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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
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Initially red to pink, flat spots (formally, "macules") and raised bumps (formally, "papules") may be seen on the skin.
Once fully developed, the classic appearance is "non-blanching, palpable purpura". This appears as deep red to purple spots that feel raised to the touch. Purpura refers to the red-purple discolored spots, while palpable implies that these spots can be felt as raised from the surrounding skin. Additionally, when gently pressed, the color does not fade to a lighter color ("non-blanching"). The red-purple color of the lesions is due to the inflammation in the blood vessels causing red blood cells to escape into the dermis skin layer.
Small fluid-filled blisters (or "vesicles"), pus-filled bumps resembling a pimple (or "pustules"), or shallow ulcers may also develop but are less common.
The location of skin lesions varies but are most commonly found symmetrically below the waist, primarily on the buttocks and legs. Other distributions include localized areas on the upper body or over several areas of the body.
With treatment, the lesions typically resolve in weeks to months and leave behind flat spots that are darker than the surrounding skin. (see "Postinflammatory hyperpigmentation" on "Hyperpigmentation")
A portion of cases may be persistent or recurrent. This tends to occur when the vasculitis is associated with chronic conditions such as connective tissue diseases.
In most cases skin lesions do not cause symptoms, however itching, burning, or pain may occur.
Frequently reported symptoms include mild fever, muscle pain, joint pain, or an overall feeling of discomfort. Additional symptoms depend on the cause of the vasculitis and if other organ systems are involved. For example, if the vasculitis is a manifestation of Henoch-Schönlein purpura, individuals may also experience abdominal pain or blood in the urine.
The condition varies from a mild, self-limited rash (E. multiforme minor) to a severe, life-threatening form known as erythema multiformer major (or erythema multiforme majus) that also involves mucous membranes.
Consensus classification:
- Erythema multiforme minor—typical targets or raised, edematous papules distributed
- Erythema multiforme major—typical targets or raised, edematous papules distributed acrally with involvement of one or more mucous membranes; epidermal detachment involves less than 10% of total body surface area (TBSiA)
- SJS/TEN—widespread blisters predominant on the trunk and face, presenting with erythematous or pruritic macules and one or more mucous membrane erosions; epidermal detachment is less than 10% TBSA for Stevens-Johnson syndrome and 30% or more for toxic epidermal necrolysis.
The mild form usually presents with mildly itchy (but itching can be very severe), pink-red blotches, symmetrically arranged and starting on the extremities. It often takes on the classical "target lesion" appearance, with a pink-red ring around a pale center. Resolution within 7–10 days is the norm.
Individuals with persistent (chronic) erythema multiforme will often have a lesion form at an injury site, e.g. a minor scratch or abrasion, within a week. Irritation or even pressure from clothing will cause the erythema sore to continue to expand along its margins for weeks or months, long after the original sore at the center heals.
Erythema induratum, or "Bazin disease", is a panniculitis on the back of the calves. It was formerly thought to be a reaction to the tuberculum bacillus. It is now considered a panniculitis that is not associated with a single defined pathogen.
Nodular vasculitis is a skin condition characterized by small, tender, reddened nodules on the legs, mostly on the calves and shins. Microscopically there are epithelioid granulomas and vasculitis in the subcutaneous tissue, making it a form of panicullitis. Most of these cases are now thought to be manifestation of tuberculosis and indeed they respond well to anti-tuberculous treatment.
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.
Symptoms include severe seborrheic dermatitis of the scalp, severe diarrhea, recurrent local and systemic infection, central nervous system problems, and failure to thrive. Other symptoms also include scaling on the trunk and limbs, red patches of skin on parts of the body that bend, fevers, reduced blood protein levels, thick red skin patches, peeling of the skin, itching, corneal ulcers. wasting of the lymph nodes, underdeveloped lymphatics, anemia, wasting, and nervous system deficiency. The disease may then spread to the rest of the epidermis with the appearance of crusty, dry, moist or greasy scaling on the scalp. Scaling could also appear behind the ears, nose or eyebrows, or around the mouth; peeling of the skin may also happen in these areas. If left untreated, the skin infections will cause loss of protein or electrolytes. Leiner’s Disease may also be accompanied by a systemic reaction that is most evident in its gastrointestinal manifestation.
It is caused by a deficit of the complement protein, C5; however, case reports have described it in relation to deficits in either C3 or C4.
