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The secret of round erythema: Why do some people suffer from mysterious discoid lupus erythematosus?
Discoid Lupus Erythematosus (DLE) is the most common type of chronic cutaneous lupus and is an autoimmune skin disease. The disease causes a red, painful, inflamed, round rash that is often rough or crusty in appearance, most commonly on the scalp, cheeks, and ears. If it occurs on the scalp, it is more likely to cause hair loss. These rashes can last for years if left untreated. The recurrence and persistence of this disease is distressing for many patients, but what are the reasons behind it?
Signs and symptoms
The skin lesions of discoid lupus erythematosus initially appear as dark red or purple-red round patches that later develop into white and attached scales.
Pattern of lesions
The skin lesions of DLE appear at first as round, flat or slightly raised hard areas. Over time, these lesions may experience significant burning and itching, along with color changes, and may eventually become extensive scarring. When these lesions occur in patients with darker skin, the pigment in the central area often disappears while the surrounding pigment deepens.
Lesion location
Skin lesions are most common in sun-exposed areas, especially the head and neck. Typical areas include the scalp, bridge of the nose, upper cheeks, lower lip, ears, and hands. About 24% of patients also develop lesions in the mouth (most commonly on the roof of the mouth), nose, eyes, or vulva.
Special features
Scalp lesions
When DLE occurs on the scalp, it initially appears as a red, flat or slightly raised area, which can later cause hair loss and develop into extensive scarring. These lesions often become less pigmented and become whiter.
Lip lesions
If the lesion is on the lips, it is usually gray or red in color, with thickening of the superficial skin, known as hyperkeratosis, or a red rim surrounding it.
Cause
Sun exposure is the main trigger of DLE rash. It is not known if there is a clear genetic component, but it is possible that genetic factors predispose some people to the disease.
Mechanism
Experts generally believe that DLE is an autoimmune disease because antibodies can be seen in skin biopsies. However, the relationship between antibodies and discoid lupus lesions has not been established. Therefore, UV light gradually releases substances in the cell nucleus after damaging skin cells, further triggering an inflammatory response of the immune system.
Diagnosis
Doctors often perform blood tests to confirm whether a patient has systemic lupus (SLE). Diagnosis is usually confirmed with a skin biopsy. Typical biopsy findings may include accumulation of IgG and IgM antibody deposits at the epidermal-dermal junction.
Treatment
Currently, treatments for DLE include using sunscreen to avoid direct sunlight and prescription powerful topical steroids. If not effective, oral antimalarials such as hydroxychloroquine may be needed. Patients can also consider more innovative treatments, such as pulsed dye laser.
Prognosis
DLE is a chronic disease and the rash can persist for years. Although 50% of patients recover spontaneously, unfortunately if there are signs of systemic lupus, patients may need long-term management of their symptoms.
Epidemiology
The incidence of DLE is unknown, but it is two to three times more common than systemic lupus. The disease primarily affects young adults and women, with women affected at about twice the rate as men.
Society and Culture
The well-known musician Seal and the once-famous singer Michael Jackson were both diagnosed with discoid lupus during their lifetime, which not only affected his appearance, but also affected his music career.
Facing discoid lupus erythematosus, we can’t help but ask, are there more undiscovered common causes and treatments waiting for us to explore?
