1/42. Inflammatory myofibroblastic tumor of the skin.We report a case of inflammatory myofibroblastic tumor (IMF) of the skin in a female with a history of Wegeners granulomatosis. The patient had a painless, erythematous, and indurated lesion of the left elbow. The resected specimen revealed a 4 cm x 3 cm nodule involving the entire dermis and superficial portions of subcutis with a stellate profile at scanning magnification. There were spindle cells in fascicles and whorls and a mixed inflammatory cell infiltrate of plasma cells, lymphocytes, neutrophils, and eosinophils. The spindle cells were immunoreactive for vimentin, muscle specific actin, and smooth muscle actin. The polyclonal and polymorphous nature of the inflammatory cells was confirmed by immunohistochemical studies. This is the first case of IMF of the skin documented by immunostaining.- - - - - - - - - - ranking = 1keywords = granulomatosis (Clic here for more details about this article) |
2/42. Neutrophil dysfunction and granulomatosis in the preleukemic state.A 40-year-old male had periods of fever, sore throat and anemia for 14 months before acute myeloblastic leukemia could be diagnosed from hematological findings. During the preleukemic state, impaired bactericidal capacity of the granulocytes was repeatedly demonstrated and multiple hepatosplenic and skin granulomas occurred. Results of granulocyte function studies may prove to be of significant aid in the diagnosis of the preleukemic state of acute myeloblastic leukemia.- - - - - - - - - - ranking = 4keywords = granulomatosis (Clic here for more details about this article) |
3/42. lymphomatoid granulomatosis in a 4-year-old boy.lymphomatoid granulomatosis is a necrotizing angiocentric and angiodestructive infiltrative process involving primarily the lung, skin, central nervous system, and kidney. The incidence is highest in middle-aged men and is rare in children. We report a case of lymphomatoid granulomatosis involving both skin and lung in a 4-year-old boy. The disease progressed to peripheral T-cell lymphoma, which was unusual in light of recent evidence suggesting a B-cell origin in the majority of cases.- - - - - - - - - - ranking = 18.041430179862keywords = lymphomatoid granulomatosis, granulomatosis (Clic here for more details about this article) |
4/42. Wegener's granulomatosis: description of a case where cutaneous involvement was correlated with elevation of the c-ANCA titer.Wegener's granulomatosis is a systemic disease characterized by necrotizing granulomas and vasculitis involving the upper and lower respiratory tract as well as the kidneys. Cutaneous manifestations consist mainly of papules or papulonecrotic lesions. c-ANCA are known to be a valuable adjunct for the diagnosis and follow-up of Wegener's granulomatosis with systemic involvement. We report the case of a 49-year-old man with Wegener's granulomatosis who developed two relapses of the disease with cutaneous manifestation and who presented with concomitant elevation of the c-ANCA and more precisely the subset PR3-ANCA during the acute phase of the disease.- - - - - - - - - - ranking = 7keywords = granulomatosis (Clic here for more details about this article) |
5/42. Granulomatous lymphangitis of the scrotum and penis. Report of a case and review of the literature of genital swelling with sarcoidal granulomatous inflammation.BACKGROUND: Acquired lymphedema of the genitalia is a rare childhood presentation and is more common in elderly individuals secondary to pelvic/abdomenal malignancy or its therapy or worldwide due to filariasis. OBJECTIVE: Herein, we report a case of a healthy 11-year-old boy who presented with a 1-year history of chronic, asymptomatic scrotal and penile swelling. biopsy revealed edema, lymphangiectases and peri- and intralymphatic sarcoidal type granulomas. This histologic pattern of granulomatous lymphangitis is most commonly associated with orofacial granulomatosis (granulomatous cheilitis and melkersson-rosenthal syndrome) and Crohn's disease. Treatment with topical steroids and physical support has resulted in marked improvement. No systemic disease (Crohn's disease) is evident 1 year later. literature review revealed 44 cases of genital lymphedema with non-infectious granulomas. The majority of these young patients had Crohn's disease, frequently with anal involvement and a minority, both with and without Crohn's disease, had orofacial granulomatosis. CONCLUSIONS: Granulomatous lymphangitis should be considered in the differential diagnosis of chronic idiopathic swelling of the genitalia, particularly in younger individuals. Further clinical examination, additional laboratory studies and close follow-up for co-existing or subsequent development of Crohn's disease should be performed. The overlap between granulomatous lymphangitis of the genitalia, Crohn's disease and orofacial granulomatosis suggest that granulomatous lymphangitis of the genitalia may represent a forme fruste of Crohn's disease.- - - - - - - - - - ranking = 3keywords = granulomatosis (Clic here for more details about this article) |
