Sebaceous trichofolliculoma, which is a variant of trichofolliculoma, is a rare disease that clinically show a centrally depressed lesion usually singly on the nose, and histologically a centrally located cavity, lined by squamous epithelium, with numerous sebaceous lobules connected to them. We describe a case of sebaceous trichofolliculoma in a 27-year-old woman who had several brown papules on the nose. We think that this is the first report of sebaceous trichofolliculloma in Korean literature.
Adult ; Epithelium ; Female ; Humans ; Nose ; Rare Diseases

Focal segmental glomerular sclerosis (FSGS) accounts for recurrence in 20% to 40% of the renal allografts after transplantation, and it causes graft loss in 13% to 20% of the cases. We report here on successfully treating acute cellular rejection (ACR) combined with FSGS after a kidney transplantation with a combination treatment of plasmapheresis, rituximab and steroid pulse therapy. A 53-year-old female patient whose primary kidney disease was unknown developed massive proteinuria after living donor kidney transplantation. A urine protein/creatinine ratio of 13.42 and an elevated serum creatinine level was detected on postoperative days (POD) 10 and a renal biopsy showed acute cellular rejection (Banff IIb) combined with FSGS. We started steroid pulse therapy on POD 11. She underwent 5 plasmapheresis sessions in the first 3 week after transplantation and she received one dose of rituximab (375 mg/m2) on POD 12. The proteinuria decreased below the nephrotic range at POD 20 and the serum creatinine level was normalized. Three months later, the proteinuria was at 35 mg/day with stable graft function. Rituximab and plasmapheresis is a possible option to treat FSGS combined with a relapse of proteinuria after renal transplantation.
Antibodies, Monoclonal, Murine-Derived ; Biopsy ; Creatinine ; Female ; Humans ; Kidney Diseases ; Kidney Transplantation ; Living Donors ; Middle Aged ; Plasmapheresis ; Proteinuria ; Recurrence ; Rejection (Psychology) ; Rituximab ; Sclerosis ; Transplantation, Homologous ; Transplants

Thrombotic thrombocytopenic purpura (TTP) and the related hemolytic uremic syndrome (HUS) are disorders characterized by thrombocytopenia, microangiopathic hemolytic anemia, a variable degree of impairment of renal function and fluctuating neurological symptoms, which are thought to be due to platelet activation and subsequent formation of thrombi in the microcirculation. The fact that there was no clear-cut clinical and laboratory features that differentiate HUS from TTP has lead to view these two syndromes as a clinical continuum. Microvascular thrombosis is the typical lesion and closely related with endothelial injury and platelet activation. Pathologic alterations of the brain parenchyma are mainly manifested by small multiple infarcts. Numerous cases of CNS complications of these syndromes have been evaluated by using CT, but few reports have mentioned the MR findings. We experienced a case of TTP-HUS that had clinical features of cortical blindness and the brain lesion was confirmed by MRI showing cerebral infarct at the occipital area but it was reversible course. So we report this case with a brief review of literature.
Anemia, Hemolytic ; Blindness, Cortical* ; Brain ; Hemolytic-Uremic Syndrome* ; Magnetic Resonance Imaging* ; Microcirculation ; Platelet Activation ; Purpura, Thrombotic Thrombocytopenic* ; Thrombocytopenia ; Thrombosis

Acute antibody-mediated rejection (AMR) developing simultaneously with acute cellular rejection has been rarely reported as a long-term complication of renal transplantation, and it can present on top of another chronic pathology affecting the graft. A 51-year-old female patient with chronic kidney disease of unknown etiology received renal transplantation 12 years ago from a living unrelated donor with 3 HLA mismatches. She received induction therapy with methylprednisolone and was maintained on steroids, mycophenolate mofetil and cyclosporine A (CsA). For a period of twelve years post-transplantation, she was clinically and biochemically stable. She presented with a rise in serum creatinine (SCr.) from 1.3 mg/dL to 2.4 mg/dL but did not have proteinuria. Graft biopsy revealed findings suggestive of acute cellular rejection on top of antibody-mediated rejection (type II) and chronic calcineurin inhibitor toxicity. Panel reactive antibody (PRA) test levels were 3.6%, 91.7% for class I and II respectively. The patient was treated with high-dose methylprednisolone for 3 days but serum creatinine was not fully normalised. After 2 weeks from initial methyl-PDS pulse therapy, she received intravenous immunoglobulin, plasma exchange and anti-CD20 (rituximab). Cyclosporine was changed to tacrolimus. She achieved a complete response, and SCr. was maintained at 1.3 mg/dL without proteinuria. Follow-up PRA test levels were 0%, 75% for class I and II. Current therapies have had considerable success in reversing mixed, acute humoral and cellular rejection since it is being identified quickly and treated aggressively. The best use of rituximab to treat AMR should be evaluated in controlled trials using dosing strategies that include longer courses or retreatment schedules.
Antibodies, Monoclonal, Murine-Derived ; Appointments and Schedules ; Biopsy ; Calcineurin ; Creatinine ; Cyclosporine ; Female ; Follow-Up Studies ; Graft Rejection ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Kidney Transplantation ; Methylprednisolone ; Middle Aged ; Mycophenolic Acid ; Plasma Exchange ; Plasmapheresis ; Proteinuria ; Rejection (Psychology) ; Renal Insufficiency, Chronic ; Retreatment ; Rituximab ; Steroids ; Tacrolimus ; Transplants ; Unrelated Donors

