What Is Lupus Pernio?

Lupus erythematosus (LE) is a typical autoimmune connective tissue disease, which is more common in women aged 15-40. Lupus erythematosus is a disease spectrum disease that can be divided into discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE), systemic lupus erythematosus (SLE), deep lupus erythematosus (LEP), and neonatal Lupus erythematosus (NLE), drug-induced lupus erythematosus (DIL) and other subtypes.

Basic Information

English name: lupus erythematosus
Visiting department: Division of Rheumatology
Multiple groups: 15 to 40 year old women
Common locations: skin

Common causes: Caused by infection, environment, endocrine and other factors
Common symptoms: Butterfly erythema, skin lesions, visceral damage, or other connective tissue disease
Contagious: no

Causes of lupus erythematosus

The cause is not fully understood, and it is currently believed to be related to the following factors.

Genetic factor

The incidence of systemic lupus erythematosus has a tendency to cluster in families. 0.4% to 0.5% of first-degree or second-degree relatives of SLE patients have LE or other autoimmune diseases; the rate of SLE in single-ovum twins can be as high as 70% (24 % 69%), and the ratio of fraternal twins is 2% -9%. At present, there are more than 50 loci associated with SLE, mostly HLA and genes, such as DR2 in HLA D region. C4AQ in DR3, DQA1, DQB1 and HLA class III genes.

2. Sex hormones

The disease is more common in women of childbearing age, and pregnancy can induce or exacerbate SLE. But the evidence is still insufficient.

3. Environmental factors and other

Ultraviolet radiation can stimulate or aggravate LE, which may be related to its damage to keratinocytes. It is related to changes in DNA or the release of "hidden antigens" or the expression of neoantigens that cause the body to produce corresponding antibodies, thereby forming immune complexes and causing damage. Drugs such as hydrazine, dacarbazine, procaine, methyldopa, isoniazid, and penicillin can induce drug-induced lupus erythematosus. Certain infections (such as streptococcus, EB virus, etc.) can also induce or exacerbate the disease.

Clinical manifestations of lupus erythematosus

Discoid lupus erythematosus: It mainly attacks the skin and is the lightest type of lupus erythematosus. A few can have mild visceral damage, and a few cases can turn into systemic lupus erythematosus. At the beginning of skin damage, there are one or several bright red spots, mung beans to soybeans are large, and there are adhesive scales on the surface. Later, they gradually expand, round or irregular, and the edge pigments are significantly deeper, slightly higher than the center. The central color is pale, can be atrophic, low-lying, and the entire skin is discoid (hence the name discoid lupus erythematosus). The damage is mainly distributed in areas exposed to sunlight, such as the face, ear wheels, and scalp. A few can affect the upper chest, back of hands, forearms, lips and oral mucosa. Most patients have no symptoms of skin lesions, but it is difficult to completely resolve them. The new damage can gradually increase or remain unchanged for many years. The damage evacuation is symmetrically distributed, or they can be merged into a piece, and the damage in the middle of the face can be merged into a butterfly shape. Discoid skin lesions worsen after sun exposure or exertion. Damage on the scalp can cause permanent hair loss. Old damage can occasionally develop into skin squamous cell carcinoma.

Subacute cutaneous lupus erythematosus, which is rarely seen clinically, is a special intermediate type. There are two types of skin damage. One is ring-shaped erythema, which is a single or multiple scattered erythema, which is ring-shaped, semi-ring-shaped, or multi-ring-shaped. The dark red edge is slightly edema, and the outer edge has redness. It has pigmentation and telangiectasias, and it occurs in the face and trunk. The other type is pimples and scaly types. The skin lesions are similar to psoriasis, including erythema, pimples, and patches. There are obvious scales on the surface, which are mainly distributed in the upper limbs and face of the trunk. Most of the two skin lesions exist separately, and a few can exist at the same time. Skin lesions often recur, and most patients have visceral damage, but severe ones are rare. The main symptoms are joint pain, muscle pain, repeated low fever, and a few have nephritis and changes in the blood system.