Erythema multiforme (EM) is a skin condition of unknown cause; it is a type of erythema possibly mediated by deposition of immune complexes (mostly IgM-bound complexes) in the superficial microvasculature of the skin and oral mucous membrane that usually follows an infection or drug exposure. It is an uncommon disorder, with peak incidence in the second and third decades of life. The disorder has various forms or presentations, which its name reflects ("multiforme", "", from "" + ""). Target lesions are a typical manifestation. Two types, one mild to moderate and one severe, are recognized (erythema multiforme minor and erythema multiforme major).
Leiner's disease is a systemic disease, a skin disorder and extends to erythroderma, typically diagnosed in early infancy. Leiner’s disease is characterized by a long-lasting seborrhea dermatitis associated with increased likelihood to infection. Other characterizations found on newborns with Leiner’s disease are a patch or a large patch of red skin normally on the bottom and spreads to the rest of the body.
This disease is also listed as a "rare disease", meaning that a small percent of the population, fewer than 200,000 people in the United States, will have this disorder.
Cats with eosinophilic granuloma complex (EGC) may have one or more of four patterns of skin disease.
The most frequent form is "eosinophilic plaque". This is a rash comprising raised red to salmon-colored and flat-topped, moist bumps scattered on the skin surface. The most common location is on the ventral abdomen and inner thigh.
Another form of EGC is the "lip ulcer". This is a painless, shallow ulcer with raised and thickened edges that forms on the upper lip adjacent to the upper canine tooth. It is often found on both sides of the upper lips.
The third form of the EGC is the "collagenolytic granuloma". This is a firm swelling that may be ulcerated. The lesions may form on the skin, especially of the face, in the mouth, or on the feet, or may form linear flat-topped raised hairless lesions on the back of the hind legs, also called "linear granuloma".
The least common form of EGC is "atypical eosinophilic dermatitis". It is unique in that it is caused by mosquito bite allergy and the lesions form on the parts of the body with the least hair affording easy access to feeding mosquitoes. This includes the bridge of the nose, the outer tips of the ears and the skin around the pads of the feet. The lesions are red bumps, shallow ulcers and crusts.
In humans, eosinophilic granulomas are considered a benign histiocytosis that occurs mainly in adolescents and young adults. Clinically, unifocal lytic lesions are found in bones such as the skull, ribs and femur. Because of this, bone pain and pathologic fractures are common.
Livedo reticularis is a common skin finding consisting of a mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin. The discoloration is caused by swelling of the venules owing to obstruction of capillaries by small blood clots. The blood clots in the small blood vessels can be a secondary effect of a condition that increases a person's risk of forming blood clots, including a wide array of pathological and nonpathological conditions . Examples include hyperlipidemia, microvascular hematological or anemia states, nutritional deficiencies, hyper- and autoimmune diseases, and drugs/toxins.
The condition may be normal or related to more severe underlying pathology. Its differential diagnosis is broadly divided into possible blood diseases, autoimmune (rheumatologic) diseases, cardiovascular diseases, cancers, and endocrine disorders. It can usually (in 80% of cases) be diagnosed by biopsy.
It may be aggravated by exposure to cold, and occurs most often in the lower extremities.
The condition's name derives from the Latin "livere" meaning bluish and "reticular" which refers to the net-like appearance.
In the skin, systemic sclerosis causes hardening and scarring. The skin may appear tight, reddish, or scaly. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage may weaken limbs and affect appearance. Patients report severe and recurrent itching of large skin areas. The severity of these symptoms varies greatly among patients: Some having scleroderma of only a limited area of the skin (such as the fingers) and little involvement of the underlying tissue, while others have progressive skin involvement. Digital ulcers — open wounds on especially on fingertips and less commonly the knuckles — are not uncommon.
The areas most affected are the toes, fingers, earlobes, nose.
- Blistering of affected area
- Burning and itching sensation in extremities
- Dermatitis in extremities
- Digital ulceration (severe cases only)
- Erythema (blanchable redness of the skin)
- Pain in affected area
- Skin discoloration, red to dark blue
Chilblains usually heal within 7–14 days.
Nodular vasculitis is a skin condition characterized by crops of small, tender, erythematous nodules on the legs, mostly on the calves and shins. Miroscopically there are epithelioid granulomas and vasculitis in the subcutaneous tissue, making it a form of panicullitis. Most of these cases are now thought to be manifestation of tuberculosis and indeed they respond well to anti-tuberculous treatment.
CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) is associated with limited scleroderma. Other symptoms include:
Ichthyosis linearis circumflexa is a distinctive skin condition of generalized hyperkeratosis and polycyclic and serpiginous erythematous plaques with a characteristic, migratory, double-edged scale at the margins, and is the typical cutaneous manifestation of Netherton's syndrome.