Cryptococcosis is recognized as one of the most important complications in an organ transplant recipient. Cryptococcosis occurs in 2.5-39% of renal transplant recipients. This infection generally presents as symptomatic disseminated disease with an accelerated clinical course, involves multiple sites including the central nervous system, lungs, and skin. And if diagnosis or treatment is delayed, the prognosis is generally poor. The asymptomatic infection is rare and there are no case reports of asymptomatic disseminated cryptococcosis after renal transplantation in Korea. We experienced a case of asymptomatic cryptococcal multi-organ infection detected incidentally in a 51-year-old male received a living related renal transplant 35 months earlier for end-stage renal disease due to diabetic nephropathy. We treated successfully with amphotericin B and fluconazole and hereby report this case with a review of the relevant literature.
Amphotericin B ; Asymptomatic Infections ; Central Nervous System ; Cryptococcosis ; Diabetic Nephropathies ; Fluconazole ; Humans ; Kidney Failure, Chronic ; Kidney Transplantation ; Korea ; Lung ; Male ; Middle Aged ; Prognosis ; Skin ; Transplants

Focal segmental glomerular sclerosis (FSGS) is known to recur in 20-40% of the renal allografts with graft loss in about half of these cases. We report a successful treatment of a recurrent FSGS after kidney transplantation with rituximab and plasmapheresis. An 16-year-old patient whose primary kidney disease was FSGS developed recurrence of proteinuria after living donor kidney transplantation despite preemptive plasmapheresis and one dose of rituximab (375 mg/m2). After kidney transplantation, nephrotic range proteinuria was detected. Kidney biopsy was done and showed recurrent FSGS. She undergone 11 times of plasmapheresis in the first 4 week post transplantation. In addition, she received additional one dose of rituximab (375 mg/m2) on day 14. Proteinuria was decreased below nephrotic range at 37 day. Ten months later, proteinuria was at 30 mg/day with excellent graft function. No significant adverse events related to rituximab or plasmapheresis were observed. Rituximab with plasmapheresis may be another option for recurrent FSGS after kidney transplantation.
Adolescent ; Antibodies, Monoclonal, Murine-Derived ; Biopsy ; Glomerulosclerosis, Focal Segmental ; Humans ; Kidney ; Kidney Diseases ; Kidney Transplantation ; Living Donors ; Plasmapheresis ; Proteinuria ; Recurrence ; Sclerosis ; Transplantation, Homologous ; Transplants ; Rituximab

It has been well known that long-term immune suppression in renal transplant patients increases the possibility of complications. Infectious disease is one of the representative complications. We experienced a case of nocardiosis with cytomegalovirus infection after third renal transplantation in China. Nocardiosis is an important opportunistic infection in immunosuppressed patients, lymphoma, sarcoidosis, and organ transplant patients. CMV can cause severe hepatitis, pneumonitis, enteritis, endometritis, and encephalitis. It can depress bone marrow, and impair the immune system so as to increase other bacterial infection and trigger rejections. Third renal transplantation causes long-term immune suppression or over-immune suppression on transplant patients. Very few cases of third renal transplantation have been reported in Korea. We reduced the dose of immune- suppressants, and treated it successfully with ganciclovir and Trimethoprim/Sulfamethoxazole (Bactrim(R)).
Bacterial Infections ; Bone Marrow ; China* ; Communicable Diseases ; Cytomegalovirus ; Cytomegalovirus Infections ; Encephalitis ; Endometritis ; Enteritis ; Female ; Ganciclovir ; Hepatitis ; Humans ; Immune System ; Kidney Transplantation* ; Korea ; Lymphoma ; Nocardia Infections* ; Opportunistic Infections ; Pneumonia ; Sarcoidosis ; Transplants

