Kaposiform hemangioendothelioma: five patients with cutaneous lesion and long follow-up.

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor of the skin, deep soft tissue, and bone in children, characterized by infiltrating nodules and sheets of spindle cells, and unmistakable resemblance to Kaposi's sarcoma. More than 60 patients with such tumor have been reported so far, and while many have died as a result of extensive disease and severe coagulopathy, the long-term biologic behavior of this tumor remains undetermined. We describe five patients with kaposiform hemangioendothelioma and a mean follow-up of 19 years, ranging from 8 to 35 years. This report emphasizes on the importance of cutaneous lesions being the most commonly affected site, but also for its clinical diversity. Early diagnosis is possible even for a small skin lesion, which may be critical for the treatment of a potentially fatal deep-seated extensive tumor. All five patients are well, and three of them with persistent vascular tumor, which has carried two patients from childhood to adult. Although the behavior of this tumor might have been modified by radiation or interferon in three patients, this series indicates that kaposiform hemangioendothelioma is incapable of metastasis, despite a protracted course of many decades with no tendency for spontaneous regression.

Grading of acute and chronic renal lesions in Henoch-Schönlein purpura.

The renal outcome of 34 patients with Henoch-Schönlein purpura nephritis was assessed clinically and by grading acute and chronic renal lesions using a system we applied to primary IgA nephropathy. On a median follow-up period of 65 months, hypertension and the serum levels of creatinine and proteinuria at the time of renal biopsy were correlated with renal survival. Acute glomerular lesions including mesangial hypercellularity, endocapillary proliferation, necrosis, cellular crescents, and leukocytes infiltration were observed, respectively, in 41%, 12%, 50%, 29%, and 32% of the cases. Of these, only glomerular necrotizing lesion and cellular crescent were correlated with the renal survival. Chronic renal lesions based on a grading system applied to primary IgA nephropathy and assessing the extent of glomerular sclerosis (glomerular grading), of tubular loss and interstitial fibrosis (tubulointerstitial grading), and of hyaline arteriolosclerosis demonstrated correlation between these lesions, as well as with renal survival. On follow-up, these chronic renal lesions were predictors of subsequent clinical events associated with disease progression, such as impaired renal function, significant proteinuria, and development of hypertension. Despite some limitations related to the relatively small size, this series indicates that distinction of acute and chronic lesions of Henoch-Schonlein purpura nephritis is important for both the prognosis and management of patients.

Rapid recurrence of ANCA-negative pauci-immune vasculitis after cadaveric renal transplantation.

We report a patient with renal failure secondary to antineutrophil cytoplasmic autoantibody (ANCA)-negative pauci-immune systemic vasculitis who developed rapid recurrence of vasculitis involving skin, eye, and kidney allograft 1 month after cadaveric renal transplant while being maintained on cyclosporine, mycophenolate mofetil, and prednisolone. Despite early institution of aggressive treatment with plasma exchange, the disease remained uncontrolled and resulted in substantial morbidity and subsequent graft failure within 1 year post-transplantation. This case confirms that ANCA-negative vasculitides may recur in transplanted patients taking mycophenolate mofetil. Transplantation is contraindicated when active disease is evident.

IgA nephropathy: a rare lesion in systemic lupus erythematosus.

We describe three patients with a well-established clinical diagnosis of systemic lupus erythematosus in whom the renal biopsy lesion unexpectedly is diagnostic of IgA nephropathy, not superimposed with any features of lupus nephritis. Whereas the clinical presentation and follow-up of renal disease in these patients indicate a relatively indolent course, the extrarenal manifestations of systemic lupus erythematosys have been relatively severe, and one patient died of systemic infection. IgA nephropathy hitherto has not been described in patients with systemic lupus erythematosus, and such an observation stresses that atypical glomerular lesions in these patients should raise the possibility of a nonlupus glomerulopathy.

Tufted angioma with complete regression.

