1/72. Cutaneous granulomatous lesions in common variable immunodeficiency: complete resolution after intravenous immunoglobulins.A 64-year-old man with common variable immunodeficiency developed a persistent papulonodular ulcerative eruption on the right leg. Histopathological examination disclosed a chronic inflammatory infiltrate with central necrosis and palisading granuloma. Repeated microbiological (bacteriological, mycological and mycobacteriological) studies failed to isolate any microorganism. After treatment with intravenous immunoglobulins, a progressive resolution of the skin lesions was observed with a complete clearing after 10 months. Clinicopathological features and therapeutic approaches of sterile granulomatous lesions associated with primary immunodeficiencies are reviewed.- - - - - - - - - - ranking = 1keywords = immunodeficiency (Clic here for more details about this article) |
2/72. Organizing pneumonia related to common variable immunodeficiency. case report and literature review.A 68-year-old woman suffering from common variable immunodeficiency (CVI) developed a typical picture of organizing pneumonia. Causative factors other than CVI were eliminated. Several antibiotic regimens failed to improve the patient's condition, while the clinical manifestations rapidly disappeared under steroid therapy, with complete radiological recovery, but relapsed after steroid withdrawal. Finally, organizing pneumonia was definitely demonstrated by pathological findings obtained by open lung biopsy. Interestingly, pathological examination exhibited two other well-known CVI-associated lesions, i.e. benign lymphoid hyperplasia and noncaseating granuloma. In view of reports in the literature, we speculate that these different histological patterns could have resulted in a spectrum of symptomatic CVI-associated pulmonary disorders that improved under steroid therapy.- - - - - - - - - - ranking = 1keywords = immunodeficiency (Clic here for more details about this article) |
3/72. Parkinsonism in a patient with AIDS and cerebral opportunistic granulomatous lesions.The localization of opportunistic infections in the basal ganglia in patients with acquired immunodeficiency syndrome (AIDS) can cause movement disorders, such as choreoathetosis, dystonia, hemiballism and, more rarely, parkinsonism. We describe the case of an AIDS patient who developed cerebral opportunistic granulomatous lesions and, subsequently, a parkinsonian akinetic-rigid syndrome. In agreement with cases reported in the literature, the parkinsonian syndrome developed only when the lesions bilaterally involved basal ganglia. The critical localization of the opportunistic lesions in the direct and indirect strio-pallidal pathways possibly associated with the hiv-related neurotoxicity might have contributed to determine this clinical picture.- - - - - - - - - - ranking = 0.40285383999708keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
4/72. Mid-dermal elastophagocytosis presenting as a persistent reticulate erythema.Two men are presented with a widespread persistent reticulate erythema concentrated within the chronically sun-damaged skin on their trunk. A fine papular element was present in one case and both lacked annular lesions. One patient was human immunodeficiency virus positive. Multiple skin biopsies showed an interstitial infiltrate of histiocytes containing multiple elastic fibres in the upper dermis. There was scant perivascular lymphocytic inflammation but no evident necrobiosis or palisaded granulomas seen typically with granuloma annulare. Elastic stains showed focal mid-dermal elastolysis. Diffuse reticulate erythema in sun-damaged skin may be a clinical marker for elastophagocytosis. This presentation differs from that previously described with actinic granuloma, diffuse granuloma annulare or the inflammatory phase of mid-dermal elastolysis and expands the clinical spectrum of this phenomenon.- - - - - - - - - - ranking = 0.2keywords = immunodeficiency (Clic here for more details about this article) |
5/72. common variable immunodeficiency treated with a recombinant human IgG, tumour necrosis factor-alpha receptor fusion protein.common variable immunodeficiency (CVI) is characterized by a failure in B-cell differentiation and impaired immunoglobulin secretion, but with a variable clinical presentation, including the development of sarcoidal granulomas and autoimmune diseases, as well as an increased incidence of malignancies. We present a 21-year-old white man who carried a diagnosis of juvenile rheumatoid arthritis and presented 6 years later with scarring alopecia showing sarcoidal granulomas. Further work confirmed the diagnosis of CVI, and with increasing systemic symptoms, it was elected to treat the patient with a tumour necrosis factor (TNF)-alpha antagonist, a TNF-alpha receptor IgG1 fusion protein. The patient showed improvement in his systemic symptoms and some hair regrowth after 3 months of therapy, and continued improvement in his systemic disease with only mild scalp hair thinning in the areas of prior involvement after almost 1 year of therapy. CVI and sarcoid may have overlapping clinical and immunological findings. Previous therapies for CVI, including intravenous immunoglobulin, have not altered the mortality of the disease. TNF-alpha is a primary cytokine and is elevated in CVI, and specific inhibition of TNF-alpha in this patient was effective in moderating his disease, including his skin disease.- - - - - - - - - - ranking = 1keywords = immunodeficiency (Clic here for more details about this article) |
