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Lucio's phenomenon is treated by anti-leprosy therapy (dapsone, rifampin, and clofazimine), optimal wound care, and treatment for bacteremia including antibiotics. In severe cases exchange transfusion may be helpful.
Disease progression may be slowed with immunosuppressives and other medications, and esophageal reflux, pulmonary hypertension and Raynaud phenomenon may benefit from symptomatic treatment. However, there is no cure for this disease as there is no cure for scleroderma in general.
Medications can be helpful for moderate or severe RP.
- Vasodilators – calcium channel blockers, such as the dihydropyridines nifedipine or amlodipine, preferably slow release preparations – are often first line treatment. They have the common side effects of headache, flushing, and ankle edema; but these are not typically of sufficient severity to require cessation of treatment. The limited evidence available shows that calcium channel blockers are only slightly effective in reducing how often the attacks happen. Peoples whose RP is secondary to erythromelalgia often cannot use vasodilators for therapy as they trigger 'flares' causing the extremities to become burning red due to there being too much blood.
- People with severe RP prone to ulceration or large artery thrombotic events may be prescribed aspirin.
- Sympatholytic agents, such as the alpha-adrenergic blocker prazosin, may provide temporary relief.
- Losartan can, and topical nitrates may, reduce the severity and frequency of attacks, and the phosphodiesterase inhibitors sildenafil and tadalafil may reduce their severity.
- Angiotensin receptor blockers or ACE inhibitors may aid blood flow to the fingers, and there is some evidence that angiotensin receptor blockers (often losartan) reduce frequency and severity of attacks, and possibly better than nifedipine.
- The prostaglandin iloprost is used to manage critical ischemia and pulmonary hypertension in RP, and the endothelin receptor antagonist bosentan is used to manage severe pulmonary hypertension and prevent finger ulcers in scleroderma.
- Statins have a protective effect on blood vessels, and SSRIs such as fluoxetine may help RP symptoms but the data is weak.
Evidence does not support the use of alternative medicine, including acupuncture and laser therapy.
There is no current cure. The only way to treat this disease is by treating symptoms. Commonly patients are prescribed immunosuppressive drugs. Another route would be to take collagen regulation drugs.
Shwartzman phenomenon, also known as Shwartzman reaction, is a rare reaction of a body to particular types of toxins, called endotoxins, which cause thrombosis in the affected tissue. A clearing of the thrombosis results in a reticuloendothelial blockade, which prevents re-clearing of the thrombosis caused by a repeat introduction of the toxin. That will cause tissue necrosis. Shwartzman phenomenon is usually observed during delivery or abortion, when foreign bodies are introduced into the tissues of the female reproductive system.
The Shwartzman phenomenon is named for Gregory Shwartzman, the doctor at Mount Sinai Hospital in New York City who was the first to develop the concept of immune system hypersensitivity in the 1920s.
This reaction was experimented using "Neisseria meningitidis" endotoxin.
This is notably seen with "Neisseria meningitidis".
Some mucoceles spontaneously resolve on their own after a short time. Others are chronic and require surgical removal. Recurrence may occur, and thus the adjacent salivary gland is excised as a preventive measure.
Several types of procedures are available for the surgical removal of mucoceles. These include laser and minimally-invasive techniques which means recovery times are reduced drastically.
Micro-marsupialization is an alternative procedure to surgical removal. Micro-marsupialization uses silk sutures in the dome of a cyst to allow new epithelialized drainage pathways. It is simpler, less traumatic, and well-tolerated by patients, especially children.
A non-surgical option that may be effective for a small or newly identified mucocele is to rinse the mouth thoroughly with salt water (one tablespoon of salt per cup) four to six times a day for a few days. This may draw out the fluid trapped underneath the skin without further damaging the surrounding tissue. If the mucocele persists, individuals should see a doctor to discuss further treatment.
Smaller cysts may be removed by laser treatment, larger cysts will have to be removed surgically in an operating room.
Treatment of an episode of cholesterol emboli is generally symptomatic, i.e. it deals with the symptoms and complications but cannot reverse the phenomenon itself. In kidney failure resulting from cholesterol crystal emboli, statins (medication that reduces cholesterol levels) have been shown to halve the risk of requiring hemodialysis.
CREST syndrome can be noted in up to 10% of patients with primary biliary cirrhosis.
