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A stinging and burning sensation with rash is often felt and noticed, but itching is less common. Often the rash is steroid responsive, initially improving with application of topical steroid. The redness caused by perioral dermatitis has been associated with variable level of depression.
Initially, there may be small pinpoint papule either side of the nostrils. Multiple small (1-2mm) papules and pustules then occur around the mouth, nose and sometimes cheeks. The area of skin directly adjacent to the lips, also called the vermillion border, is spared and looks normal. There may be some mild background redness and occasional scale. These areas of skin are felt to be drier and therefore there is a tendency to moisturise them more frequently. Hence, they do not tolerate drying agents well and the rash can be worsened by them.
Perioral dermatitis is also known by other names including rosacea-like dermatoses, periorofacial dermatitis and periorificial dermatitis.
Unlike rosacea which involves mainly the nose and cheeks, there is no telangiectasia in perioral dermatitis. Rosacea also has a tendency to be present in older people. Acne can be distinguished by the presence of comedones and by its wider distribution on the face and chest. There are no comedones in personal dermatitis.
Other skin diseases which may resemble perioral dermatitis include:
- Rosacea
- Acne vulgaris
- Seborrheic dermatitis
- Allergic contact dermatitis
- Irritant contact dermatitis
Generally, the areas affected are exposed skin not usually protected by clothing; however it can also occur in areas covered by clothing. Areas constantly subjected to the sun's rays may only be slightly affected if at all. People with extreme cases will also have reactions to light bulbs that emit a UV wavelength (any bulb that is not an LED). Parts of the body only thinly covered can also potentially be subjected to an outbreak.
Life with SU can be difficult. Patients are subject to constant itching and pain, as within minutes of the initial exposure to UV radiation a rash will appear. The urticarial reaction begins in the form of pruritus, later progressing to erythema and edema in the exposed areas of the skin. If vast areas of the body are affected, the loss of fluid into the skin could lead to light-headedness, headache, nausea, and vomiting. Extremely rarely, patients have been reported to experience an increase in heart rate that can cause a stroke or heart attack due to the body cavity swelling. Other rare side effects can be bronchospasm and glucose instability issues. Also, if a large area of the body is suddenly exposed the person may be subject to an anaphylactic reaction. Once free of exposure, the rash will usually fade away within several hours; rare and extreme cases can take a day or two to normalize depending on severity of the reaction.
Urticarial dermatoses are distinct from urticaria, which examples being drug-induced urticaria, eosinophilic cellulitis and bullous pemphigoid. It is important to distinguish urticaria from urticarial dermatoses. The individual wheals of urticaria are ‘here today and gone tomorrow’ (i.e. they last less than 24 hours), whereas with urticarial dermatoses, the individual lesions last for days or longer.
Perioral dermatitis is a type of skin rash. Symptoms include multiple small (1–2 mm) bumps and blisters with sometimes background redness and scale, localized to the skin around the mouth and nostrils. Less commonly the eyes and genitalia may be involved. It can be persistent or recurring and resembles particularly rosacea and to some extent acne and allergic dermatitis. The term dermatitis is a misnomer because this is not an eczematous process.
The cause is unclear. Topical steroids are associated with the condition and moisturizers and cosmetics may contribute. The underlying mechanism may involve blockage of the skin surface followed by subsequent excessive growth of skin flora. Fluorinated toothpaste and some micro-organisms including candida may also worsen the condition, but their roles in this condition is unclear. It is considered a disease of the hair follicle with biopsy samples showing microscopic changes around the hair follicle. Diagnosis is based on symptoms.
Treatment is typically by stopping topical steroids, changing cosmetics, and in more severe cases, taking tetracyclines by mouth. Stopping steroids may initially worse the rash. The condition is estimated to affect 0.5-1% of people a year in the developed world. Up to 90% of those affected are women between the ages of 16 and 45 years, though it also affects children and the elderly, and has an increasing incidence in men.
