Von Recklen Hausen's disease:
Congenital dysplasia is characterized by manifestations of skin, nervous, muscle and bone.
Clinical manifestations:
Skin manifestations:
The distinctive skin features include coffee au lait, which are flat and rounded patches with dark brown spots. When they are six or more in size 1.5 cm 2 diameter at least, the diagnosis of fibroblastoma is usually confirmed.
Coffee spots with milk are the first manifestations of the disease in all children. Other manifestations of the disease include: feverish hair, increased pigmentation in bronze color, hypotension, and hair loss on the lower back.
Many fibroblasts occur along the surrounding nerves as well as soft bags (which distinguish them from the young pills that are harsh and other distinctive manifestations) and embraced, most of them on the trunk and extremities and may exist in the hundreds. Ranging from several millimeters to several centimeters in diameter.
The other type of disease is characterized by the occurrence of an acoustic neuroma on the sides usually in addition to tumors of meningitis or other tumors in the nervous system.
The manifestations of mucous membranes:
Oral lesions are found in 5-10% of cases, such as papillary tumors in the palate, buccal, mucous membranes of the tooth and lips or, like the tongue, usually unilateral.
In-kind manifestations:
"Les patches" (pigmented Hamartoma pigmented melanocytes) appear conical lesions in the dome, superficial around the iris and can be observed by checking the lamp healing.
Itching: Itching may be the symptom of fibroblastoma, the presence of a large number of primary cells in the skin in this case. The itching response to antihistamines raises the belief that histamine is the cause of itching.
Inflammatory fibroblastoma: "Elephantiasis" is a fibrous neuropathy spread on the nerves of the trunk, accompanied by hyperglycemia. The skin becomes wrinkled and relaxed and may cause severe deformation.
Internal features:
Neurofibromatosis may also affect the viscera and blood vessels.
Neural manifestations:
An intracranial tumor in the ocular nerve area may occur in many cases in addition to the type Astrosaitoma and Chuanoma.
In addition to the symptoms based on the location of the tumor, there are other symptoms may be the result of high pressure inside the skull, such as: headache vomiting, cramping and seizures.
Cancers may also affect peripheral nerves and spinal nerves.
Sarcoma may accompany some cases as well as leukemia and kidney cancers.
Path of the disease:
The disease is of the chronic type where the spots appear in the color of milk coffee early in childhood and also show early neuropathy gradually increasing number. The appearance of such symptoms at an early age suggests a poor progression and end to the disease.
Gastrointestinal, renal and CNS infections are also serious complications. In rare cases, however, the disease may lead to death.
Pregnancy exacerbates the disease as it becomes more prevalent as well as severe headaches that may worry the patient.
Diagnosis methods:
Disease can be diagnosed without difficulty with clinical and anatomical phenomena. If there are six patches of skin and milk color or more milk, these give an indication to confirm the diagnosis. Associated conditions such as seizures may also help.
All affected patients should be screened, such as IQ testing, electroencephalogram, hearing planning, bottom-of-the-eye examinations, and cochlear imaging especially for the spine.
All persons from relatives as well as those of the first order so that they did not show symptoms should undergo a thorough genetic examination.
Methods of Treatment:
- Treatment of symptoms.
- Remove the deformities surgically.
- We used the type CO2 laser in removing the compass and some of the deformities associated with the disease and gave encouraging results.
Congenital dysplasia is characterized by manifestations of skin, nervous, muscle and bone.
Clinical manifestations:
Skin manifestations:
The distinctive skin features include coffee au lait, which are flat and rounded patches with dark brown spots. When they are six or more in size 1.5 cm 2 diameter at least, the diagnosis of fibroblastoma is usually confirmed.
Coffee spots with milk are the first manifestations of the disease in all children. Other manifestations of the disease include: feverish hair, increased pigmentation in bronze color, hypotension, and hair loss on the lower back.
Many fibroblasts occur along the surrounding nerves as well as soft bags (which distinguish them from the young pills that are harsh and other distinctive manifestations) and embraced, most of them on the trunk and extremities and may exist in the hundreds. Ranging from several millimeters to several centimeters in diameter.
The other type of disease is characterized by the occurrence of an acoustic neuroma on the sides usually in addition to tumors of meningitis or other tumors in the nervous system.
The manifestations of mucous membranes:
Oral lesions are found in 5-10% of cases, such as papillary tumors in the palate, buccal, mucous membranes of the tooth and lips or, like the tongue, usually unilateral.
In-kind manifestations:
"Les patches" (pigmented Hamartoma pigmented melanocytes) appear conical lesions in the dome, superficial around the iris and can be observed by checking the lamp healing.
Itching: Itching may be the symptom of fibroblastoma, the presence of a large number of primary cells in the skin in this case. The itching response to antihistamines raises the belief that histamine is the cause of itching.
Inflammatory fibroblastoma: "Elephantiasis" is a fibrous neuropathy spread on the nerves of the trunk, accompanied by hyperglycemia. The skin becomes wrinkled and relaxed and may cause severe deformation.
Internal features:
Neurofibromatosis may also affect the viscera and blood vessels.
Neural manifestations:
An intracranial tumor in the ocular nerve area may occur in many cases in addition to the type Astrosaitoma and Chuanoma.
In addition to the symptoms based on the location of the tumor, there are other symptoms may be the result of high pressure inside the skull, such as: headache vomiting, cramping and seizures.
Cancers may also affect peripheral nerves and spinal nerves.
Sarcoma may accompany some cases as well as leukemia and kidney cancers.
Path of the disease:
The disease is of the chronic type where the spots appear in the color of milk coffee early in childhood and also show early neuropathy gradually increasing number. The appearance of such symptoms at an early age suggests a poor progression and end to the disease.
Gastrointestinal, renal and CNS infections are also serious complications. In rare cases, however, the disease may lead to death.
Pregnancy exacerbates the disease as it becomes more prevalent as well as severe headaches that may worry the patient.
Diagnosis methods:
Disease can be diagnosed without difficulty with clinical and anatomical phenomena. If there are six patches of skin and milk color or more milk, these give an indication to confirm the diagnosis. Associated conditions such as seizures may also help.
All affected patients should be screened, such as IQ testing, electroencephalogram, hearing planning, bottom-of-the-eye examinations, and cochlear imaging especially for the spine.
All persons from relatives as well as those of the first order so that they did not show symptoms should undergo a thorough genetic examination.
Methods of Treatment:
- Treatment of symptoms.
- Remove the deformities surgically.
- We used the type CO2 laser in removing the compass and some of the deformities associated with the disease and gave encouraging results.