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The types of neurosyphilis include asymptomatic, acute syphilitic meningitis, meningovascular syphilis, parenchymatous syphilis (which includes general paresis and tabes dorsalis), and optic atrophy.
Gummas have a firm, necrotic center surrounded by inflamed tissue, which forms an amorphous proteinaceous mass. The center may become partly hyalinized.
These central regions begin to die through coagulative necrosis, though they also retain some of the structural characteristics of previously normal tissues, enabling a distinction from the granulomas of tuberculosis where caseous necrosis obliterates preexisting structures. Other histological features of gummas include an "intervening zone" containing epithelioid cells with indistinct borders and multinucleated giant cells, and a "peripheral zone" of fibroblasts and capillaries. Infiltration of lymphocytes and plasma cells can be seen in the peripheral zone as well. With time, gummas eventually undergo fibrous degeneration, leaving behind an irregular scar or a round fibrous nodule.
It is restricted to necrosis involving spirochaetal infections that cause syphilis. Growths that have the appearance of gummas are described as gummatous.
Meningeal syphilis (as known as syphilitic aseptic meningitis or meningeal neurosyphilis) is a chronic form of syphilis infection that affects the central nervous system. Treponema pallidum, which is a spirochate bacterium, is the main cause of syphilis, which spreads drastically throughout the body and can infect all the systems of the body if not treated appropriately. The bacterium is the main cause of the onset of meningeal syphilis and other treponemal diseases, and it consists of a cytoplasmic and outer membrane that can cause a diverse array of diseases in the central nervous system and brain.
Early symptomatic neurosyphillis (or acute syphilitic meningitis or neurorecurrence) is the onset of meningeal syphilis. The symptoms arise as a result of inflamed meninges, which eventually lead up to signs of meningitis.
"Treponema pallidum" invades the nervous system within three to eighteen months after the primary infection. The initial series of events is asymptomatic meningitis, which can remain in the human body system and produce more damage within the body. Every form of neurosyphilis has meningitis as a component; however, every case differs in severity. The individual is infected with syphilis through a gram negative bacteria that only humans can obtain. Syphilis has four stages of infection, which are primary, secondary, latent, and tertiary. If syphilis is not treated, the disease can affect various other systems in the body, including the brain, heart, and vessels. The infection of the heart and vessels leads to meningovascular syphilis, which is usually presented during the secondary stage of syphilis. If syphilis is prolonged, it can affect the nervous system, which is known as neurosyphilis. Meningeal syphilis is a component of neurosyphilis, which usually occurs in the tertiary stage of syphilis.
A gumma is a soft, non-cancerous growth resulting from the tertiary stage of syphilis. It is a form of granuloma. Gummas are most commonly found in the liver ("gumma hepatis"), but can also be found in brain, heart, skin, bone, testis, and other tissues, leading to a variety of potential problems including neurological disorders or heart valve disease.
Possible symptoms include:
- General symptoms: Fever, weight loss
- Skin: Palpable purpura, livedo reticularis
- Muscles and joints: Myalgia or myositis, arthralgia or arthritis
- Nervous system: Mononeuritis multiplex, headache, stroke, tinnitus, reduced visual acuity, acute visual loss
- Heart and arteries: Myocardial infarction, hypertension, gangrene
- Respiratory tract: Nose bleeds, bloody cough, lung infiltrates
- GI tract: Abdominal pain, bloody stool, perforations
- Kidneys: Glomerulonephritis
Garre's sclerosing osteomyelitis is a type of chronic osteomyelitis also called proliferative periostitis, periostitis ossificans and Garré's sclerosing osteomyelitis.
It is a rare disease. It mainly affects children and young adults. It is associated with a low grade infection, which may be due to dental caries (cavities in the teeth).
The body of the mandible may show irregular lucent/opaque changes with subperiosteal opaque layering along inferior border. It is a chronic osteomyelitis with subperiosteal bone and collagen deposition.
There is no suppuration and sinus formation.
It was first described by the Swiss surgeon Carl Garré.
