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Tumid lupus erythematosus (also known as "lupus erythematosus tumidus") is a rare, but distinctive entity in which patients present with edematous erythematous plaques, usually on the trunk.
Lupus erythematosus tumidus (LET) was reported by Henri Gougerot and Burnier R. in 1930. It is a photosensitive skin disorder, a different subtype of cutaneous lupus erythematosus (CLE) from discoid lupus erythematosus (DLE) or subacute CLE (SCLE). LET is usually found on sun-exposed areas of the body. Skin lesions are edematous, urticarialike annular papules and plaques. Topical corticosteroids are not effective as treatment for LET, but many will respond to chloroquine. LET resolves with normal skin, no residual scarring, no hyperpigmentation or hypopigmentation. Cigarette smokers who have LET may not respond very well to chloroquine.
It has been suggested that it is equivalent to Jessner lymphocytic infiltrate of the skin.
Localised discoid lupus erythematosus typically presents with skin lesions localised above the neck, with favoured sites being the scalp, bridge of the nose, cheeks, above the lips and ears as well as the arms hair scratching glasses touching name="Andrews1"/> Another form of discoid lupus erythematosus includes oral discoid. Oral discoid lupus erythematosus results in oral lesions which present themselves as white spots, ulcers and central erythema lesions. Oral discoid lesions most commonly occur on the labial mucosa, vermillion border and buccal mucosa. Atrophy may be observed in some cases. Dentists may be important in establishing the diagnosis before the cutaneous lesions become apparent.
Discoid lupus erythematosus (DLE) is a chronic skin condition of sores with inflammation and scarring favouring the face, ears, and scalp and at times on other body areas. These lesions develop as a red, inflamed patch with a scaling and crusty appearance. The centre areas may appear lighter in colour with a rim darker than the normal skin.
Discoid lupus erythematosus can be divided into localised, generalised, and childhood discoid lupus erythematosus.
Verrucous lupus erythematosus (also known as "Hypertrophic lupus erythematosus") presents with non-pruritic papulonodular lesions on the arms and hands, resembling keratoacanthoma or hypertropic lichen planus.
Erythema nodosum is a form of panniculitis characterised by tender red nodules, 1–10 cm, associated with systemic symptoms including fever, malaise, and joint pain. Nodules may become bluish-purple, yellowing, and green, and subside over a period of 2–6 weeks without ulcerating or scarring. Erythema nodosum is associated with infections, including Hepatitis C, EBV and tuberculosis, Crohn's disease and sarcoidosis, pregnancy, medications including sulfonamides, and some cancers, including Non-Hodgkin lymphoma and pancreatic cancer.
Chilblain lupus erythematosus (also known as "chilblain lupus erythematosus of Hutchinson") is a chronic, unremitting form of lupus erythematosus with the fingertips, rims of ears, calves, and heels affected, especially in women.
Acute cutaneous lupus erythematosus is a cutaneous condition characterized by a bilateral malar rash (also known as a "butterfly rash") and lesions that tend to be transient, and that follow sun exposure.
Lupus erythematosus panniculitis (also known as "Lupus erythematosus profundus", "Lupus panniculitis", "Lupus profundus", and "Subcutaneous lupus erythematosus") presents with subcutaneous nodules that are commonly firm, sharply defined and nontender.
The condition should be distinguished from:
- Basal cell carcinoma
- Sarcoidosis
- Discoid lupus erythomatosus
- Leprosy
- Deep fungal infection
Tufted folliculitis presents with doll's hair-like bundling of follicular units, and is seen in a wide range of scarring conditions including chronic staphylococcal infection, chronic lupus erythematosus, lichen planopilaris, Graham-Little syndrome, folliculitis decalvans, acne keloidalis nuchae, immunobullous disorders, and dissecting cellulitis.
It begins as painless reddish-brown nodules which slowly enlarge to form irregularly shaped red plaque.
Symptoms vary from person to person, and may come and go. Almost everyone with lupus has joint pain and swelling. Some develop arthritis. Frequently affected joints are the fingers, hands, wrists, and knees.
Other common symptoms include:
- chest pain during respiration
- joint pain
- oral ulcer
- fatigue
- fever with no other cause
- general discomfort, uneasiness, or ill feeling (malaise)
- hair loss
- sensitivity to sunlight
- skin rash – a "butterfly" rash in about half people with SLE
- swollen lymph nodes
Alpha-1 antitrypsin deficiency panniculitis is a panniculitis associated with a deficiency of the α-antitrypsin enzyme.
Pemphigus erythematosus (also known as "Senear–Usher syndrome") is simply a localized form of pemphigus foliaceus with features of lupus erythematosus.
Disease presentation varies widely from patient to patient, as UCTD is by definition nonspecific. Symptoms typically include constitutional complaints that are common to connective tissue diseases such as fatigue, a general sense of feeling unwell, and fever.