Systemic lupus erythematosus is the most severe type of lupus erythematosus. The majority of patients have multiple systemic damage at the time of onset, and a few patients develop from other types of lupus erythematosus. Some patients are also accompanied by other connective tissue diseases, such as scleroderma, dermatomyositis, Sjogren's syndrome, etc., forming various overlapping syndromes. Systemic lupus erythematosus has various clinical manifestations, is intricate and complicated, and is more serious. It can endanger patients' lives due to the side effects of lupus nephritis, lupus encephalopathy, and long-term heavy use of drugs.

Deep lupus erythematosus, also known as lupus panniculitis, is also an intermediate type of lupus erythematosus. Skin lesions are nodules or plaques, located in deep or subcutaneous fatty tissue of the dermis, of varying size and number, with a normal or pale red skin color, solid texture, and no mobility. Damage can occur anywhere, most commonly on the cheeks, hips, arms, followed by the calf and chest. After chronic, which can last for months to years, skin atrophy and depression remain after healing. Deep lupus erythematosus is unstable in nature and can exist alone. It can later be transformed into discoid lupus erythematosus, systemic lupus erythematosus, or both.

Neonatal lupus erythematosus, which is manifested as skin circular erythema and congenital heart block, is self-limiting. It usually resolves itself within 4 to 6 months after birth, and heart disease often persists.

Drug-induced lupus erythematosus is mainly manifested as fever, joint pain, muscle pain, facial butterfly erythema, oral ulcers, and serositis. ANA, anti-histone antibodies, anti-ss-DNA antibodies, etc. may be positive. Gradually improved after stopping the drug, those with more severe conditions can be given an appropriate amount of glucocorticoids.

Lupus erythematosus examination

Laboratory tests include routine blood, urine, and fecal examinations, immunological examinations, and pathological examinations of skin lesions.

Lupus erythematosus diagnosis

DLE is mainly based on the characteristics of rash and skin pathology. Those who have the conditions can do immunofluorescence test and autoantibody test to help confirm the diagnosis. The diagnosis of SLE is mainly based on a comprehensive history, clinical manifestations, and laboratory tests. At present, the SLE diagnostic standard revised by the American College of Rheumatology in 1982 is generally used. A patient can be diagnosed with 4 or more of the 11 criteria, either consecutively or simultaneously.

Lupus erythematosus treatment

General treatment

(1) Strong confidence should be built to fight disease.

(2) Avoid sunlight, especially those who are sensitive to sunlight. Should pay attention to sun protection when going out, avoid using photosensitivity drugs, such as phenothiazine, hydrochlorothiazide, sulfa drugs and griseofulvin.

(3) Avoid overwork. Acute or active SLE should stay in bed. To avoid pregnancy, it is not advisable to take contraceptives, and those with impaired renal function or multiple system impairment should have early treatment abortion.

(4) Avoid colds or other infections.

(5) Enhance resistance, pay attention to nutrition and vitamin supplement.

2. Treatment of skin lupus erythematosus

(1) Systemic treatment Antimalarial drugs, such as hydroxychloroquine, will be reduced to half when the condition improves. The general course of treatment is 2 to 3 years. Thalidomide can be tried. After the effect appears, the drug is reduced and maintained. The treatment is continued for 3 to 5 months. It is effective in most patients, but it is easy to relapse after stopping the drug. Oral cases can be treated with low-dose hormones orally.

(2) Local treatment Topical glucocorticoid ointment, 2 times a day, or packaged. Or intradermal injection of glucocorticoids.

3. Treatment of SLE

Personalization is very important. Before SLE patients start treatment, they must evaluate the disease activity of SLE patients, such as antinuclear antibodies, anti-DNA antibodies and hypocomplementemia and the degree of organ damage such as heart, kidney, lung disease, skin And serositis, and then make a correct evaluation before treatment. At present, the Systemic Lupus Erythematosus Activity Index (SLEDAI) is mostly used. Based on the clinical performance of patients before the evaluation at the time of evaluation 10, it is recommended that when SLEDAI> 12 points, the original double glucocorticoid dose or hospitalization is required. .

Research progress in lupus erythematosus

In June 2019, Tu's team proposed practical treatment solutions to the problem of "artemisinin resistance" and made new achievements in "artemisinin for indications such as lupus erythematosus" and "going out with traditional Chinese medicine research" Progress has been highly recognized by the World Health Organization and authoritative experts at home and abroad. ^[1]