A maculopapular rash is a type of rash characterized by a flat, red area on the skin that is covered with small confluent bumps. It may only appear red in lighter-skinned people. The term "maculopapular" is a compound: "macules" are small, flat discolored spots on the surface of the skin; and "papules" are small, raised bumps. It is also described as erythematous, or red.
This type of rash is common in several diseases and medical conditions, including scarlet fever, measles, Ebola virus disease, rubella, secondary syphilis (Congenital syphilis, which is asymptomatic, the newborn may present this type of rash), erythrovirus (parvovirus B19), chikungunya (alphavirus), zika, and heat rash. It is also a common manifestation of a skin reaction to the antibiotic amoxicillin or chemotherapy drugs. Cutaneous infiltration of leukemic cells may also have this appearance. Maculopapular rash is seen in graft-versus-host disease (GVHD) developed after a hematopoietic stem cell transplant (bone marrow transplant), which can be seen within one week or several weeks after the transplant. In the case of GVHD, the maculopapular rash may progress to a condition similar to toxic epidermal necrolysis. In addition, this is the type of rash that some patients presenting with Ebola virus hemorrhagic (EBO-Z) fever will reveal but can be hard to see on dark skin people. It is also seen in patients with Marburg hemorrhagic fever, a filovirus not unlike Ebola.
This type of rash can be as a result of large doses of niacin or no-flush niacin (2000 – 2500 mg), used for the management of low HDL cholesterol.
This type of rash can also be a symptom of Sea bather's eruption. This stinging, pruritic, maculopapular rash affects swimmers in some Atlantic locales (e.g., Florida, Caribbean, Long Island). It is caused by hypersensitivity to stings from the larvae of the sea anemone (e.g., "Edwardsiella lineate") or the thimble jellyfish ("Linuche unguiculata"). The rash appears where the bathing suit contacts the skin.
This type of rash can also be a symptom of acute arsenic intoxication, appearing 2 weeks later.
A number of conditions may cause the appearance of livedo reticularis:
- Cutis marmorata telangiectatica congenita, a rare congenital condition
- Sneddon syndrome – association of livedoid vasculitis and systemic vascular disorders, such as strokes, due to underlying genetic cause
- Idiopathic livedo reticularis – the most common form of livedo reticularis, completely benign condition of unknown cause affecting mostly young women during the winter: It is a lacy purple appearance of skin in extremities due to sluggish venous blood flow. It may be mild, but ulceration may occur later in the summer.
- Secondary livedo reticularis:
- Vasculitis autoimmune conditions:
- Livedoid vasculitis – with painful ulceration occurring in the lower legs
- Polyarteritis nodosa
- Systemic lupus erythematosus
- Dermatomyositis
- Rheumatoid arthritis
- Lymphoma
- Pancreatitis
- Chronic pancreatitis
- Tuberculosis
- Drug-related:
- Adderall (side effect)
- Amantadine (side effect)
- Bromocriptine (side effect)
- Beta IFN treatment, "i.e." in multiple sclerosis
- Livedo reticularis associated with rasagiline
- Methylphenidate and dextroamphetamine-induced peripheral vasculopathy
- Gefitinib
- Obstruction of capillaries:
- Cryoglobulinaemia – proteins in the blood that clump together in cold conditions
- Antiphospholipid syndrome due to small blood clots
- Hypercalcaemia (raised blood calcium levels which may be deposited in the capillaries)
- Haematological disorders of polycythaemia rubra vera or thrombocytosis (excessive red cells or platelets)
- Infections (syphilis, tuberculosis, Lyme disease)
- Associated with acute renal failure due to cholesterol emboli status after cardiac catheterization
- Arteriosclerosis (cholesterol emboli) and homocystinuria (due to Chromosome 21 autosomal recessive Cystathionine beta synthase deficiency)
- Intra-arterial injection (especially in drug addicts)
- Ehlers-Danlos syndrome – connective tissue disorder, often with many secondary conditions, may be present in all types
- Pheochromocytoma
- Livedoid vasculopathy and its association with factor V Leiden mutation
- FILS syndrome (polymerase ε1 mutation in a human syndrome with facial dysmorphism, immunodeficiency, livedo, and short stature)
- Primary hyperoxaluria, oxalosis (oxalate vasculopathy)
- Cytomegalovirus infection (very rare clinical form, presenting with persistent fever and livedo reticularis on the extremities and cutaneous necrotizing vasculitis of the toes)
- Generalized livedo reticularis induced by silicone implants for soft tissue augmentation
- As a rare skin finding in children with Down syndrome
- Idiopathic livedo reticularis with polyclonal IgM hypergammopathy
- CO angiography (rare, reported case)
- A less common skin lesion of Churg-Strauss syndrome
- Erythema nodosum-like cutaneous lesions of sarcoidosis showing livedoid changes in a patient with sarcoidosis and Sjögren's syndrome
- Livedo vasculopathy associated with IgM antiphosphatidylserine-prothrombin complex antibody
- Livedo vasculopathy associated with plasminogen activator inhibitor-1 promoter homozygosity and prothrombin G20210A heterozygosity
- As a first sign of metastatic breast carcinoma (very rare)
- Livedo reticularis associated with renal cell carcinoma (rare)
- Buerger's disease (as an initial symptom)
- As a rare manifestation of Graves hyperthyroidism
- Associated with pernicious anaemia
- Moyamoya disease (a rare, chronic cerebrovascular occlusive disease of unknown cause, characterized by progressive stenosis of the arteries of the circle of Willis leading to an abnormal capillary network and resultant ischemic strokes or cerebral hemorrhages)
- Associated with the use of a midline catheter
- Familial primary cryofibrinogenemia.
