-induced lupus erythematosus

Drug-induced lupus is a rare, almost always temporary form of lupus that can occur as a side effect of certain medications, including several drugs commonly used to treat heart disease and hypertension. Unusual when compared to statistics for other forms of lupus, men are more likely to develop drug-induced lupus than women. Drug induced lupus only occurs after long-term (months to years) daily use of a medication, and once the medication is stopped, symptoms of drug-induced lupus typically disappear completely within six months. Drug induced lupus does not lead to systemic lupus. For a list of drugs that cause lupus click here.

 

3. Neonatal lupus erythematosus

This is a rare form of lupus in newborn babies whose mothers have lupus that can cause problems at birth or in rare cases, a serious heart defect. This occurs when a mother with certain kinds of lupus [antibodies] transfers them to her child at the time of birth. The mother may have the antibodies but not have lupus herself. In fact, less than 50% of mothers of babies with neonatal lupus actually have lupus.

 

4. Systemic Lupus erythematosus

Systemic Lupus Erythematosus (SLE) is typically the most common form of lupus and is generally considered more serious than the other three forms. SLE can affect many parts of a person’s body, including kidneys, heart, lungs, brain, blood and skin. Symptoms tend to vary among patients and can change often and suddenly. In SLE the immune system cannot differentiate between foreign invaders and healthy tissues, thereby causing inflammation, pain and damage in body with the generation of autoantibodies.

 

References

1. Sontheimer RD, Thomas JR, Gilliam JN. Subacute cutaneous lupus erythematosus: A cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol. 1979;115:1409–15. [PubMed]
2. Gilliam JN, Sontheimer RD. Distinctive cutaneous subsets in the spectrum of lupus erythematosus. J Am Acad Dermatol. 1981;4:471–5. [PubMed]
3. Kuhn A, Bein D, Bonsmann G. The 100th anniversary of lupus erythematosus tumidus. Autoimmun Rev. 2009;8:441–8. [PubMed]
4. Cardinali C, Caproni M, Bernacchi E, Amato L, Fabbri P. The spectrum of cutaneous manifestations in lupus erythematosus-the Italian experience. Lupus. 2000;9:417–23. [PubMed]