Other symptoms associated with UCTD include:
- dry eyes
- dry mouth
- hair loss
- joint inflammation
- joint pain
- oral ulcers
- positive ANA test
- raynaud's phenomenon
- sun sensitive rash
Lung involvement, such as nonspecific interstitial pneumonia, is a possible disease complication.
The disease mimics many other dermatoses and can be confused with conditions, such as sarcoidosis, discoid lupus erythematosus, mycosis fungoides, and fixed drug eruption.
As many as 70% of people with lupus have some skin symptoms. The three main categories of lesions are chronic cutaneous (discoid) lupus, subacute cutaneous lupus, and acute cutaneous lupus. People with discoid lupus may exhibit thick, red scaly patches on the skin. Similarly, subacute cutaneous lupus manifests as red, scaly patches of skin but with distinct edges. Acute cutaneous lupus manifests as a rash. Some have the classic malar rash (or "butterfly rash") associated with the disease. This rash occurs in 30 to 60% of people with SLE.
Hair loss, mouth and nasal ulcers, and lesions on the skin are other possible manifestations.
The most commonly sought medical attention is for joint pain, with the small joints of the hand and wrist usually affected, although all joints are at risk. More than 90 percent of those affected will experience joint and/or muscle pain at some time during the course of their illness. Unlike rheumatoid arthritis, lupus arthritis is less disabling and usually does not cause severe destruction of the joints. Fewer than ten percent of people with lupus arthritis will develop deformities of the hands and feet. People with SLE are at particular risk of developing osteoarticular tuberculosis.
A possible association between rheumatoid arthritis and SLE has been suggested, and SLE may be associated with an increased risk of bone fractures in relatively young women.
Because the TVC's entry point usually is the site of a trauma, wound or puncture in the skin (during an autopsy, for example), the most frequent site for the wart are the hands. But it can occur anywhere in the skin, such as in the sole of the feet, in the anus, and, in the case of children from developing countries, in the buttocks and knees. This is because children from countries of high incidence of tuberculosis can contract the lesion after contact with tuberculous sputum, by walking barefoot, sitting or playing on the ground.
When recent, the skin lesion has the outside appearance of a wart or verruca, thus it can be confused with other kinds of warts. It evolves to an annular red-brown plaque with time, with central healing and gradual expansion in the periphery. In this phase, it can be confused with fungal infections such as blastomycosis and chromoblastomycosis.
Subacute cutaneous lupus erythematosus (SCLE) is a clinically distinct subset of cases of lupus erythematosus that is most often present in white women aged 15 to 40, consisting of skin lesions that are scaly and evolve as polycyclic annular lesions or plaques similar to those of plaque psoriasis.
Characteristically the lesions appear in sun-exposed areas such as the vee of the neckline or the forearms, but not the face. It may be brought on by sun-sensitizing medications, but is usually associated with autoimmune disorders such as rheumatoid arthritis and Sjögren's syndrome.
Treatment generally involves sun avoidance and protection and topical corticosteroids. Sometimes systemic drug treatment is necessary. Besides corticosteroids other immunosuppressants such as methotrexate are also used.
Lesions of SCLE may have an annular configuration, with raised red borders and central clearing.
Lupus erythematosus is a name given to a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. The most common and severe form is systemic lupus erythematosus.
Neonatal lupus erythematosus is the occurrence of systemic lupus erythematosus (SLE) symptoms in an infant born from a mother with SLE, most commonly presenting with a rash resembling discoid lupus erythematosus, and sometimes with systemic abnormalities such as complete heart block or hepatosplenomegaly.
The infants have no skin lesions at birth, but develop them during the first weeks of life. Neonatal lupus is usually benign and self-limited.
It is associated with mothers who carry the Ro/SSA antibodies.
Collagen disease is a term previously used to describe systemic autoimmune diseases (e.g., rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis), but now is thought to be more appropriate for diseases associated with defects in collagen, which is a component of the connective tissue.
The term "collagen disease" was coined by Dr. Alvin F. Coburn in 1932, on his quest to discover streptococcal infection as the cause for rheumatic fever.
Undifferentiated connective tissue disease (UCTD) is a disease in which the body mistakenly attacks its own tissues. It is diagnosed when there is evidence of an existing autoimmune condition which does not meet the criteria for any specific autoimmune disease, such as systemic lupus erythematosus or scleroderma. Latent lupus and incomplete lupus are alternative terms that have been used to describe this condition.
The term is sometimes used interchangeably with mixed connective tissue disease, an overlap syndrome. However, MCTD is thought by some researchers to be a clinically distinct entity and is strongly associated with the presence of high titers of ribonucleoprotein (RNP) antibodies.
It is estimated that up to 25 percent of people with systemic autoimmune disease could be considered to have UCTD.
Granuloma faciale (GF) is an uncommon benign chronic skin disease of unknown origin characterized by single or multiple cutaneous nodules, usually occurring over the face. Occasionally, extrafacial involvement is noted, most often on sun-exposed areas.