Acute periostitis is due to infection, is characterized by diffuse formation of pus, severe pain, constitutional symptoms, and usually results in necrosis. It can be caused by excessive physical activity as well, as in the case of medial tibial stress syndrome (also referred to as tibial periostalgia, soleus periostalgia, or shin splints). Congenital infection with syphilis can also cause periostitis in newborn infants.
Vasculitis can be classified by the cause, the location, the type of vessel or the size of vessel.
- "Underlying cause". For example, the cause of syphilitic aortitis is infectious (aortitis simply refers to inflammation of the aorta, which is an artery.) However, the causes of many forms of vasculitis are poorly understood. There is usually an immune component, but the trigger is often not identified. In these cases, the antibody found is sometimes used in classification, as in ANCA-associated vasculitides.
- "Location of the affected vessels". For example, ICD-10 classifies "vasculitis limited to skin" with skin conditions (under "L"), and "necrotizing vasculopathies" (corresponding to systemic vasculitis) with musculoskeletal system and connective tissue conditions (under "M"). Arteritis/phlebitis on their own are classified with circulatory conditions (under "I").
- "Type or size of the blood vessels" that they predominantly affect. Apart from the arteritis/phlebitis distinction mentioned above, vasculitis is often classified by the caliber of the vessel affected. However, there can be some variation in the size of the vessels affected.
According to the size of the vessel affected, vasculitis can be classified into:
- Large vessel: Polymyalgia rheumatica, Takayasu's arteritis, Temporal arteritis
- Medium vessel: Buerger's disease, Kawasaki disease, Polyarteritis nodosa
- Small vessel: Behçet's syndrome, Eosinophilic granulomatosis with polyangiitis, Cutaneous vasculitis, Henoch–Schönlein purpura, Microscopic polyannulomatosis ConditionofSome disorders have vasculitis as their main feature. The major types are given in the table below:
Takayasu's arteritis, polyarteritis nodosa and giant cell arteritis mainly involve arteries and are thus sometimes classed specifically under arteritis.
Furthermore, there are many conditions that have vasculitis as an accompanying or atypical feature, including:
- Rheumatic diseases, such as rheumatoid arthritis, systemic lupus erythematosus, and dermatomyositis
- Cancer, such as lymphomas
- Infections, such as hepatitis C
- Exposure to chemicals and drugs, such as amphetamines, cocaine, and anthrax vaccines which contain the Anthrax Protective Antigen as the primary ingredient.
In pediatric patients varicella inflammation may be followed by vasculitis of intracranial vessels. This condition is called post varicella angiopathy and this may be responsible for arterial ischaemic strokes in children.
Several of these vasculitides are associated with antineutrophil cytoplasmic antibodies. These are:
- Granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis)
- Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome)
- Microscopic polyangiitis
Late congenital syphilis is a subset of cases of congenital syphilis. By definition, it occurs in children at or greater than 2 years of age who acquired the infection trans-placentally.
Symptoms include
- blunted upper incisor teeth known as Hutchinson's teeth
- inflammation of the cornea known as interstitial keratitis
- deafness from auditory nerve disease
- frontal bossing (prominence of the brow ridge)
- saddle nose (collapse of the bony part of nose)
- hard palate defect
- swollen knees
- saber shins
- short maxillae
- protruding mandible
A frequently-found group of symptoms is Hutchinson's triad, which consists of Hutchinson's teeth (notched incisors), keratitis and deafness and occurs in 63% of cases.
Treatment (with penicillin) before the development of late symptoms is essential.
Tertiary syphilis may occur approximately 3 to 15 years after the initial infection, and may be divided into three different forms: gummatous syphilis (15%), late neurosyphilis (6.5%), and cardiovascular syphilis (10%). Without treatment, a third of infected people develop tertiary disease. People with tertiary syphilis are not infectious.
Gummatous syphilis or late benign syphilis usually occurs 1 to 46 years after the initial infection, with an average of 15 years. This stage is characterized by the formation of chronic gummas, which are soft, tumor-like balls of inflammation which may vary considerably in size. They typically affect the skin, bone, and liver, but can occur anywhere.