We describe 2 patients with tufted angioma (angioblastoma of Nakagawa) which regressed spontaneously. Both patients had a solitary but large lesion on the trunk. The 1st patient, a 3-month-old girl, presented with a 6-cm plaque which began to regress 2 years later. It regressed completely over 1 year and had not recurred after a further 6-year follow-up. The other patient, a 3-month-old boy, presented with a 12-cm mass of coalescent angiomatous papules which slowly regressed over 3-years, while small new lesions appeared in the adjacent contiguous skin. Eventually, gradual and near complete regression was observed after 8 years. The histology in both cases was typical, but bore some resemblance to the recently described kaposiform hemangioendothelioma, lobular capillary hemangioma and spindle cell hemangioendothelioma.

Cutaneous Rosai-Dorfman disease presenting as a suspicious breast mass.

Cutaneous Rosai-Dorfman disease in a male patient presented as a clinically malignant left breast mass. The intra-operative frozen section indicated a benign inflammatory lesion and a simple excision was performed. The disease was apparently limited to the skin and subcutis of the left breast with no lymph nodes or other organs involvement. While the lesion demonstrated the characteristic morphology of Rosai-Dorfman disease, the diagnosis of granulomatous mastitis, epithelioid sarcoma and other cutaneous xanthohistiocytic proliferations were considered. In the absence of lymphadenopathy, a high index of diagnostic awareness is required to recognize the uncommon, solitary cutaneous Rosai-Dorfman disease.

The clinicopathological characteristics of IgA nephropathy in Hong Kong.

The clinicopathologic data of 237 Chinese patients with IgA nephropathy from Hong Kong are reviewed in an attempt to identify the features pertinent to Chinese patients. Although the nephropathy is commonest in the 26-35 year age group, 11% of the IgA nephritic patients were children below 16 years. The male predilection reported in Caucasian populations is not observed and the male:female ratio is 0.94 in our series. The commonest renal manifestation is microscopic hematuria (25%) and 19% of the patients present with macroscopic hematuria, not infrequently synpharyngitic. Nephrotic syndrome occurs in 15% of our patients and proteinuria more than 1 gm/day is documented in 58% of these IgA nephritic patients. The degree of proteinuria does not correlate with prognosis. A small proportion of these nephrotic patients respond to steroid therapy, suggesting a variant of IgA nephropathy that resembles lipoid nephrosis in its steroid-responsiveness. Seventeen percent of the patients (18/104) are hepatitis B virus carriers and 61% of these patients demonstrate viral antigens in their renal biopsies, indicating that hepatitis B virus infection may sometimes play a pathogenetic role.

Hepatitis B virus-related glomerulopathy in patients with systemic lupus erythematosus.

The clinical data and renal pathologic information from three patients with systemic lupus erythematosus (SLE), active glomerular disease, and hepatitis B virus (HBV) antigenemia are presented. All three patients fulfilled the American Rheumatism Association criteria for the diagnosis of SLE. However, the renal pathologic results excluded the diagnosis of lupus nephritis. The common findings shared by these patients included the following: presence of hepatitis B surface antigen (HBsAg) in both serum and glomeruli and of glomerular hepatitis B core antigen (HBcAg), and the absence of polyclonal immunoglobulins, C1q and C4, deposition in renal tissue. These common features and the renal pathologic results indicated that the glomerulopathy was associated with HBV antigenemia. The cases described here may represent a subset of patients with SLE in whom expression of lupus nephritis was altered by the concomitant HBV-related glomerulonephritis.

Microwave fixation in diagnostic renal pathology.

Microwave-fixed tissues were examined in 10 patients undergoing diagnostic renal biopsy. A small portion of renal tissue was fixed by microwave irradiation and subsequently processed by routine methods for light microscopic, immunofluorescent and electron microscopic studies. The remaining portion of specimen was fixed and processed by conventional methods. In light microscopic examination, the renal architecture and cell morphology were well-preserved. Pathological changes were identical to those seen with formalin-fixed tissue. The pattern, distribution and intensity of positive immunofluorescence in microwave-fixed tissue were similar to those in tissues directly snap-frozen and stained. In electron microscopy, the normal and pathological features were well-demonstrated and not different from those observed in glutaraldehyde-fixed specimens. Specific ultrastructural lesions were clearly demonstrated and, apparently, were not altered by microwave irradiation. Our preliminary data indicate that microwave fixation can be effectively applied in the processing of renal biopsies. As the fixation is rapid, this method may be valuable in circumstances when an urgent diagnosis is required.