This type of hives can occur right away, precisely after a pressure stimulus or as a deferred response to sustained pressure being enforced to the skin. In the deferred form, the hives only appear after about six hours from the initial application of pressure to the skin. Under normal circumstances, these hives are not the same as those witnessed with most urticariae. Instead, the protrusion in the affected areas is typically more spread out. The hives may last from eight hours to three days. The source of the pressure on the skin can happen from tight fitted clothing, belts, clothing with tough straps, walking, leaning against an object, standing, sitting on a hard surface, etc. The areas of the body most commonly affected are the hands, feet, trunk, abdomen, buttocks, legs and face. Although this appears to be very similar to dermatographism, the cardinal difference is that the swelled skin areas do not become visible quickly and tend to last much longer. This form of the skin disease is, however, rare.
Clinically, the earliest lesions may appear urticarial (like hives), but could also appear dermatitic, targetoid, lichenoid, nodular, or even without visible rash (essential pruritus). Tense bullae eventually erupt, most commonly at the inner thighs and upper arms, but the trunk and extremities are frequently both involved. Any part of the skin surface can be involved. Oral lesions are present in a minority of cases. The disease may be acute, but typically will wax and wane. Several other skin diseases may have similar symptoms. However, milia are more common with epidermolysis bullosa acquisita, because of the deeper antigenic targets. A more ring-like configuration, with a central depression or centrally collapsed bullae may indicate linear IgA disease. Nikolsky's sign is negative unlike pemphigus vulgaris where it is positive.
The symptoms of aquagenic urticaria or water allergy (being allergic to water) are similar to all the other types of physical hives that are caused by various things. This may include wheals or small raised hives, intense itching, skin flushing in the areas that are prone to water contact. The symptoms may appear within minutes after the body comes in contact with the water. Aquagenic urticaria is a rare condition in which itchy urticaria (hives) develop rapidly after the skin comes in contact with water, regardless of its temperature. The hives associated with aquagenic urticaria are typically small (approximately 1–3 mm), red- or skin-colored welts (called wheals) with clearly defined edges. The rash most commonly develops on the neck, upper trunk and arms, although it can occur anywhere on the body. Some people have itching too. Once the water source is removed, the rash generally fades within 30 to 60 minutes.
Dermatographic urticaria (also known as dermatographism or "skin writing") is marked by the appearance of weals or welts on the skin as a result of scratching or firm stroking of the skin. Seen in 4–5% of the population, it is one of the most common types of urticaria, in which the skin becomes raised and inflamed when stroked, scratched, rubbed, and sometimes even slapped.
The skin reaction usually becomes evident soon after the scratching, and disappears within 30 minutes. Dermatographism is the most common form of a subset of chronic hives, acknowledged as "physical hives".
It stands in contrast to the linear reddening that does not itch seen in healthy people who are scratched. In most cases, the cause is unknown, although it may be preceded by a viral infection, antibiotic therapy, or emotional upset. Dermographism is diagnosed by taking a tongue blade and drawing it over the skin of the arm or back. The hives should develop within a few minutes. Unless the skin is highly sensitive and reacts continually, treatment is not needed. Taking antihistamines can reduce the response in cases that are annoying to the patient.
Solar urticaria (SU) is a rare condition in which exposure to ultraviolet or UV radiation, or sometimes even visible light, induces a case of urticaria or hives that can appear in both covered and uncovered areas of the skin. It is classified as a type of physical urticaria. The classification of disease types is somewhat controversial. One classification system distinguished various types of SU based on the wavelength of the radiation that causes the breakout; another classification system is based on the type of allergen that initiates a breakout.
The agent in the human body responsible for the reaction to radiation, known as the photoallergen, has not yet been identified. The disease itself can be difficult to diagnose properly because it is so similar to other dermatological disorders, such as polymorphic light eruption or PMLE. The most helpful test is a diagnostic phototest, a specialized test which confirms the presence of an abnormal sunburn reaction. Once recognized, treatment of the disease commonly involves the administration of antihistamines, and desensitization treatments such as phototherapy. In more extreme cases, the use of immunosuppressive drugs and even plasmapheresis may be considered.
