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Weber–Christian disease, also known as relapsing febrile nodular nonsuppurative panniculitis, is a cutaneous condition characterized by recurrent subcutaneous nodules that heal with depression of the overlying skin.
It is type of panniculitis.
It is a rare disease seen in females 30–60 years of age. It is a recurring inflammation of fatty layers of tissue present beneath the skin. Clinical course is characterised by exacerbations and remissions. Lesions are bilaterally symmetrical and are usually seen in the lower legs.
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.
Non-vasculitis forms of panniculitis that may occur include:
- "Cytophagic Histiocytic Panniculitis" was first described in 1980 by Winkelmann as a chronic histiocytic disease of the subcutaneous adipose tissue, which is characterized clinically by tender erythematous nodules, recurrent high fever, malaise, jaundice, organomegaly, serosal effusions, pancytopenia, hepatic dysfunction and Coagulation abnormalities. CHP may occur either isolated or as part of cutaneous manifestations of Hemophagocytic syndrome (HPS). CHP is a rare and often fatal form of panniculitis with multisystem involvement. But it can also present in a benign form involving only the subcutaneous tissue, thus having a broad clinical spectrum.
- "Traumatic panniculitis" is a panniculitis that occurs following trauma to the skin.
- "Cold panniculitis" is a panniculitis occurring after exposure to cold, most often seen in infants and young children. This condition has been described in children who suck ice or popsicles, and therefore is sometimes referred to as "popsicle panniculitis." The term was coined when a patient with a rash of unknown origin on her cheek was taken to a dermatologist.
- "Gouty panniculitis" is a panniculitis caused by deposition of uric acid crystals in gout.
- "Pancreatic panniculitis" (also known as "Enzymatic panniculitis," "Pancreatic fat necrosis," and "Subcutaneous fat necrosis") is a panniculitis most commonly associated with pancreatic carcinoma, and more rarely with anatomic pancreatic abnormalities, pseudocysts, or drug-induced pancreatitis.
- "Factitial panniculitis" is a panniculitis that may be induced by the injection of organic materials, povidone, feces, saliva, vaginal fluid, and oils.
The first signs of erythema nodosum are often flu-like symptoms such as a fever, cough, malaise, and aching joints. Some people also experience stiffness or swelling in the joints and weight loss.
Erythema nodosum is characterised by nodules (rounded lumps) below the skin surface, usually on the shins. These subcutaneous nodules can appear anywhere on the body, but the most common sites are the shins, arms, thighs, and torso. Each nodule typically disappears after around two weeks, though new one may continue to form for up to six or eight weeks. A new nodule usually appears red and is hot and firm to touch. The redness starts to fade and it gradually becomes softer and smaller until it disappears. Each nodule usually heals completely without scarring over the course of about two weeks. Joint pain and inflammation sometimes continues for several weeks or months after the nodules appear.
Less common variants of erythema nodosum include:
- Ulcerating forms, seen in Crohn's disease
- Erythema contusiforme, when a subcutaneous hemorrhage (bleeding under the skin) occurs with a erythema nodosum lesion, causing the lesion to look like a contusion (bruise)
- Erythema nodosum migrans (also known as subacute nodular migratory panniculitis), a rare form of chronic erythema nodosum characterized by asymmetrical nodules that are mildly tender and migrate over time.
Pain may be the first noticed symptom. People with lipodermatosclerosis have tapering of their legs above the ankles, forming a constricting band resembling an inverted champagne bottle. In addition, there may be brownish-red pigmentation and induration.
Lipodermatosclerosis (also known as "chronic panniculitis with lipomembranous changes", "hypodermitis sclerodermiformis", "sclerosing panniculitis", and "stasis panniculitis") is a skin and connective tissue disease. It is a form of lower extremity panniculitis, an inflammation of the layer of fat under the epidermis.
Septal panniculitis is a condition of the subcutaneous fat affecting the layer of adipose tissue that lies between the dermis and underlying fascia, of which there are two forms: acute erythema nodosum and chronic erythema nodosum.
It is named after Frederick Parkes Weber and Henry Asbury Christian.
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.