Urticaria pigmentosa is characterized by excessive amounts of mast cells in the skin. Red or brown spots are often seen on the skin, typically around the chest and forehead. These mast cells, when irritated (e.g. by rubbing the skin, heat exposure), produce too much histamine, triggering an allergic reaction that leads to hives localized to the area of irritation, sometimes referred to as Darier's sign. Severe itching usually follows, and scratching the area only serves to further symptoms. Symptoms can be mild (flushing and hives that require no treatment), moderate (diarrhea, tachycardia, nausea/vomiting, headache, and fainting), or life-threatening (vascular collapse requiring emergency treatment and hospitalization).
Chilblains — also known as pernio, chill burns and perniosis — is a medical condition that occurs when a predisposed individual is exposed to cold and humidity, causing tissue damage. It is often confused with frostbite and trench foot. Damage to capillary beds in the skin causes redness, itching, inflammation, and sometimes blisters. Chilblains can be reduced by keeping the feet and hands warm in cold weather, and avoiding extreme temperature changes. Chilblains can be idiopathic (spontaneous and unrelated to another disease), but may also be a manifestation of another serious medical condition that must be investigated. A history of chilblains suggests a connective tissue disease (such as lupus). In infants, chilblains together with severe neurologic disease and unexplained fevers occurs in Aicardi–Goutières syndrome, a rare inherited condition.
Urticaria pigmentosa (also known as generalized eruption of cutaneous mastocytosis (childhood type)
) is the most common form of cutaneous mastocytosis. It is a rare disease caused by excessive numbers of mast cells in the skin that produce hives or lesions on the skin when irritated.
Raynaud's phenomenon is frequently the first manifestation of CREST/lcSSc, preceding other symptoms by years. Stress and cold temperature induce an exaggerated vasoconstriction of the small arteries, arterioles, and thermoregulatory vessels of the skin of the digits. Clinically this manifests as a white-blue-red transitions in skin color. Underlying this transition is pallor and cyanosis of the digits, followed by a reactive hyperemia as they rewarm. When extreme and frequent, this phenomenon can lead to digital ulcerations, gangrene, or amputation.
Ulceration can predispose to chronic infections of the involved site.
CREST causes thickening and tightening of the skin with deposition of calcific nodules ("calcinosis").
The erythema migrans rash is classically 5 to 6.8 cm in diameter, appearing as an annular homogenous erythema (59%), central erythema (30%), central clearing (9%), or central purpura (2%). Because of the "bull's-eye" description to describe the Lyme disease rash, the condition commonly called ringworm is sometimes confused with Lyme disease. Uncommonly, EM may be less than 5 cm in diameter. Multiple painless EM rashes may occur, indicating disseminated infection.
The EM rash occurs in 80% to 90% of those infected with "Borrelia". A systematic review of the medical literature showed 80% of patients have an expanding EM rash, at the site of the tick bite, although some patients with EM do not recall a tick bite. In endemic areas of the United States, homogeneously red rashes are more frequent.
Advocates of a diagnosis called "chronic lyme disease" dispute the generally accepted incidence of the rash, claiming it occurs in less than 50% of infections.
The development of ichthyosis in adulthood can be a manifestation of systemic disease, and it has been described in association with malignancies, drugs, endocrine and metabolic disease, HIV, infection, and autoimmune conditions.
It usually is associated with people who have Hodgkin's disease but it is also occurs in people with mycosis fungoides, other malignant sarcomas, Kaposi's sarcoma and visceral carcinomas. It can occur in people suffering from leprosy, AIDS, tuberculosis, and typhoid fever.