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It is usually asymptomatic unless the complication and infection is severe. But in some recorded cases, symptoms include nasopharyngitis accompanied by pain, itching of throat and ears. Coughing, hemoptysis and vomiting are verifiable indications as well as sneezing, bleeding, dyspnea, and inflammation.
Eating raw or semi-cooked infected liver or lymph nodes infected with nymphal L. serrata causes severe symptoms in the human nasopharynx. Submaxillary and cervical lymph nodes sometimes enlarge and the neck is swollen. Complications include abscesses in the auditory canals, facial paralysis, and enlarged tonsils producing asphyxiation. These symptoms are well recognized as a disease call “halzoun syndrome” in Lebanon and nearby countries.
In Egypt, infected camels and buffalo may also be a source of infection for dogs, which are companions of man in desert and semi-desert areas where grazing is a major profession, and in villages, where dogs are also common. Infected dogs, in turn, are a source of infection to man who may be an intermediate host.
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.
Paraneoplastic acrokeratosis, Bazex syndrome (also known as acrokeratosis paraneoplastica of Bazex and acrokeratosis neoplastica) is a cutaneous condition characterized by psoriasiform changes of hands, feet, ears, and nose, with involvement of the nails and periungual tissues being characteristic and indistinguishable from psoriatic nails. The condition is associated with carcinomas of the upper aerodigestive tract.
This condition should not be confused with the other unrelated disease called Bazex syndrome, otherwise referred to as Bazex-Dupre-Christol syndrome.
This is a subset of cases of congenital syphilis. Newborns may be asymptomatic and are only identified on routine prenatal screening. If not identified and treated, these newborns develop poor feeding and runny nose. By definition, early congenital syphilis occurs in children between 0 and 2 years old. After, they can develop late congenital syphilis.
Symptomatic newborns, if not stillborn, are born premature, with an enlarged liver and spleen, skeletal abnormalities, pneumonia and a bullous skin disease known as pemphigus syphiliticus.
The rabbit ear mite, "Psoroptes cuniculi", is larger than "Otodectes cynotis". It causes thick firm debris to form in the ear canal, and can eventually migrate to the skin of the outer ear and face. Symptoms include scratching and shaking of the head. Treatment includes topical selamectin, or injections of ivermectin and frequent cleanings of the rabbit's environment.
The ear mite is the most common cause of ear infections in cats, quickly spreading from one cat to another through direct contact. Ear mites cause inflammatory symptoms, similar to bacterial and yeast infections. Symptoms include itching and redness of the ears. Other, more serious problems can result from untreated infections, such as skin disease in areas other than the ear like the neck and tail, and deafness.
Symptoms include skin irritations, which may be itchy or painful, and are sometimes confused with hives. These irritations appear upon or shortly after exposure to sunlight, and may last from 1 to 7 days. Lesions have been photographed showing fluid-filled blisters.
Secondary symptoms include flu-like symptoms; body aches, skin sensitivity, muscle aches, fever, dizziness, exhaustion, vision impairment, and disorientation.
Benign cephalic histiocytosis (also known as "Histiocytosis with intracytoplasmic worm-like bodies", and not to be confused with "Neonatal cephalic pustulosis") is a rare cutaneous condition affecting boys and girls equally, characterized by skin lesions that initially present on the head in all cases, often the cheeks, eyelids, forehead, and ears.
Angiolymphoid hyperplasia with eosinophilia (also known as: "Epithelioid hemangioma," "Histiocytoid hemangioma," "Inflammatory angiomatous nodule," "Intravenous atypical vascular proliferation," "Papular angioplasia," "Inflammatory arteriovenous hemangioma," and "Pseudopyogenic granuloma") usually presents with pink to red-brown, dome-shaped, dermal papules or nodules of the head or neck, especially about the ears and on the scalp.
It, or a similar lesion, has been suggested as a feature of IgG4-related skin disease, which is the name used for skin manifestations of IgG4-related disease.
Newborns with harlequin-type ichthyosis present with thick, fissured armor-plate hyperkeratosis. Sufferers feature severe cranial and facial deformities. The ears may be very poorly developed or absent entirely, as may the nose. The eyelids may be everted (ectropion), which leaves the eyes and the area around them very susceptible to infection. Babies with this condition often bleed during birth. The lips are pulled back by the dry skin (eclabium). Joints are sometimes lacking in movement, and may be below the normal size. Hypoplasia is sometimes found in the fingers. Polydactyly has also been found on occasion. In addition, the fish mouth appearance, mouth breathing, and xerostomia place affected individuals at extremely high risk for developing rampant dental decay.
Patients with this condition are extremely sensitive to changes in temperature due to their hard cracked skin, which prevents normal heat loss. Respiration is also restricted by the skin, which impedes the chest wall from expanding and drawing in enough air. This can lead to hypoventilation and respiratory failure. Patients are often dehydrated, as their plated skin is not well suited to retaining water.