Patients mostly present with a hard lump in one breast without any sign of a systemic disease. Other possible symptoms include nipple retraction, pain, inflammation of the overlying skin, nipple discharge, fistula, enlarged lymph nodes, in rare case peau d'orange-like changes.
Presentation is mostly unilateral although a significant share of cases is bilateral, also in many cases contralateral or bilateral recurrences were documented.
Several cases occurring together with fever, polyarthralgia and erythema nodosum were documented.
Characteristic for idiopathic granulomatous mastitis are multinucleated giant cells and epithelioid histiocytes forming non-caseating granulomas around lobules. Often minor ductal and periductal inflammation is present. The lesion is in some cases very difficult to distinguish from breast cancer and other causes such as infections (tuberculosis, syphilis, corynebacterial infection, mycotic infection), autoimmune diseases (sarcoidosis, granulomatosis with polyangiitis), foreign body reaction and granulomatous reaction in a carcinoma must be excluded.
The condition is diagnosed very rarely. As the diagnosis is a lengthy differential diagnosis of exclusion there is considerable uncertainty about incidence. It has been suspected that some cases diagnosed as IGM in developing countries may have other explanations. On the other hand, IGM is usually diagnosed only after complications and referral to a secondary breast care center so light cases may resolve spontaneously or after symptomatic treatment and thus never be diagnosed as IGM. As a completely pathogen free breast will be exceedingly rare even in completely healthy population there is also uncertainty when to consider pathogens as causative or as mere coincidental finding.
Granulomatous mastitis can be divided into idiopathic granulomatous mastitis (also known as granular lobular mastitis) and granulomatous mastitis occurring as a rare secondary complication of a great variety of other conditions such as tuberculosis and other infections, sarcoidosis and granulomatosis with polyangiitis. Special forms of granulomatous mastitis occur as complication of diabetes. Some cases are due to silicone injection (Silicone-induced granulomatous inflammation) or other foreign body reactions.
Primary presentation of any of these conditions as mastitis is very rare and in many cases probably predisposed by other breast or systemic conditions.
Although granulomatous mastitis is easily confused with cancer it is a completely benign (non-cancerous) condition.
Treatment is radically different for idiopathic granulomatous mastitis and other granulomatous lesions of the breast, the precise diagnosis is therefore very important.
The symptoms of CANDLE syndrome can manifest themselves in a variety of different ways and combinations related to skin disorders, internal inflammatory responses, and fever-based conditions. The types of outwardly visible conditions involve facies not matching other known disorders, contracture of the joints, and skin lesions appearing across any part of the body. The multiple inflammatory developments include nonspecific lymphadenopathy, hepatosplenomegaly, and autoimmune hemolytic anemia. Other possible conditions are hypertriglyceridemia and lipodystrophy.
Other novel mutations resulting in the syndrome have also involved the manifestation of other conditions, such as Sweet's syndrome and pericarditis. Another case in 2015 showcased previously undescribed dental symptoms, such as microdontia and osteopenia of the jaw, along with a general case of diabetes mellitus.
Neutrophilic lobular panniculitis is a cutaneous condition characterized by inflammation of the subcutaneous fat.
Affected individuals typically present with sudden painful proptosis, redness, and edema. Proptosis will vary according to the degree of inflammation, fibrosis, and mass effect. Occasionally, ptosis, chemosis, motility dysfunction (ophthalmoplegia), and optic neuropathy are seen. In the setting of extensive sclerosis there may be restriction, compression, and destruction of orbital tissue. Symptoms usually develop acutely (hours to days), but have also been seen to develop over several weeks or even months.Malaise, headaches, and nausea may accompany these symptoms. Other unusual presentations described include cystoid macular edema, temporal arteritis, and cluster headaches.
Pediatric IOI accounts for about 17% of cases idiopathic orbital inflammation. The most common sign is proptosis, but redness and pain are also experienced. Presentation varies slightly compared to adults with bilateral involvement, uveitis, disc edema and tissue eosinophilia being more common in this population. The presence of uveitis generally implies a poor outcome for pediatric IOI. Bilateral presentation may have a higher incidence of systemic disease.
