Cutaneous lupus is part of the lupus erythematosus spectrum. The two main types of Lupus are Systemic lupus and Cutaneous lupus. Approximately 10% of cases affected with lupus are that of cutaneous lupus and 65% of the people who have systemic lupus get cutaneous lupus later.
Women are at a higher risk of having lupus. Most of the women having lupus are around 15-44 years of age. However, people of all ages and genders can be affected by it.
In order to diagnose cutaneous lupus, proper understanding of the subtypes through different laboratory tests, physical examination, antibody serology, histology is required. Alongside that, proper application of sun protection and appropriate systemic and topical agents is required for treatment purposes.
What is Cutaneous Lupus?
Lupus is characterized as an autoimmune disease in which the body itself attacks healthy tissues. The main manifestation of cutaneous lupus is the appearance of rashes on skin which is mainly due to sun exposure.
Types of Cutaneous Lupus Erythematosus
Cutaneous Lupus Erythematosus (CLE) is divided into three subtypes:
- Acute cutaneous lupus erythematosus (ACLE): It is characterized by the presence of butterfly rash or malar rash on the face primarily on the cheeks and bridge of the nose. They may also appear on other body parts. And they lead to skin discoloration.
- Subacute cutaneous lupus erythematosus (SCLE): It is associated with annular lesions along with red borders mainly on the parts of the body which are exposed to sunlight. They may give a ring-like appearance which has a darker red outline. They are not itchy, hurtful, or scary. The lesions can result from a reaction to certain medications.
- Chronic cutaneous lupus erythematosus (CCLE): It is characterized by the presence of discoid lesions on the face, ears or scalp which leads to scarring. The lesions appear thick, scaly, red and are round shaped. They are painful and itchy and may give a burning sensation. This condition can lead to discoloration or scarring of skin. If the lesions appear on scalp, then it may lead to permanent hair loss. And severe cases may lead to skin cancer.
Symptoms of Cutaneous Lupus
Following are the symptoms of cutaneous lupus:
- A butterfly-shaped rash that extends to the cheeks and the bridge of the nose
- Sunlight irritation
- Discoid rash, most commonly on the head, arms, chest, or back
- Joint inflammatory disease
- Kidney issues
- Heart or lung problems
- Joint pain, stiffness, and swelling
- The fingers and toes turn white or blue when exposed to stress or cold
- Dry eyes
- Memory loss, headaches, and confusion
- Ulcers in the mouth
Causes of Cutaneous Lupus
The causes and symptoms of cutaneous lupus can result from genetic, environmental, or hormonal factors. This disease is known to run in families and more than 50 genes are commonly found in people with lupus.
It is seen that women are more prone to this disease and the estrogen hormone is believed to play a key role in the development of the disease. And symptoms tend to aggravate during the menstrual period and during gestation.
Cutaneous lupus can result in rash formation when the immune system of the body causes inflammation in the skin. Exposure to ultra-violet rays from fluorescent light and sunlight aggravates the symptoms of this disease. Some medications are also known to cause skin lupus.
Proper diagnosis and treatment of cutaneous lupus is essential to address the issue at the earliest.
Cutaneous Lupus Diagnosis and Testing
The cutaneous lupus diagnosis requires a combination of histopathological examination, clinical assessment, and immunological testing.
Skin Biopsy and Histopathology: A skin biopsy is often conducted to interpret the cutaneous lupus diagnosis. Histopathological features of cutaneous lupus include:
- Interface dermatitis: Lymphocytic infiltration at the dermo epidermal junction.
- Epidermal atrophy: Thinning of the epidermis.
- Follicular plugging: Keratinous material in hair follicles.
These histological findings do indicate the presence of the disease, but they are not exclusive for CLE, so a comprehensive diagnostic approach is required.
Clinical Assessment: Dermatologists play a key role in the initial assessment of CLE. Detailed patient history, including photosensitivity and previous episodes of skin rashes, aids in forming a preliminary diagnosis. Physical examination focuses on identifying characteristic lesions and patterns.
Direct Immunofluorescence (DIF): It is a valuable tool for the cutaneous lupus diagnosis. It involves applying fluorescent antibodies to skin samples to detect the presence of immune deposits. Typical findings in CLE include granular deposits of IgG, IgM, and C3 at the dermo-epidermal junction.
Serological Tests: These tests are not exclusive for CLE; certain serological tests can support the cutaneous lupus diagnosis:
- Antinuclear antibodies (ANA): Frequently positive in SLE, but less specifically for the cutaneous lupus diagnosis.
- Anti-Ro/SSA and Anti-La/SSB antibodies: Often associated with subacute cutaneous lupus erythematosus (SCLE).
MCQ:
Which cutaneous lupus diagnosis method is typically used to confirm the presence of cutaneous lupus erythematosus through the examination of skin tissue?
- Serological tests
- Direct immunofluorescence (DIF)
- Skin biopsy and histopathology
- Reflectance confocal microscopy (RCM)
Treatment of Cutaneous Lupus
Certain lifestyle changes and medications are required to manage Cutaneous lupus:
- Oral medications: The most common drug used by doctors to treat this condition is hydroxychloroquine. Along with this other drug may also be used such as methotrexate to repress the immune system.
- Injections: Corticosteroid medication can be directly injected into the rash which will help to reduce inflammation. They are required to be injected for a few weeks.
- Reduce sun and fluorescent light exposure: It is recommended to limit the exposure to sun and fluorescent light as lupus skin rashes are sensitive to them. It is required to use sunscreen, wear a hat, and sun-protective clothing when one cannot avoid the sun.
- Topical Creams: The two types of topical medications are tacrolimus ointment and corticosteroid creams. The use of topical lotions, creams, and medications can aid in reducing inflammation in the skin. It is recommended to apply these once or twice a day.
Discover more about the treatment options for Cutaneous Lupus.
MCQ:
Which of the following treatments is commonly used as the first-line therapy for cutaneous lupus erythematosus?
- Systemic corticosteroids
- Antimalarial drugs such as hydroxychloroquine
- Methotrexate
- Biologic agents like rituximab
Key Takeaways
The cutaneous lupus diagnosis and testing remain complex, involving various clinical, histological, and immunological evaluations. However, with lifestyle changes, appropriate treatment, and regular monitoring it is possible to manage the symptoms. With that, early cutaneous lupus diagnosis can help in prevention of hair loss, scarring, and other long-term dermatological issues. Moreover, there are multiple research organizations working dedicatedly to find potential treatment options for cutaneous lupus.