TBS patients may have the following symptoms:
- Abnormalities of the external ears (unusually large or small, unusually shaped, sometimes with sensorineural hearing loss or deafness due to lesions or dysfunctions of part of the internal ear or its nerve tracts and centers or conductive hearing loss from the external or middle ear), dysplastic ears, lop ear (over-folded ear helix), preauricular tags or pits (a rudimentary tag of ear tissue typically located just in front of the ear).
- Anorectal malformations, including imperforate anus/absence of an anal opening, rectovaginal fistula, anal stenosis, unusually placed anus.
- Renal abnormalities, sometimes leading to impaired renal function or renal failure, including hypoplastic kidneys (underdeveloped), multicystic kidneys, dyspastic kidneys.
- Heart abnormalities, including tetralogy of fallot and defects of the ventricular septum.
- Hand and foot abnormalities, such as hypoplastic thumbs, fingerlike thumbs, syndactyly (webbed fingers/toes), fusion of the wrist bones, overlapping foot and/or toe bones.
Learning difficulties have been reported in some children with TBS. For others, intelligence is within the normal range.
These abnormalities, which are present prenatally, can range from minor to severe, and as with similar disorders, most individuals with this condition have some, but not all, of these traits.
The key affected features of this condition are described in its name.
Scalp: There are raised nodules over the posterior aspect of the scalp, covered by scarred non-hair bearing skin.
Ears: The shape of the pinnae is abnormal, with the superior edge of the pinna being turned over more than usual. The size of the tragus, antitragus and lobule may be small.
Nipples: The nipples are absent or rudimentary. The breasts may be small or virtually absent.
Other features of the condition include:
Dental abnormalities: missing or widely spaced teeth
Syndactyly: toes or fingers may be partially joined proximally
Renal abnormalities: renal hypoplasia, pyeloureteral duplication
Eye abnormalities: Cataract, coloboma of the iris and asymmetric pupils.
The disease occurs much more in males than females (due to the X-linked recessive pattern of inheritance) and is estimated to occur in between 1 and 10 males per million. The first signs of WAS are usually petechiae and bruising, resulting from a low platelet count. Spontaneous nose bleeds and bloody diarrhea are common. Eczema develops within the first month of life. Recurrent bacterial infections develop by three months. Enlargement of the spleen is not an uncommon finding. The majority of WAS children develop at least one autoimmune disorder, and cancers (mainly lymphoma and leukemia) develop in up to a third of patients. Immunoglobulin M (IgM) levels are reduced, IgA and IgE are elevated, and IgG levels can be normal, reduced, or elevated. In addition to low blood platelet counts (i.e. thrombocytopenia), ~30% of afflicted individuals exhibit eosinophilia, i.e. high blood eosinophil counts.
Chilblain lupus erythematosus (also known as "chilblain lupus erythematosus of Hutchinson") is a chronic, unremitting form of lupus erythematosus with the fingertips, rims of ears, calves, and heels affected, especially in women.
Most of the signs of MWS are present during the neonatal period. The most common signs at this state are multiple congenital joint contractures, dysmorphic features with mask-like face, blepharophimosis, ptosis, micrognathia, cleft or high arched palate, low-set ears, arachnodactyly, chest deformation as pectus, kyphoscoliosis and absent deep tendon reflexes are frequent minor malformations have also been described and consist of renal anomalies, cardiovascular abnormalities, hypospadias, omphalomesenteric duct, hypertriphic pyloric stenosis, duodenal bands, hyoplastic right lower lobe of the lung, displacement of the larynx to the right and vertebral abnormalities, cerebral malformations.
- 75% of children with MWS have blepharophimosis, small mouth, micrognathia, kyphosis/scoliosis, radio ulnar synostose and multiple contractures.
- They have severe developmental delay; congenital joint contractures and blepharophimosis should be present in every patient
- 2 out of 3 of the following signs should be manifested: post natal growth, mask-like faces, retardation, and decreased muscular mass.
- Some may require additional signs such as; micrognathia, high arched or cleft palate, low set ears, kyphoscoliosis.
- The symptoms of MWS are normally diagnosed during the newborn period
The natural history of MWS is not well known: many patients died in infancy and clinical follow-up has been reported in few surviving adults. However, diagnosis may be more difficult to establish in adults patients, such as: blepharophimosis, contractures, growth retardation, and developmental delay, whereas minor face anomalies are less noticeable as the patient grows older. Throughout the development of the patient from young child to older adult changes the behavior drastically, from kindness to restless and hyperactive to aggressive.
Two subtypes have been described:
- "Juvenile spring eruption" is a cutaneous condition that affected the helices of the ears, particularly in boys because their ears are relatively more exposed to sunlight.
- "Benign summer light eruption" is a cutaneous condition, and a name used in continental Europe, and particularly France, to describe a clinically short-lived, itchy, papular eruption particularly affecting young women after several hours of sunbathing at the beginning of summer or on sunny vacations. After a person experiences this condition once, it will likely recur annually. Onset is generally in the teen years or 20s; the condition can then last the remainder of a person's life, with annual flare-ups after the first exposure to the sun each year.