The condition most commonly is located at the junction of the hard and soft palate. However, the condition may arise anywhere minor salivary glands are located. It has also been occasionally reported to involve the major salivary glands. It may be present only on one side, or both sides. The lesion typically is 1–4 cm in diameter.
Initially, the lesion is a tender, erythematous (red) swelling. Later, in the ulcerated stage, the overlying mucosa breaks down to leave a deep, well-circumscribed ulcer which is yellow-gray in color and has a lobular base.
There is usually only minor pain, and the condition is often entirely painless. There may be prodromal symptoms similar to flu before the appearance of the lesion.
Sclerosing mesenteritis may present with no or nearly no signs or symptoms, but many people have chronic and severe pain in the abdomen as the most common chief complaint. Other people have chronic problems with bowel movements, resulting in diarrhea, bloating, gas, and cramping which can range from severe to mild.
The disorder is identified by histopathology showing fat necrosis, fibrosis and chronic inflammation of the small intestine. Examination of the mesentery may indicate a solitary mass, but diffuse mesentery thickening is common.
It often mimics other abdominal diseases such as pancreatic or disseminated cancer. CT scanning is important for making the initial diagnosis.
Idiopathic scrotal calcinosis (also known as idiopathic calcified nodules of the scrotum) is a cutaneous condition characterized by calcification of the skin resulting from the deposition of calcium and phosphorus occurring on the scrotum. However, the levels of calcium and phosphate in the blood are normal. Idiopathic scrotal calcinosis typically affects young males, with an onset between adolescence and early adulthood. The scrotal calcinosis appears, without any symptoms, as yellowish nodules that range in size from 1 mm to several centimeters.
The cause is not well defined. Originally considered idiopathic condition. Now accepted that majority of cases develop from dystrophic calcification of cyst contents.
A differential diagnosis includes lymphoproliferative lesions, thyroid ophthalmopathy, IgG4-related ophthalmic disease, sarcoidosis, granulomatosis with polyangiitis, orbital cellulitis and carotid-cavernous fistula.
Atrophic connective tissue panniculitis is a rare condition, and often occurs on the upper or lower extremities.
Calcinosis cutis may be divided into the following types:
- Dystrophic calcinosis cutis
- Metastatic calcinosis cutis
- Iatrogenic calcinosis cutis
- Traumatic calcinosis cutis
- Idiopathic calcinosis cutis
- Idiopathic scrotal calcinosis
- Subepidermal calcified nodule
- Tumoral calcinosis
- Osteoma cutis
AIP is relatively uncommon and is characterized by the following features:
1. Scleral Icterus (yellow eyes), jaundice (yellow skin) which is usually painless, usually without acute attacks of pancreatitis.
2. Relatively mild symptoms, such as minimal weight loss or nausea.
3. Increased serum levels of gamma globulins, immunoglobulin G (IgG) or IgG4.
4. The presence of serum autoantibodies such as anti-nuclear antibody (ANA), anti-lactoferrin antibody, anti-carbonic anhydrase II antibody, and rheumatoid factor (RF).
5. Contrast-enhanced CT demonstrates a diffusely enlarged (sausage-shaped) pancreas.
6. Diffuse irregular narrowing of the main pancreatic duct, and stenosis of the intrapancreatic bile duct on endoscopic retrograde cholangiopancreatography (ERCP).
7. Rare pancreatic calcification or cyst formation.
8. Marked responsiveness to treatment with corticosteroids.
Two-thirds of patients present with either obstructive painless jaundice or a "mass" in the head of the pancreas mimicking carcinoma. It is mandatory to rule out carcinoma prior to making a diagnosis of AIP.
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
Necrotizing sialometaplasia (NS) is a benign, ulcerative lesion, usually located towards the back of the hard palate. It is thought to be caused by ischemic necrosis (death of tissue due to lack of blood supply) of minor salivary glands in response to trauma. Often painless, the condition is self-limiting and should heal in 6–10 weeks.
Although entirely benign and requiring no treatment, due to its similar appearance to oral cancer, it is sometimes misdiagnosed as malignant. Therefore, it is considered an important condition, despite its rarity.