Reynolds syndrome is a rare secondary laminopathy, consisting of the combination of primary biliary cirrhosis and progressive systemic sclerosis. In some patients this syndrome has also been associated with Sjögren's syndrome and hemolytic anemia. Typical clinical features include jaundice, elevated blood levels of alkaline phosphatase, calcinosis cutis, telangiectasias, and pruritus. This disease may cause white or yellow-ish spots on the arms or legs. The syndrome, a special case of scleroderma, is named after the American physician, Telfer B. Reynolds, MD (1921–2004), who first described it. He is also known for creating one of the world's first hepatology programs at the University of Southern California.
It should not be confused with the more common Raynaud's phenomenon.
The diffuse leprosy of Lucio and Latapí, also known as diffuse lepromatous leprosy or "pretty leprosy" is a clinical variety of lepromatous leprosy. It was first described by Lucio and Alvarado in 1852 and re-identified by Latapí in 1936. It is common in Mexico (23% leprosy cases) and in Costa Rica and very rare in other countries.
The Mazzotti reaction, first described in 1948, is a symptom complex seen in patients after undergoing treatment of onchocerciasis with the medication diethylcarbamazine (DEC). Mazzotti reactions can be life-threatening, and are characterized by fever, urticaria, swollen and tender lymph nodes, tachycardia, hypotension, arthralgias, oedema, and abdominal pain that occur within seven days of treatment of microfilariasis. The Mazzotti reaction correlates with intensity of infection; however, there are probably multiple infection intensity-dependent mechanisms responsible for mediating this complex reaction.
The phenomenon is so common when DEC is used for the treatment of onchocerciasis that this drug is the basis of a skin patch test used to confirm that diagnosis. The drug patch is placed on the skin, and if the patient is infected with the microfilaria of "O. volvulus", localized pruritus and urticaria are seen at the application site.
A case of the Mazzotti reaction has been reported after presumptive treatment of schistosomiasis and strongyloidiasis with ivermectin, praziquantel and albendazole. The patient had complete resolution of symptoms after intravenous therapy with methylprednisolone.
Management of these partial-thickness wounds has been influenced by the Winter principle of moist wound healing, which suggests that wounds heal more rapidly in a moist environment. Hydrocolloid dressings applied directly to these wounds prevent the evaporation of moisture from the exposed dermis and create a moist environment at the wound site that promotes cell migration.
Mees' lines appear after an episode of poisoning with arsenic, thallium or other heavy metals, and can also appear if the subject is suffering from renal failure. They have been observed in chemotherapy patients.
Anonychia is the absence of nails, an anomaly, which may be the result of a congenital ectodermal defect, ichthyosis, severe infection, severe allergic contact dermatitis, self-inflicted trauma, Raynaud phenomenon, lichen planus, epidermolysis bullosa, or severe exfoliative diseases.
IgG4-related skin disease is the recommended name for skin manifestations in IgG4-related disease (IgG4-RD). Multiple different skin manifestations have been described.
This is rare and is usually due to mutations in the R-spondin 4 (RSPO4) gene which is located on the short arm of chromosome 20 (20p13). Clinically it is manifest by the absence (anonychia) or hypoplasia (hyponuchia) of finger- and/or toenails.
They are typically white bands traversing the width of the nail. As the nail grows they move towards the end, and finally disappear when trimmed.
Treatment is usually unnecessary. In severe cases, surgery with a bilateral levator excision and frontalis brow suspension may be used.
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.
The term "toxic abortion" was first used to identify this phenomenon in humans in the earliest studies of the effects of pollutants on pregnancy in 1928, "An Experimental Investigation Concerning Toxic Abortion Produced by Chemical Agents" by Morris M. Datnow M.D.
Toxic abortion chemicals studied at that time were:
Petrochemicals,
Heavy metals,
Organic solvents,
Tetrachloroethylene,
Glycol ethers,
2-Bromopropane,
Ethylene oxide,
Anesthetic gases, and
Antineoplastic drugs.
In 1932, the "Journal of State Medicine" reported on a natural variation, with the occurrence of "a considerable number of cases of toxic abortion" being caused by untreated dental caries.
Study of pollution-caused abortion in humans ceased for a considerable time, interest renewing in the 2000s. A 2009 study found that fossil fuels play a role, as "pregnant African-American women who live within a half mile of freeways and busy roads were three times more likely to have miscarriages than women who don't regularly breathe exhaust fumes." A 2011 study found a correlation between exposure to workplace toxins and spontaneous abortion, and called for further study. "Newsweek" magazine reported in May 2014 that a spike in stillborn babies in the town of Vernal, in Utah, had correlated with an increase in pollution from new gas and oil drilling. "Newsweek" reported that "Vernal’s rate of neonatal mortality appears to have climbed from about average in 2010 (relative to national figures) to six times the normal rate three years later." "Newsweek" quoted one expert's observation that "We know that pregnant women who breathe more air pollution have much higher rates of virtually every adverse pregnancy outcome that exists." A study published in the "Journal of Environmental Health" in October 2014 found tetrachloroethylene or PCE, to be "linked to increased risk for stillbirths and other pregnancy complications."
