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Characteristics may vary according to the subtype of pustular psoriasis. For example, it can be localized, commonly to the hands and feet (localized pustular psoriasis), or generalized with widespread patches occurring randomly on any part of the body (generalized pustular psoriasis). However, all forms of pustular psoriasis share in common the presence of red and tender blotchy skin covered with pustules.
Pustular psoriasis can be localized, commonly to the hands and feet (palmoplantar pustulosis), or generalized with widespread patches occurring randomly on any part of the body. Acrodermatitis continua is a form of localized psoriasis limited to the fingers and toes that may spread to the hands and feet. Pustulosis palmaris et plantaris is another form of localized pustular psoriasis similar to acrodermatitis continua with pustules erupting from red, tender, scaly skin found on the palms of the hands and the soles of the feet.
Generalized pustular psoriasis (GPP) is also known as (von Zumbusch) acute generalized pustular psoriasis in acute cases, and as impetigo herpetiformis during pregnancy. GPP is a rare and severe form of psoriasis that may require hospitalization. This form of psoriasis is characterized by an acute onset of numerous pustules on top of tender red skin. This skin eruption is often accompanied by a fever, muscle aches, nausea, and an elevated white blood cell count. Annular pustular psoriasis (APP), a rare form of GPP, is the most common type seen during childhood. APP tends to occur in women more frequently than in men, and is usually less severe than other forms of generalized pustular psoriasis such as impetigo herpetiformis. This form of psoriasis is characterized by ring-shaped plaques with pustules around the edges and yellow crusting. APP most often affects the torso, neck, arms, and legs.
Discoid eczema (nummular eczema, exudative eczema, microbial eczema) is characterized by round spots of oozing or dry rash, with clear boundaries, often on lower legs. It is usually worse in winter. Cause is unknown, and the condition tends to come and go. (ICD-10 L30.0)
Pemphigus vulgaris most commonly presents with oral blisters (buccal and palatine mucosa, especially), but also includes cutaneous blisters. Other mucosal surfaces, the conjunctiva, nose, esophagus, penis, vulva, vagina, cervix, and anus, may also be affected. Flaccid blisters over the skin are frequently seen with sparing of the skin covering the palms and soles.
Blisters commonly erode and leave ulcerated lesions and erosions. A positive Nikolsky sign (induction of blistering in normal skin or at the edge of a blister) is indicative of the disease.
Severe pain with chewing can lead to weight loss and malnutrition.
Dyshidrosis (dyshidrotic eczema, pompholyx, vesicular palmoplantar dermatitis) only occurs on palms, soles, and sides of fingers and toes. Tiny opaque bumps called vesicles, thickening, and cracks are accompanied by itching, which gets worse at night. A common type of hand eczema, it worsens in warm weather. (ICD-10 L30.1)
There are several types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, Intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus.
- Pemphigus vulgaris (PV - ICD-10 L10.0) is the most common form of the disorder and occurs when antibodies attack "Desmoglein 3". Sores often originate in the mouth, making eating difficult and uncomfortable. Although pemphigus vulgaris may occur at any age, it is most common among people between the ages of 40 and 60. It is more frequent among Ashkenazi Jews. Rarely, it is associated with myasthenia gravis. Nail disease may be the only finding and has prognostic value in management.
- Pemphigus foliaceus (PF) is the least severe of the three varieties. "Desmoglein 1", the protein that is destroyed by the autoantibody, is found in only the top dry layer of the skin. PF is characterized by crusty sores that often begin on the scalp, and may move to the chest, back, and face. Mouth sores do not occur. This form is also frequent among Ashkenazi Jews. It is not as painful as pemphigus vulgaris, and is often mis-diagnosed as dermatitis or eczema
- Intraepidermal neutrophilic IgA dermatosis is characterized histologically by intraepidermal bullae with neutrophils, some eosinophils, and acantholysis.