6/72. Granulomatous renal disease in a patient with common variable immunodeficiency.common variable immunodeficiency (CVID), the most common cause of primary hypogammaglobulinemia, is characterized by a decreased serum immunoglobulin level, recurrent infections, and the occurrence of various autoimmune diseases. Granulomatous disease has been reported previously in several patients with CVID, with granuloma occurring in the lymph nodes, spleen, liver, central nervous system, and bone marrow. We report the first published case of renal granulomatous disease in a CVID patient presenting with subacute renal failure. Renal function partially recovered after corticosteroid treatment and intravenous immunoglobulin infusions. The pathogenesis of granulomatous disease in CVID is unclear but may involve monocyte and T-cell abnormalities.- - - - - - - - - - ranking = 1keywords = immunodeficiency (Clic here for more details about this article) |
7/72. Cutaneous granulomas masquerading as tuberculoid leprosy in a patient with congenital combined immunodeficiency.Combined immunodeficiency disorders are characterized by abnormalities in cellular and humoral immunity. This classification includes common variable immunodeficiency (CVI), a primary immunodeficiency disorder characterized by hypogammaglobulinemia, recurrent bacterial infections, and significant T-cell abnormalities. Associated autoimmune diseases include rheumatoid arthritis, pernicious anemia, idiopathic thrombocytopenic purpura, and systemic lupus erythematous. Granulomatous lesions in lymphoid tissues, solid organs, and skin have been reported. We describe a patient with CVI who developed cutaneous granulomas with perineural invasion; to our knowledge, this is a previously undescribed feature.- - - - - - - - - - ranking = 1.4keywords = immunodeficiency (Clic here for more details about this article) |
8/72. A unique renal lesion in common variable immunodeficiency.This article reports the case of a 33-year-old woman with common variable immunodeficiency (CVI) who developed renal failure 17 years after diagnosis and initiation of treatment with monthly IVIG. A renal biopsy revealed mesangial and paramesangial immune complex deposition and interstitial granulomatous infiltration. Renal function improved with oral corticosteroids, but did not return to normal. Decreasing the dose of IVIG had no effect on renal function. Immune dysfunction can be associated with both granulomatous disease and immune complex glomerulonephritis, or the latter may be related to chronic infection or immunoglobulin use. This is the first report of concomitant glomerular-tubulointerstitial lesions in this immunodeficiency syndrome. Renal function should be closely followed in patients with CVI.- - - - - - - - - - ranking = 1.4028538399971keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
9/72. pityriasis lichenoides in a girl with the granulomatous form of common variable immunodeficiency.pityriasis lichenoides (PL) is a cutaneous disease of unknown origin. In our 8-year-old female patient with the granulomatous form of common variable immunodeficiency (CVID), PL occurred together with massive splenomegaly and intra-abdominal lymphadenopathy. prednisone was efficient for treatment of her splenomegaly and autoimmune cytopenias. However, PL was resistant to both topical and systemic steroid treatment. Healing of PL was achieved with the use of a super-potent topical steroid, clobetasol propionate. A defect of T-cell function in CVID may contribute to development of PL. In the granulomatous form of CVID, sarcoid-like granulomas are the most commonly reported cutaneous lesions. PL has not been previously reported.- - - - - - - - - - ranking = 1keywords = immunodeficiency (Clic here for more details about this article) |
10/72. Pediatric granulomatous cerebral amebiasis: a delayed diagnosis.We present four cases of cerebral amebae infection treated at our neurosurgical department. Patient 1 was a 12-year-old male with skin lesions of 2 years' progression involving the midface. He received a corticosteroid course, and, after that, he presented a right body hemiparesis. Patient 2 was a 5-year-old male, with a past surgical history of fibula fracture and osteomyelitis of 1-year evolution, associated with lesions of the surrounding skin that presented with partial seizures. Patient 3 was a 3-year-old female who presented with a stroke-like episode and with partial seizures. Patient 4 was a 6-year-old male who had ulcerative lesions in the face of 1-year evolution. After a corticosteroid course, he presented with right-body hemiparesis. All patients were human immunodeficiency virus-negative and died 1 month or less after surgery because of progressive evolution of the disease. Histopathology revealed granulomatous amebic encephalitis. All patients revealed infection from balamuthia mandrillaris (Leptomyxiidae). Treatment consisting of pentamidine, clarithromycin, fluconazole, and 5-fluorocytosine was ineffective. Although extremely uncommon, granulomatous amebic encephalitis should be considered in the differential diagnosis of cerebral lesions while nonspecific, associated granulomatous skin lesions support the diagnosis of amebiasis.- - - - - - - - - - ranking = 0.2keywords = immunodeficiency (Clic here for more details about this article) |
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