Chronic granulomatous disease:
It is a genetic disorder that affects the function of bacterial killers. Characterized by the presence of granular lesions in some tissues, short stature, and this is a dominant clinical appearance in children and adults with chronic granular disease.
The recovery rate is varied but improvement occurs in about 50% of patients. When the symptoms start after the baby's age, it bodes well.
Pathological Anatomy:
In chronic granuloma, macrophages can naturally swallow germs but can not kill them later. There is also the same abnormality in eosinophils and mononucleotides. The survival of germs in the cells leads to the occurrence of granuloma in the lymph nodes, skin, lungs, liver, gastrointestinal tract and bones.
Clinical manifestations:
- Psoriasis is the most common and most common.
- Sensation in the areas around the mouth and around the openings of the nose, ears, mouth and eyes.
- nodular lesions and necrotizing ulcers.
- Subcutaneous nodules may occur where the vaccine and sometimes tend to ulcer.
- Bad wound healing and bleeding nodular skin lesions are very characteristic and frequent and regular appearance.
- Thrombocytopenia in the superficial lymph nodes.
Other symptoms include:
- Chronic pyelonephritis.
- Scalp infection.
- Ulcerative inflammation of the mouth.
- Infections occur in other organs especially the lungs, visceral lymph nodes, liver and bones.
- Controlling pulmonary disease, recurrent pneumonia, capillaries, lung abscesses.
- Gastrointestinal manifestations include: malabsorption abscesses around the anus, fistulas and oral ulcers. Distinctive manifestations are obstructive lesions associated with myocardial infarction.
Blood image:
- Peripheral leukocytosis is characteristic, and reflects an increase in the number of circulating neutrophils.
- Hypoproteinemia minus pigment minus cells.
- Levels of the three classes of immunoglobulin are increased with IgE plus or normal level.
Treatment:
- Skin care.
- Hyperthermia should be investigated carefully to show the location of infection and responsible organisms.
Antibiotics should be carefully selected and treatment should be concentrated.
- Anticonvulsant therapy, bactrim, septrin fort, or trimethoprim alone, is the standard treatment line now.
- Continuous treatment with antifungal agents such as ketoconazole does not appear to be effective in the prevention of aspergillus but it is hoped that itraconazole gives benefit under evaluation and proved to be very effective.
- Interferon subcutaneous.
Gamaglobulin may have good results.
Blood transfusion may be required to treat anemia.
- systemic corticosteroids for systemic intestinal obstruction.
Bone marrow transplant.
It is a genetic disorder that affects the function of bacterial killers. Characterized by the presence of granular lesions in some tissues, short stature, and this is a dominant clinical appearance in children and adults with chronic granular disease.
The recovery rate is varied but improvement occurs in about 50% of patients. When the symptoms start after the baby's age, it bodes well.
Pathological Anatomy:
In chronic granuloma, macrophages can naturally swallow germs but can not kill them later. There is also the same abnormality in eosinophils and mononucleotides. The survival of germs in the cells leads to the occurrence of granuloma in the lymph nodes, skin, lungs, liver, gastrointestinal tract and bones.
Clinical manifestations:
- Psoriasis is the most common and most common.
- Sensation in the areas around the mouth and around the openings of the nose, ears, mouth and eyes.
- nodular lesions and necrotizing ulcers.
- Subcutaneous nodules may occur where the vaccine and sometimes tend to ulcer.
- Bad wound healing and bleeding nodular skin lesions are very characteristic and frequent and regular appearance.
- Thrombocytopenia in the superficial lymph nodes.
Other symptoms include:
- Chronic pyelonephritis.
- Scalp infection.
- Ulcerative inflammation of the mouth.
- Infections occur in other organs especially the lungs, visceral lymph nodes, liver and bones.
- Controlling pulmonary disease, recurrent pneumonia, capillaries, lung abscesses.
- Gastrointestinal manifestations include: malabsorption abscesses around the anus, fistulas and oral ulcers. Distinctive manifestations are obstructive lesions associated with myocardial infarction.
Blood image:
- Peripheral leukocytosis is characteristic, and reflects an increase in the number of circulating neutrophils.
- Hypoproteinemia minus pigment minus cells.
- Levels of the three classes of immunoglobulin are increased with IgE plus or normal level.
Treatment:
- Skin care.
- Hyperthermia should be investigated carefully to show the location of infection and responsible organisms.
Antibiotics should be carefully selected and treatment should be concentrated.
- Anticonvulsant therapy, bactrim, septrin fort, or trimethoprim alone, is the standard treatment line now.
- Continuous treatment with antifungal agents such as ketoconazole does not appear to be effective in the prevention of aspergillus but it is hoped that itraconazole gives benefit under evaluation and proved to be very effective.
- Interferon subcutaneous.
Gamaglobulin may have good results.
Blood transfusion may be required to treat anemia.
- systemic corticosteroids for systemic intestinal obstruction.
Bone marrow transplant.
Labels
skin diseases