Neurosyphilis refers to an infection involving the central nervous system. It may occur early, being either asymptomatic or in the form of syphilitic meningitis, or late as meningovascular syphilis, general paresis, or tabes dorsalis, which is associated with poor balance and lightning pains in the lower extremities. Late neurosyphilis typically occurs 4 to 25 years after the initial infection. Meningovascular syphilis typically presents with apathy and seizure, and general paresis with dementia and tabes dorsalis. Also, there may be Argyll Robertson pupils, which are bilateral small pupils that constrict when the person focuses on near objects but do not constrict when exposed to bright light.
Cardiovascular syphilis usually occurs 10–30 years after the initial infection. The most common complication is syphilitic aortitis, which may result in aneurysm formation.
Death from congenital syphilis is usually due to bleeding into the lungs.
Secondary syphilis occurs approximately four to ten weeks after the primary infection. While secondary disease is known for the many different ways it can manifest, symptoms most commonly involve the skin, mucous membranes, and lymph nodes. There may be a symmetrical, reddish-pink, non-itchy rash on the trunk and extremities, including the palms and soles. The rash may become maculopapular or pustular. It may form flat, broad, whitish, wart-like lesions known as condyloma latum on mucous membranes. All of these lesions harbor bacteria and are infectious. Other symptoms may include fever, sore throat, malaise, weight loss, hair loss, and headache. Rare manifestations include liver inflammation, kidney disease, joint inflammation, periostitis, inflammation of the optic nerve, uveitis, and interstitial keratitis. The acute symptoms usually resolve after three to six weeks; about 25% of people may present with a recurrence of secondary symptoms. Many people who present with secondary syphilis (40–85% of women, 20–65% of men) do not report previously having had the classic chancre of primary syphilis.
Bejel usually begins in childhood as a small mucous patch, often on the interior of the mouth, followed by the appearance of raised, eroding lesions on the limbs and trunk. Periostitis (inflammation) of the leg bones is commonly seen, and gummas of the nose and soft palate develop in later stages.
Periostitis, also known as periostalgia, is a medical condition caused by inflammation of the periosteum, a layer of connective tissue that surrounds bone. The condition is generally chronic, and is marked by tenderness and swelling of the bone and an aching pain.
Bejel, or endemic syphilis, is a chronic skin and tissue disease caused by infection by the "endemicum" subspecies of the spirochete "Treponema pallidum".
Bejel is also known by a variety of other names, including belesh, dichuchwa, endemic syphilis, nonvenereal syphilis, frenga, njovera, skerljevo, siti, or treponematosis-bejel type.
Symptom may include pain in a specific bone with overlying redness, fever, and weakness. Onset may be sudden or gradual.
These are only local and no systemic manifestations are present.
The ulcer characteristically:
- Ranges in size dramatically from 3 to 50 mm (1/8 inch to two inches) across
- Is painful
- Has sharply defined, undermined borders
- Has irregular or ragged borders
- Has a base that is covered with a gray or yellowish-gray material
- Has a base that bleeds easily if traumatized or scraped
- painful swollen lymph nodes occurs in 30 to 60% of patients.
- dysuria (pain with urination) and dyspareunia (pain with intercourse) in females
About half of infected men have only a single ulcer. Women frequently have four or more ulcers, with fewer symptoms.
The initial ulcer may be mistaken as a "hard" chancre, the typical sore of primary syphilis, as opposed to the "soft chancre" of chancroid.
Approximately one-third of the infected individuals will develop enlargements of the inguinal lymph nodes, the nodes located in the fold between the leg and the lower abdomen.
Half of those who develop swelling of the inguinal lymph nodes will progress to a point where the nodes rupture through the skin, producing draining abscesses. The swollen lymph nodes and abscesses are often referred to as buboes.
Granuloma inguinale (also known as donovanosis) is a bacterial disease caused by "Klebsiella granulomatis" (formerly known as "Calymmatobacterium granulomatis") characterized by ulcerative genital lesions. It is endemic in many less developed regions. It is also known as donovanosis, granuloma genitoinguinale, granuloma inguinale tropicum, granuloma venereum, granuloma venereum genitoinguinale, lupoid form of groin ulceration, serpiginous ulceration of the groin, ulcerating granuloma of the pudendum, and ulcerating sclerosing granuloma.