Photosensitivity is also found in some of the Porphyrias. Nearly all cases of Porphyria cutanea tarda exhibit blister formation on the skin within 2–4 days of light exposure. Variegate porphyria and Hereditary coproporphyria can also exhibit symptoms of light induced blisters.
Purpura is a condition of red or purple discolored spots on the skin that do not blanch on applying pressure. The spots are caused by bleeding underneath the skin usually secondary to vasculitis or dietary deficiency of vitamin C (scurvy). They measure 0.3–1 cm (3–10 mm), whereas petechiae measure less than 3 mm, and ecchymoses greater than 1 cm.
Purpura is common with typhus and can be present with meningitis caused by meningococci or septicaemia. In particular, meningococcus ("Neisseria meningitidis"), a Gram-negative diplococcus organism, releases endotoxin when it lyses. Endotoxin activates the Hageman factor (clotting factor XII), which causes disseminated intravascular coagulation (DIC). The DIC is what appears as a rash on the affected individual.
Purpura are a common and nonspecific medical sign; however, the underlying mechanism commonly involves one of:
- Platelet disorders (thrombocytopenic purpura)
- Primary thrombocytopenic purpura
- Secondary thrombocytopenic purpura
- Post-transfusion purpura
- Vascular disorders (nonthrombocytopenic purpura)
- Microvascular injury, as seen in senile (old age) purpura, when blood vessels are more easily damaged
- Hypertensive states
- Deficient vascular support
- Vasculitis, as in the case of Henoch–Schönlein purpura
- Coagulation disorders
- Disseminated intravascular coagulation (DIC)
- Scurvy (vitamin C deficiency) - defect in collagen synthesis due to lack of hydroxylation of procollagen results in weakened capillary walls and cells
- Meningococcemia
- Cocaine use with concomitant use of the one-time chemotherapy drug and now veterinary deworming agent levamisole can cause purpura of the ears, face, trunk, or extremities, sometimes needing reconstructive surgery. Levamisole is purportedly a common cutting agent.
- Decomposition of blood vessels including purpura is a symptom of acute radiation poisoning in excess of 2 Grays of radiation exposure. This is an uncommon cause in general, but is commonly seen in victims of nuclear disaster.
Cases of psychogenic purpura are also described in the medical literature, some claimed to be due to "autoerythrocyte sensitization". Other studies suggest the local (cutaneous) activity of tissue plasminogen activator can be increased in psychogenic purpura, leading to substantial amounts of localized plasmin activity, rapid degradation of fibrin clots, and resultant bleeding. Petechial rash is also characteristic of a rickettsial infection.
Cauliflower ear is an irreversible condition that occurs when the external portion of the ear is hit and develops a blood clot or other collection of fluid under the perichondrium. This separates the cartilage from the overlying perichondrium that supplies its nutrients, causing it to die and resulting in the formation of fibrous tissue in the overlying skin. As a result, the outer ear becomes permanently swollen and deformed, resembling a cauliflower.
The condition is common in martial arts such as boxing, mixed martial arts or wrestling, and in full-contact sports such as rugby union football.
Harlequin-type ichthyosis is a genetic disorder which results in thickened skin over nearly the entire body at birth. The skin forms large, diamond-shaped plates that are separated by deep cracks. They affect the shape of the eyelids, nose, mouth, and ears, and limit movement of the arms and legs. Restricted movement of the chest can lead to breathing difficulties. These plates fall off over weeks. Other complications can include premature birth, infection, problems with body temperature, and dehydration.
Harlequin-type ichthyosis is due to mutations of the "ABCA12" genes. It is inherited from a person's parents in an autosomal recessive manner. Diagnosis is often based on appearance at birth and confirmed by genetic testing. Before birth amniocentesis or ultrasound may support the diagnosis.
There is no cure. Early in life constant supportive care is typically required. Treatments may include moisturizing cream, antibiotics, etretinate, or retinoids. It affects about 1 per 300,000 births. Both sexes are affected equally commonly. Long term problems are common. Death in the first month is relatively common. The condition was first documented in 1750.
Lachiewicz–Sibley syndrome is a rare autosomal dominant disorder characterized by preauricular pits and renal disease. Persons with this disease may have hypoplasic kidneys or proteinuria. This disease was first described in a Caucasian family of British and Irish descent that emigrated to Ohio in the 19th century before settling in Nebraska. Many of the members of this family still live in Nebraska, although the relatives are now scattered throughout the country.
Unlike branchio-oto-renal (BOR) syndrome, Lachiewicz–Sibley syndrome is characterized by only preauricular pitting and renal disease. Persons with BOR syndrome also present with hearing loss, branchial fistulas or cysts, malformed ears, and lacrimal stenosis. Other anomalies in BOR syndrome may include a long narrow face, a deep overbite, and facial paralysis.
It was characterized in 1985.
The cause is bleeding within the external portion of the ear, a condition known as hematoma auris, perichondrial hematoma, or traumatic auricular hematoma.
Weathering nodules of ear is a cutaneous condition seen on the helices of the ears of Caucasian men who have a history of significant cumulative sun exposure.