6/42. Resolution of orofacial granulomatosis with amalgam removal.A 61-year-old woman presented with a 2-year history of an abnormal erythematous swelling on the upper lip and cheek. Upon examination there were no other physical findings. Histological examination found discreet sarcoidal granulomas in the lower dermis. Routine laboratory studies, chest radiographs and pulmonary functions were all normal. Clinical presentation and histological findings were, therefore, compatible with the diagnosis of orofacial granulomatosis (OFG). The patient was patch tested with an extended standard series that included metal-salt, dental prosthesis, bakery and corticosteroids series. The patch test was positive (score ) after 48 and 72 h for mercury in the metal-salt and dental prosthesis series. During the past decade the patient had received amalgam fillings of several dental cavities, including one adjacent to the swollen cheek. The unilateral localization of the soft tissue swelling adjacent to the amalgam tooth fillings, along with the positive patch test for mercury, raised the possibility that the OFG was part of a delayed hypersensitive reaction to the fillings. The patient therefore underwent a total amalgam replacement procedure; complete disappearance of the swelling overlying the right cheek was observed within 7 weeks and the swelling of the upper lip subsided completely within 6 months. We propose that mercury in amalgam tooth fillings is another cause of OFG and suggest appropriate patch testing in patients who do not have an apparent cause of OFG.- - - - - - - - - - ranking = 5keywords = granulomatosis (Clic here for more details about this article) |
7/42. Isolated EBV lymphoproliferative disease in a child with wiskott-aldrich syndrome manifesting as cutaneous lymphomatoid granulomatosis and responsive to anti-CD20 immunotherapy.patients with primary immunodeficiencies such as the wiskott-aldrich syndrome (WAS) are prone to develop Epstein-Barr virus (EBV) related lymphoproliferative disorders (LPDs). EBV LPD is most frequently seen in patients receiving immunosuppressive treatment after organ transplantation (post-transplant lymphoproliferative disorder), but can also arise in the primary immunodeficiencies. Typically, EBV LPD presents as a diffuse systemic disease with lymphadenopathy and organ involvement. A rare angiocentric and angiodestructive form of EBV associated B cell LPD, lymphomatoid granulomatosis (LyG), has also been reported in association with WAS. LyG most commonly involves the lung, but can also be seen in brain, kidney, liver, and skin. This report describes the case of a 16 year old boy with WAS who presented with an isolated non-healing ulcerating skin lesion. biopsy revealed an EBV related LPD with the histological features of LyG. This cutaneous lesion responded dramatically to treatment with specific anti-CD20 immunotherapy and the patient remains clinically free of LPD at 18 months.- - - - - - - - - - ranking = 65.20715089931keywords = lymphomatoid granulomatosis, granulomatosis (Clic here for more details about this article) |
8/42. Fine needle aspiration diagnosis of lymphomatoid granulomatosis. A case report.The diagnosis of lymphomatoid granulomatosis (LG) of the lung depends on obtaining adequate histologic material to demonstrate the characteristic angioinvasive, polymorphous, lymphoid infiltrate and normally requires an open lung biopsy. Fine needle aspiration biopsy (FNAB), if only smeared directly, does not allow an assessment of the lymphoid infiltrate in relation to blood vessels. However, we report a case diagnosed by FNAB in which the specimen was processed by an alternative method that allows cell blocks to be made from all visible particles. Percutaneous FNAB of a nodular pulmonary infiltrate was performed after bronchoscopy and transbronchial needle biopsy failed to yield a diagnosis. The FNAB specimen was placed in 50% alcohol and submitted for processing. The specimen was then filtered through a fine sieve, and all visible tissue was embedded in bacteriologic agar and processed as a standard surgical specimen. The filtrate was processed as standard fluid cytology. The atypical, angioinvasive, lymphoid infiltrate was clearly demonstrated on the cell blocks, and the diagnosis of LG was made. This diagnosis was confirmed by subsequent open lung and skin biopsies.- - - - - - - - - - ranking = 65.20715089931keywords = lymphomatoid granulomatosis, granulomatosis (Clic here for more details about this article) |
9/42. Nasal Wegener's and skin sarcoid--a rare combination of two granulomatous diseases.Wegener's granulomatosis (WG) and sarcoidosis are two distinct granulomatous diseases characterized by multisystem involvement. We report a patient who initially presented with symptoms of limited WG predominantly affecting the nose, followed by a facial rash, which was histologically proven to be due to sarcoidosis. The sequential development of these two diseases in one patient is very rare, and to our knowledge, only one such case has been reported in the last 50 years (Am J kidney Dis 28:893-898, 1996).- - - - - - - - - - ranking = 1keywords = granulomatosis (Clic here for more details about this article) |
10/42. Combined CNS and pituitary involvement as a primary manifestation of wegener granulomatosis.wegener granulomatosis (WG) is a systemic vasculitis of small and medium vessels. It predominantly affects the upper and/or lower respiratory airway and kidneys. Its pathogenesis is not fully understood. WG relatively frequently affects the nervous system (in 30-50% according to the different studies). Most frequently, it manifests as necrotizing vasculitis that leads to the peripheral neuropathies or to the cranial nerves palsy. Impairment of the central nervous system (CNS) is less frequent and occurs in 2-8% of patients. Three major pathogenetic mechanisms were described: CNS vasculitis, spreading of granulomas from the adjacent anatomical areas (paranasal cavities, orbit etc.), and new formation of granulomas in brain tissue. This case report describes patients in whom WG manifested in the form of localized skin involvement and combined CNS involvement that included pituitary gland. Atypical presentation of WG impedes and slows down the process of diagnosis and emphasizes the need for collaboration between medical specialists.- - - - - - - - - - ranking = 5keywords = granulomatosis (Clic here for more details about this article) |
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