The initial discovery of the disease is credited to P. Merklen in 1904, but it did not have a name until the suggestion of "solar urticaria" was given by Duke in 1923. However, their research contributed to the study of this uncommon disease. More than one hundred cases have been reported in the past century.
The exact underlying cause of aquagenic urticaria is poorly understood.
Autoimmune estrogen dermatitis presents as a cyclic skin disorder, that may appear eczematous, papular, bullous, or urticarial. with pruritus typically present, skin eruptions that may be chronic but which are exacerbated premenstrually or occur immediately following menses.
Urticarial allergic eruption is a cutaneous condition characterized by annular or gyrate urticarial plaques that persist for greater than 24 hours.
Bullous pemphigoid is an acute or chronic autoimmune skin disease, involving the formation of blisters, more appropriately known as bullae, at the space between the epidermis and dermis skin layers. It is classified as a type II hypersensitivity reaction, with the formation of anti-hemidesmosome antibodies.
The most common type of eruption is a morbilliform (resembling measles) or erythematous rash (approximately 90% of cases). Less commonly, the appearance may also be urticarial, papulosquamous, pustular, purpuric, bullous (with blisters) or lichenoid. Angioedema can also be drug-induced (most notably, by angiotensin converting enzyme inhibitors).
Some of the most severe and life-threatening examples of drug eruptions are erythema multiforme, Stevens–Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), hypersensitivity vasculitis, Drug induced hypersensitivity syndrome (DIHS), erythroderma and acute generalized exanthematous pustulosis (AGEP). These severe cutaneous drug eruptions are categorized as hypersensitivity reactions and are immune-mediated. There are four types of hypersensitivity reactions and many drugs can induce one or more hypersensitivity reactions.
Acneiform eruptions are a group of dermatoses including acne vulgaris, rosacea, folliculitis, and perioral dermatitis. Restated, acneiform eruptions are follicular eruptions characterized by papules and pustules resembling acne.
The hybrid term "acneiform", literally, refers to an appearance similar to acne.
The terminology used in this field can be complex, and occasionally contradictory. Some sources consider acne vulgaris part of the differential diagnosis for an acneiform eruption. Other sources classified acne vulgaris under acneiform eruption. MeSH explicitly excludes perioral dermatitis from the category of "acneiform eruptions", though it does group acneiform eruptions and perioral dermatitis together under "facial dermatoses".
Diagnosis of PG becomes clear when skin lesions progress to tense blisters during the second or third trimester. The face and mucous membranes are usually spared. PG typically starts as a blistering rash in the navel area and then spreads over the entire body. It is sometimes accompanied by raised, hot, painful welts called plaques. After one to two weeks, large, tense blisters typically develop on the red plaques, containing clear or blood-stained fluid. PG creates a histamine response that causes extreme relentless itching (pruritus). PG is characterized by flaring and remission during the gestational and sometimes "post partum" period. Usually after delivery, lesions will heal within months, but may reoccur during menstruation.
Urticarial erythema multiforme is an unusual reaction virtually always associated with antibiotic ingestions, characterized by skin lesions that consist of urticarial papules and plaques, some of which clear centrally forming annular lesions, but with no true urticarial lesions.
Papular mucinosis is chronic and may be progressive. The dermal layer of the skin breaks out into small and solid bumps, usually conical in shape and measured from 2 to 4 mm or sometimes flat-topped papules. Unlike pustules, these bumps do not contain pus. Instead they contain mucin, a substance of mucus, the body's natural and protective lubricant found in saliva and epithelial cells in lungs and the sensitive part of the nose. They usually come in clusters such as linear arrays. Less frequently, urticarial, nodular, or sometimes annular lesions may be appreciated. The dorsal aspect of the hands, face, elbows, and extensor portions of the extremities are most frequently affected. Mucosal lesions are absent. The coalescence of papules on the face, particularly on the glabella, results in longitudinal folding and gives the appearance of a leonine facies.