The PCE study found that "pregnancies with high exposure to PCE were 2.4 times more likely to end with stillborn babies and 1.4 times more likely to experience placental abruption — when the placenta peels away from uterine wall before delivery, causing the mother to bleed and the baby to lose oxygen — compared with pregnancies never exposed to PCE." Higher exposure lead to a 35 percent higher risk of abruption. PCE has also been tied to an increased risk for cancer. Children exposed to PCE as fetuses and toddlers are more likely to use drugs later in life. The toxin has been linked to mental illness, an increased risk of breast cancer and some birth defects. It has been tied to anxiety, depression, and impairments in cognition, memory and attention. PCE contamination has been found in the Massachusetts water supply and "on military bases across the country," and "water systems in California and Pennsylvania and have also been found to be contaminated with PCE."
In 2015, "Newsweek" reported that chemicals found in fast food wrappers multiply miscarriage risk by sixteen times.
Some instances have been reported of women intentionally seeking to induce toxic abortion, where circumstances make medical abortion difficult to obtain, by exposing themselves to environmental toxins.
Although treatment may be unnecessary, there may be social implications, especially in young children when venturing from a supportive home environment to a public environment (e.g., starting school). Continued support, including monitoring behavior and educating the child about his or her appearance as seen by others, is encouraged. Gradual or sudden withdrawal from interaction with others is a sign that may or may not be related to such behavior. Studies are being conducted to elucidate these implications.
Given the unknown nature of MES, treatments have been largely dependent on an individual basis. Treatments can vary from being as little as self-reassurance to pharmaceutical medications.
Medications can be helpful, such as antipsychotics, benzodiazepines or antiepileptics, but there is very limited evidence for this. Some case studies have found that switching to a prednisolone steroid after a betamethasone steroid which caused MES helped alleviate hallucinations or the use of the acetylcholinesterase inhibitor, Donepezil, have also found that it successfully treated an individual's MES. However, because of the heterogeneous etiology, these methods cannot be applied as general treatment.
Other than treatment by medicinal means, individuals have also successfully alleviated musical hallucinations by cochlear implants, listening to different songs via an external source, or by attempting to block them through mental effort, depending on how severe their condition is.
Scleromyositis or the PM/Scl overlap syndrome is a complex autoimmune disease (a disease in which the immune system attacks the body). Patients with scleromyositis have symptoms of both systemic scleroderma and either polymyositis or dermatomyositis, and is therefore considered an overlap syndrome. Although it is a rare disease, it is one of the more common overlap syndromes seen in scleroderma patients, together with MCTD and Antisynthetase syndrome. Autoantibodies often found in these patients are the anti-PM/Scl (anti-exosome) antibodies.
The symptoms that are seen most often are typical symptoms of the individual autoimmune diseases and include Raynaud's phenomenon, arthritis, myositis and scleroderma. Treatment of these patients is therefore strongly dependent on the exact symptoms with which a patient reports to a physician and is similar to treatment for the individual autoimmune disease, often involving either immunosuppressive or immunomodulating drugs.
- Clinical characteristics:
- Overlap Syndrome: scleroderma overlap syndrome
- Autoimmune disease
- Scleroderma myositis overlap syndrome
Phlegmasia cerulea dolens (literally: "painful blue edema") is an uncommon severe form of deep venous thrombosis which results from extensive thrombotic occlusion (blockage by a thrombus) of the major and the collateral veins of an extremity. It is characterized by sudden severe pain, swelling, cyanosis and edema of the affected limb. There is a high risk of massive pulmonary embolism, even under anticoagulation. Foot gangrene may also occur. An underlying malignancy is found in 50% of cases. Usually, it occurs in those afflicted by a life-threatening illness.
This phenomenon was discovered by Jonathan Towne, a vascular surgeon in Milwaukee, who was also the first to report the "white clot syndrome" (now called heparin induced thrombocytopenia [HIT]). Two of their HIT patients developed phlegmasia cerulea dolens that went on to become gangrenous.
Treatment by Catheter directed thrombolytic therapy.