- The least common and most severe type of pemphigus is paraneoplastic pemphigus (PNP). This disorder is a complication of cancer, usually lymphoma and Castleman's disease. It may precede the diagnosis of the tumor. Painful sores appear on the mouth, lips, and the esophagus. In this variety of pemphigus, the disease process often involves the lungs, causing bronchiolitis obliterans (constrictive bronchiolitis). Though much less frequent, it is still found the most in the Ashkenazi Jewish population. Complete removal of and/or cure of the tumor may improve the skin disease, but lung damage is generally irreversible.
- Endemic pemphigus foliaceus, including the Fogo Selvagem, the new variant of endemic pemphigus folaiceus in El Bagre, Colombia, South America, and the Tunisian endemic pemphigus in North Africa.
Note that Hailey-Hailey disease, also called familial benign pemphigus, is an inherited (genetic) skin disease, not an autoimmune disease. It is therefore not considered part of the Pemphigus group of diseases.
The rash is composed of small papular lesions, each on a separate reddened base.
Dermatitis herpetiformis is characterized by intensely itchy, chronic papulovesicular eruptions, usually distributed symmetrically on extensor surfaces (buttocks, back of neck, scalp, elbows, knees, back, hairline, groin, or face). The blisters vary in size from very small up to 1 cm across.
The condition is extremely itchy, and the desire to scratch can be overwhelming. This sometimes causes the sufferer to scratch the blisters off before they are examined by a physician. Intense itching or burning sensations are sometimes felt before the blisters appear in a particular area.
Untreated, the severity of DH can vary significantly over time, in response to the amount of gluten ingested.
Dermatitis herpetiformis symptoms typically first appear in the early years of adulthood between 20 and 30 years of age.
Although the first signs and symptoms of dermatitis herpetiformis are intense itching and burning, the first visible signs are the small papules or vesicles that usually look like red bumps or blisters. The rash rarely occurs on other mucous membranes, excepting the mouth or lips. The symptoms range in severity from mild to serious, but they are likely to disappear if gluten ingestion is avoided and appropriate treatment is administered.
Dermatitis herpetiformis symptoms are chronic, and they tend to come and go, mostly in short periods of time. Sometimes, these symptoms may be accompanied by symptoms of coeliac disease, commonly including abdominal pain, bloating or loose stool, and fatigue.
The rash caused by dermatitis herpetiformis forms and disappears in three stages. In the first stage, the patient may notice a slight discoloration of the skin at the site where the lesions appear. In the next stage, the skin lesions transform into obvious vesicles and papules that are likely to occur in groups. Healing of the lesions is the last stage of the development of the symptoms, usually characterized by a change in the skin color. This can result in areas of the skin turning darker or lighter than the color of the skin on the rest of the body. Because of the intense itching, patients usually scratch, which can lead to the formation of crusts.
Erythema toxicum neonatorum (also known as erythema toxicum, urticaria neonatorum and toxic erythema of the newborn) is a common rash in neonates. It appears in up to half of newborns carried to term, usually between day 2–5 after birth; it does not occur outside the neonatal period.
Erythema toxicum is characterized by blotchy red spots on the skin with overlying white or yellow papules or pustules. These lesions may be few or numerous. The eruption typically resolves within first two weeks of life, and frequently individual lesions will appear and disappear within minutes or hours. It is a benign condition thought to cause no discomfort to the baby.
Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus. It is classified as a type II hypersensitivity reaction in which antibodies are formed against desmosomes, components of the skin that function to keep certain layers of skin bound to each other. As desmosomes are attacked, the layers of skin separate and the clinical picture resembles a blister. Over time the condition inevitably progresses without treatment: lesions increase in size and distribution throughout the body, behaving physiologically like a severe burn.
Before the advent of modern treatments, mortality for the disease was close to 90%. Today, the mortality rate with treatment is between 5-15%.
Pemphigus ( or ) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. The name is derived from the Greek root "pemphix" meaning "pustule".
In pemphigus, autoantibodies form against desmoglein. Desmoglein forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes "unglued", a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a significant area of the skin.
Originally, the cause of this disease was unknown, and "pemphigus" was used to refer to any blistering disease of the skin and mucosa. In 1964, researchers found that the blood of patients with pemphigus contained antibodies to the layers of skin that separate to form the blisters. In 1971, an article investigating the autoimmune nature of this disease was published.
