Monday, January 10, 2011

Malacoplakie

Malacoplakie Malacoplakia is an inflammatory condition that presents as a plaque or a nodule usually affecting the genitourinary tract and skin. Microscopically it is characterized by the presence histiocitelor sparkling with distinct basophilia inclusions known as Michaelis-Gutmann corpus. Malacoplakia skin is uncommon but present in immunocompromised patients with defective macrophage function. Lesions are yellow-pink papules, but may present as nodule or ulcerated and are diagnosed only after biopsy.
Malacoplakia is resistant to treatment, with an average duration of skin lesions of 3-6 months. Mortality in these patients is due to the pathological condition of the fund. Significant morbidity is related to chronicity condition that resist local and systemic therapy. Purulent sinuses, persistent disfiguring skin lesions and internal organ involvement is important morbidity in patients with malacoplakie.
Includes antibiotic therapy that focuses macrophage. Vitamin C is used as a strategy for effective therapy. To treat malacoplakia is necessary to discontinue immunosuppressive therapy. Against infection with E. coli indicate lesion excision surgery and antibiotic therapy. Malacoplakia has a benign evolution.
Pathogenesis and causes
Malacoplakia result is considered inadequate destruction of bacteria by macrophage and monocyte activity fagolizozomala deficient. Partially digested bacteria accumulate in these cells and lead to calcium and iron storage in the residual bacterial glycolipids. This is considered pathognomonic inclusions malacoplakie basophilia. Bacteria that can not be totally destroyed include Escherichia coli enteric bacteria, Staphylococcus aureus, Pseudomonas aeruginosa and Rhodococcus. Risk factors for development malacoplakiei include: -Prolonged systemic steroid therapy, organ transplantation -Diabetes mellitus, lymphoma, rheumatoid arthritis.
Signs and symptoms
Malacoplakia skin presents to patients with a wide age limit. Mean age at the time of presentation is 53 years. The incidence of this disease is associated with an immunocompromised affecting monocytes and macrophages. Typically patients have a history of immunosuppression in renal transplantation, diabetes, lymphoma or a history of long-term therapy with systemic corticosteroids. A quarter of patients have internal organ damage, most of the retroperitoneal area, kidney, bladder or colonel. Show affecting systemic symptoms, but patients have purulent sinus originating from the attachment and deep organs. Malacoplakia rarely presents in patients with HIV infection and AIDS. May mimic colonic carcinoma, gastric, pancreatic, orofarinfian. Bone and lung involvement causing difficulty of diagnosis.
Physical examination. The lesions present as papules, plaques or ulcers or yellow-pink color. Can this suppurations. Common localizations are anal or groin area, thighs and abdominal wall. Abscesses / suppurate sinuses are reported near the urethra, vulva and anus. The lesions may be misdiagnosed as lymphoma, neoplasms, abscesses. This can occur fluctuenta Solitary nodule or as a soft or a group of papules. Common injuries are chronic but not debilitating for the patient.
Diagnosis
The imaging studies using positron emission tomography or magnetic resonance to demonstrate malacoplakia in internal damage.
Histological examination Show histiocite bodied sparkling Gutmann formed in lysosomes filled with partially digested bacteria. The differential diagnosis is made with the following conditions: actinomicoza, sarcoidosis, squamous cellular carcinoma, histiocytosis X, malignant lymphoma, histiocitom, skin abscesses, botriomicoza.
Treatment
Drug therapy is indicated that focuses macrophages: quinolones, Trimethoprim-sulfametoxazole) associated with high rate of efficiency. Antibiotic therapy against Escherichiei coli in combination with surgery allow the highest recovery. Betanecholul, a cholinergic agonist is useful in combination with antibiotics and surgery. It may correct loss believed to interfere with cGMP-total destruction of the bacteria. Ascorbic acid is used to increase cGMP in monocytes and AMPc a therapy effective strategy. Discontinuation of immunosuppressive therapy is needed to effectively treat malacoplakia.

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