In scleromyxedema, symptoms can occur on larger part of the body. Erythema and scleroderma-like induration occurs on the skin. In addition, the mobility of the lips, hands, arms, and legs is reduced. Proximal myopathy, inflammatory polyarthritis, central nervous system symptoms, esophageal aperistalsis, and hoarseness are among the notable systemic symptoms. If viscera is involved, the disease will be fatal. The dermatoneuro syndrome is a rare neurological complication of the disease presenting with fever, seizures and altered mental status.
Autoimmune progesterone dermatitis may appear as urticarial papules, deep gyrate lesions, papulovesicular lesions, an eczematous eruption, or as targetoid lesions. Autoimmune progesterone dermatitis initially manifests with eye symptoms, e.g. burning, and progresses into rashes. Its relapsing-remitting pattern in women correspond to the progesterone levels during the menstrual cycle, which spike twice a month. It is an extremely rare disease, only perhaps 50 cases have ever been reported.
PVA can be characterized by speckled, combined hyper- and hypopigmentation in the plaques or patches of affected skin. Hyperpigmentation is excess coloration, or darkening of the skin, while hypopigmentation is a diminished or pallid coloring to the skin. Pigmentation changes in PVA, apparent in the epidermal (outermost) skin layer, may be attributed to incontinence (leaking out) of melanin from melanocytes into the dermal skin layer below. Inflammation of the skin and cutaneous tissue, common with PVA, also contributes to color changes in the skin, typified by redness. Telangiectasia, the visible "vascular" element of PVA, is the of small blood vessels near the skin surface. Skin atrophy, a wasting-away of the tissue comprising the skin, is a prominent part of PVA and effects the dermal, and particularly the epidermal layer. This, in part, is the result of degenerative of the stratum basale (bottom cell-layer) of the epidermis. Atrophy of the skin gives it a thin, dry and wrinkled appearance, which in PVA-affected individuals has been described as "cigarette paper". Hyperkeratosis, a thickening of the stratum corneum (top cell-layer of the epidermis), has also been reported.
Grover's disease often starts quite suddenly. There are intensely itchy spots on the central back, mid chest and occasionally elsewhere. Frequently, it follows sweating or some unexpected heat stress.
The itchy eruption lasts an average of 10–12 months. It is characterized by papules and papulovesicles with excoriations occurring on the chest, back, lower sternum, arms, and thighs. The papules are most commonly found on the mid chest.
Sometimes the features of Grover's are found in people who do not itch or have a conspicuous rash. Most of the people with Grover's who visit a dermatologist, however, itch a lot.
The reaction to a sting is of three types. The normal reaction involves the area around the bite with redness, itchiness, and pain. A large local reaction occurs when the area of swelling is greater than 5 cm. Systemic reactions are when symptoms occur in areas besides that of the bites.
With insect stings a large local reaction may occur (an area of skin redness greater than 10 cm in size). It can last one to two days. It occurs in about 10% of those bitten.
Although a clear understanding of the various skin lesions in IgG4-related disease is a work in progress, skin lesions have been classified into subtypes based on documented cases:
- Angiolymphoid hyperplasia with eosinophilia (or lesions that mimic it) and cutaneous pseudolymphoma
- Cutaneous plasmacytosis
- Eyelid swelling (as part of Mikulicz's disease)
- Psoriasis-like eruptions
- Unspecified maculopapular or erythematous eruptions
- Hypergammaglobulinemic purpura and urticarial vasculitis
- Impaired blood supply to fingers or toes, leading to Raynaud's phenomenon or gangrene
Note:
In addition, Wells syndrome has also been reported in a case of IgG4-related disease.
PG often is confused with pruritic urticarial papules and plaques of pregnancy (PUPPP), especially if it occurs in a first pregnancy. PUPPP typically begins in stretch mark areas of the abdomen and usually ends within two weeks after delivery. PUPPP is not an autoimmune disease.
Diagnosing PG is done by biopsy using direct immunofluorescence, appearance, and blood studies.