Infection of the skin is distinguished from dermatitis, which is inflammation of the skin, but a skin infection can result in skin inflammation. Skin inflammation due to skin infection is called "infective dermatitis."
Bacterial skin infections affected about 155 million people and cellulitis occurred in about 600 million people in 2013.
Bacterial skin infections include:
- Folliculitis is an infection of the hair follicle that can resemble pimples.
- Impetigo is a highly contagious bacterial skin infection most common among pre-school children. It is primarily caused by "Staphylococcus aureus", and sometimes by "Streptococcus pyogenes".
- Erysipelas is an acute streptococcus bacterial infection of the deep epidermis with lymphatic spread.
- Cellulitis is a diffuse inflammation of connective tissue with severe inflammation of dermal and subcutaneous layers of the skin. Cellulitis can be caused by normal skin flora or by exogenous bacteria, and often occurs where the skin has previously been broken: cracks in the skin, cuts, blisters, burns, insect bites, surgical wounds, intravenous drug injection or sites of intravenous catheter insertion. Skin on the face or lower legs is most commonly affected by this infection, though cellulitis can occur on any part of the body.
Pustular psoriasis is classified into two major forms: localized and generalized pustular psoriasis. Within these two categories there are several variants:
Bullous impetigo, mainly seen in children younger than 2 years, involves painless, fluid-filled blisters, mostly on the arms, legs, and trunk, surrounded by red and itchy (but not sore) skin. The blisters may be large or small. After they break, they form yellow scabs.
This most common form of impetigo, also called nonbullous impetigo, most often begins as a red sore near the nose or mouth which soon breaks, leaking pus or fluid, and forms a honey-colored scab, followed by a red mark which heals without leaving a scar. Sores are not painful, but they may be itchy. Lymph nodes in the affected area may be swollen, but fever is rare. Touching or scratching the sores may easily spread the infection to other parts of the body.
Skin ulcers with redness and scarring also may result from scratching or abrading the skin.
Vesicular pemphigoid is a cutaneous condition, a clinical variant of bullous pemphigoid, characterized by a dermatitis herpetiformis-like presentation with grouped small tense blisters.
A vesiculobullous disease is a type of mucocutaneous disease characterized by vesicles and bullae (i.e. blisters). Both vesicles and bullae are fluid-filled lesions, and they are distinguished by size (vesicles being less than 5–10 mm and bulla being larger than 5–10 mm, depending upon which definition is used). In the case of vesiculobullous diseases which are also immune disorders, the term "immunobullous" is sometimes used. Examples of vesiculobullous diseases include:
- "Infectious: (viral)"
- Herpes simplex
- Varicella-Zoster infection
- Hand, foot and mouth disease
- Herpangina
- Measles (Rubeola)
- "Immunobullous:"
- Pemphigus vulgaris
- Pemphigoid
- Dermatitis herpetiformis
- Linear immunoglobulin-A disease (linear IgA disease)
- "Genetic:"
- Epidermolysis bullosa
Some features are as follows:
Bullous impetigo can appear around the diaper region, axilla, or neck. The bacteria causes a toxin to be produced that reduces cell-to-cell stickiness (adhesion), causing for the top layer of skin (epidermis), and lower layer of skin (dermis) to separate. Vesicles rapidly enlarge and form the bullae which is a blister more than 5mm across. Bullae is also known as Staphylococcal scalded skin syndrome. Other associated symptoms are itching, swelling of nearby glands, fever and diarrhea. It should also be noted that pain is very rare.
Long-term effects: once the scabs on the bullous have fallen off, scarring is minimal. Possible long-term effects are kidney disease.
Dermatitis herpetiformis (DH), or Duhring's disease, is a chronic blistering skin condition, characterised by blisters filled with a watery fluid. Despite its name, DH is neither related to nor caused by herpes virus: the name means that it is a skin inflammation having an appearance similar to herpes.