The disease often goes untreated because of the scarcity of medical treatment in the countries in which it is found. In addition, the painless genital ulcers can be mistaken for syphilis. The ulcers ultimately progress to destruction of internal and external tissue, with extensive leakage of mucus and blood from the highly vascular lesions. The destructive nature of donovanosis also increases the risk of superinfection by other pathogenic microbes.
The diagnosis could be differentiated from other known psychoses and dementias by a characteristic abnormality in eye pupil reflexes (Argyll Robertson pupil), and, eventually, the development of muscular reflex abnormalities, seizures, memory impairment (dementia) and other signs of relatively pervasive neurocerebral deterioration. Definitive diagnosis is based on the analysis of cerebrospinal fluid and tests for syphilis.
Osteomyelitis (OM) is an infection of bone. Symptoms may include pain in a specific bone with overlying redness, fever, and weakness. The long bones of the arms and legs are most commonly involved in children while the feet, spine, and hips are most commonly involved in adults.
The cause is usually a bacterial infection and rarely a fungal infection. It may occur via spread from the blood or from surrounding tissue. Risks for developing osteomyelitis include diabetes, intravenous drug use, prior removal of the spleen, and trauma to the area. Diagnosis is typically suspected based on symptoms. This is then supported by blood tests, medical imaging, or bone biopsy.
Treatment often involves both antimicrobials and surgery. In those with poor blood flow, amputation may be required. With treatment outcomes are often generally good when the condition has only been present a short time. About 2.4 per 100,000 people are affected a year. The young and old are more commonly affected. Males are more commonly affected than females. The condition was described at least as early as the 300s BC by Hippocrates. Before the availability of antibiotics the risk of death was significant.
Tabes dorsalis is caused by demyelination by advanced syphilis infection (tertiary syphilis), when the primary infection by the causative spirochete bacterium, "Treponema pallidum", is left untreated for an extended period of time (past the point of blood infection by the organism).
Small, painless nodules appear after about 10–40 days of the contact with the bacteria. Later, the nodules burst, creating open, fleshy, oozing lesions. The infection spreads, mutilating the infected tissue. The infection will continue to destroy the tissue until treated. The lesions occur at the region of contact typically found on the shaft of the penis, the labia, or the perineum. Rarely, the vaginal wall or cervix is the site of the lesion. At least one case in India led to partial autoamputation of the penis. The patient tested positive for HIV-2 and had been infected for six years.
Arthritis mutilans is a rare medical condition involving severe inflammation damaging the joints of the hands and feet, and resulting in deformation and problems with moving the affected areas; it can also affect the spine. As an uncommon arthropathy, arthritis mutilans was originally described as affecting the hands, feet, fingers, and/or toes, but can refer in general to severe derangement of any joint damaged by arthropathy. First described in modern medical literature by Marie and Leri in 1913, in the hands, arthritis mutilans is also known as opera glass hand ("la main en lorgnette" in French), or chronic absorptive arthritis. Sometimes there is foot involvement in which toes shorten and on which painful calluses develop in a condition known as opera glass foot, or "pied en lorgnette".
Although there were recorded cases of remission of the symptoms, especially if they had not passed beyond the stage of psychosis, these individuals almost invariably suffered relapse within a few months to a few years. Otherwise, the patient was seldom able to return home because of the complexity, severity and unmanageability of the evolving symptom picture. Eventually, the patient would become completely incapacitated, bedfast, and die, the process taking about three to five years on average.
For a person with arthritis mutilans in the hands, the fingers become shortened by arthritis, and the shortening may become severe enough that the hand looks paw-like, with the first deformity occurring at the interphalangeal and metacarpophalangeal joints. The excess skin from the shortening of the phalanx bones becomes folded transversely, as if retracted into one another like opera glasses, hence the description "la main en lorgnette". As the condition worsens, luxation, phalangeal and metacarpal bone absorption, and skeletal architecture loss in the fingers occurs.