Peritonitis is a major cause of morbidity in continuous ambulatory peritoneal dialysis (CAPD) patients. Achromobacter xylosoxidans is a rarely reported cause of peritonitis in CAPD patients. In this report, a peritonitis case due to Achromobacter xylosoxidans in a 60-year-old male patient with end-stage renal failure receiving CAPD for 7 years, has been reported. White blood cell (WBC) count in peritoneal fluid was 3,160/mm3 with 95% neutrophil. Gram staining of the peritoneal fluid yielded gram negative rod. Empirical antibiotic therapy with ceftriaxone was initiated intraperitoneally. But drug sensitivity test revealed these regimens were resistant. On fourth hospital day, Achromobacter xylosoxidans was cultured from peritoneal effluent, the antibiotic regimen was switched to piperacillin/tazobactam intraperitoneally. The patient rapidly recovered and the WBC count of the peritoneal effluent decreased. The therapy was continued for 14 days and then the patient was discharged. The peritoneal catheter was not removed.
Achromobacter ; Achromobacter denitrificans ; Ascitic Fluid ; Catheters ; Ceftriaxone ; Humans ; Kidney Failure, Chronic ; Leukocytes ; Male ; Middle Aged ; Neutrophils ; Peritoneal Dialysis, Continuous Ambulatory ; Peritonitis

Secondary hyperparathyroidism is a major complication in ESRD patients undergoing dialysis. In hemodialysis patients with secondary hyperparathyroidism, intravenous administration of paricalcitol became widely utilized. In CAPD patients, however, the intravenous administration of paricalcitol which requires frequent visits to the clinic is not practical. The subject of this study was one CAPD patient with secondary hyperparathyroidism. He had already received oral calcitriol pulse therapy for 6 months and thereafter refused parathyroidectomy and intravenous paricalcitol which required frequent visits to the hospital. Furthermore, paricalcitol capsule is not yet introduced in Korea. Consequently, intraperitoneal paricalcitol therapy was tried whereby the patient was taught how to inject the paricalcitol (5 ug) directly into the dialysate for three times per week before bedtime. Blood samples for measurement of intact parathyroid hormone (iPTH), serum ionized calcium, serum phosphate, serum total alkaline phosphatase levels were obtained at baseline and after 1, 2, 3 and 4 months of treatment. After usage of intraperitoneal paricalcitol for 2 months, there was a significant decrease in iPTH level. In conclusion, intraperitoneal paricalcitol therapy might be effective for suppressing iPTH in CAPD patients with secondary hyperparathyroidism. A large-scale and long-term study must be conducted for safety and clinical effect.
Administration, Intravenous ; Alkaline Phosphatase ; Calcitriol ; Calcium ; Dialysis ; Ergocalciferols ; Humans ; Hyperparathyroidism, Secondary ; Injections, Intraperitoneal ; Kidney Failure, Chronic ; Korea ; Parathyroid Hormone ; Parathyroidectomy ; Peritoneal Dialysis, Continuous Ambulatory ; Renal Dialysis

PURPOSE: The presence of C4d in peritubular capillaries (C4d (PTC)) as a diagnostic in-situ marker of acute humoral rejection and CD20 as marker of B-cell deposition in graft kidney has been reported to be related to steroid resistance and poor outcome. In this retrospective study, we evaluated the clinical significance of C4d and CD20 in allograft renal biopsies by immunohistochemistry technique. And we also evaluated the relationships between C4d and CD20 positive B lymphocytes. METHODS: We studied 22 patients who had been biopsied for suspected acute rejection. Biopsies were classified by updated Banff 97 criteria. Of the 22 cases, borderline rejection and Banff 1A were 11 cases respectively and no case had a vascular lesion. Paraffin sections were stained with monoclonal antibodies (anti-C4d and -CD20) using an immunohistochemistry technique and the results of immunohistochemistry were analyzed by clinical data. RESULTS: Of the 22 cases, 22.7% (5/22) showed diffuse and 40.9% (9/22) showed focal C4d positivity in peritubular capillaries. The grafts failed to survive in 20% (1/5) of the diffuse (P), 44.4% (4/9) of the focal, and 0% (0/8) of the negative group for 2 years since postbiopsies, however, the C4d staining was not statistically related to graft loss and graft survival rates (P=0.091, P=0.106 respectively). The C4d positivity was significantly related to the level of serum creatinine (P=0.042) and to steroid pulsing therapy resistance (P=0.030). However C4d deposition was not associated with recipient gender, age, type of donor (living vs deceased), HLA matching, induction, and Banff classification. On the CD20 immunostaining, 50.0% (11/22) showed negative reactivity, 9.1% (2/22) one nodule, 40.9% (9/22) 2 nodules. The presence and the number of CD20 positive nodules were not correlated to the C4d clinical data. But, the degree of C4d staining was statistically related with the presence of CD20 positive nodules (P=0.029). CONCLUSION: The peritubular capillary C4d is clinically important however, not likely a significant predictor of grafts survival rates in mild rejection. The clinical implication of CD20 positive B lymphocyte nodules in acute rejection was not demonstrated in this study. But, CD20 positive B lymphocyte may be a positive linkage with C4d and participate in humoral rejection.
Antibodies, Monoclonal ; B-Lymphocytes ; Biopsy ; Capillaries ; Creatinine ; Graft Survival ; Humans ; Immunohistochemistry ; Kidney ; Lymphocytes ; Paraffin ; Rejection (Psychology) ; Retrospective Studies ; Survival Rate ; Tissue Donors ; Transplantation, Homologous ; Transplants