DH was first described by Louis Adolphus Duhring in 1884. A connection between DH and celiac disease was recognised in 1967, although the exact causal mechanism is not known. DH is a specific manifestation of celiac disease.
The age of onset is usually about 15-40, but DH can also affect children and the elderly. Men and women are equally affected. Estimates of DH prevalence vary from 1 in 400 to 1 in 10,000. It is most common in patients of northern European/northern Indian ancestry, and is associated with the human leukocyte antigen (HLA) haplotype HLA-DQ2 along with coeliac disease and gluten sensitivity.
The disease presents with the widespread formation of fluid-filled blisters that are thin walled and easily ruptured and the patient can be positive for Nikolsky's sign. Ritter's Disease of the Newborn is the most severe form of SSSS with similar signs and symptoms. SSSS often includes a widespread painful erythroderma, often involving the face, diaper, and other intertriginous areas. Extensive areas of desquamation might be present. Perioral crusting and fissuring are seen early in the course. Unlike toxic epidermal necrolysis, SSSS spares the mucous membranes. It is most common in children under 6 years, but can be seen in adults who are immunosuppressed or have renal failure.
Cradle cap is seborrheic dermatitis that affects infants. It presents on the scalp as greasy patches of scaling, which appear thick, crusty, yellow, white or brown. The affected regions are not usually itchy and do not bother the child. Other affected areas can include the eyelids, ear, around the nose, and in the groin. Hair loss can also occur.
Eczema vaccinatum is a rare severe adverse reaction to smallpox vaccination.
It is characterized by serious local or disseminated, umbilicated, vesicular, crusting skin rashes in the face, neck, chest, abdomen, upper limbs and hands, caused by widespread infection of the skin in people with previous diagnosed skin conditions such as eczema or atopic dermatitis, even if the conditions are not active at the time. Other signs and symptoms include fever and facial and supraglottic edema. The condition may be fatal if severe and left untreated. Survivors are likely to have some scarring (pockmarks).
Smallpox vaccine should not be given to patients with a history of eczema. Because of the danger of transmission of vaccinia, it also should not be given to people in close contact with anyone who has active eczema and who has not been vaccinated. People with other skin diseases (such as atopic dermatitis, burns, impetigo, or herpes zoster) also have an increased risk of contracting eczema vaccinatum and should not be vaccinated against smallpox.
Eczema is also associated with increased complications related to other vesiculating viruses such as chickenpox; this is called eczema herpeticum.
Bullous Impetigo is a skin condition that characteristically occurs in the newborn, and is caused by a bacterial infection, presenting with bullae.
It can be caused by Exfoliative toxin A. The phyogenic superficial infection can be divided into two other subdivisions; Impetigo, and non-bullous impetigo. Bullous impetigo is caused by "Staphylococcus aureus," which produces exfoliative toxins, whereas non-bullous impetigo is caused by either "Staphylococcus aureus", or "Streptococcus pyogenes." Thirty percent of all Impetigo cases are related to Bullous impetigo. Bullous impetigo, in newborns, children, or adults who are immunocompromised and/or are experiencing renal failure, can develop into a more severe and generalized form called Staphylococcal scalded skin syndrome (SSSS). The mortality rate is less than 3% for infected children, but up to 60% in adults.
Cradle cap is a yellowish, patchy, greasy, scaly and crusty skin rash that occurs on the scalp of recently born babies. It is usually not itchy and does not bother the baby. Cradle cap most commonly begins sometime in the first three months but can occur in later years. Similar symptoms in older children are more likely to be dandruff than cradle cap. The rash is often prominent around the ear, the eyebrows or the eyelids. It may appear in other locations as well, where it is called seborrhoeic dermatitis rather than cradle cap. Some countries use the term "pityriasis capitis" for cradle cap. It is extremely common, with about half of all babies affected. Most of them have a mild version of the disorder. Severe cradle cap is rare.
Impetigo herpetiformis is a form of severe pustular psoriasis occurring in pregnancy which may occur during any trimester.
It is the only well known pustular psoriasis which is treated with steroids.