Acquired perforating dermatosis with associated complicated cellulitis and amputation in a hemodialysis patient.

INTRODUCTION: Cutaneous manifestations related to chronic kidney disease (CKD) are common and associated with high morbidity. Acquired perforating dermatosis (APD) occurs mostly in diabetic or CKD patients, namely those undergoing hemodialysis. CASE REPORT: A 58-year-old male with type 2 diabetes, with long-term insulin use, several microvascular and macrovascular complications, and on maintenance hemodialysis for 5 years presented with a 1-week history of painful, pruritic, umbilicated papules and some punctiform necrotic lesions on his left forearm, both hands, and both amputation stumps. There was no evidence of infection or vascular alterations, and the patient was not responsive to an initial course of topical corticosteroid. These lesions rapidly evolved to larger, coalescent necrotic injuries, with aggravated pain, intense left-hand skin peeling, and the appearance of similar lesions in the trunk, requiring hospital admission. Calciphylaxis and APD were suspected. Skin biopsy was performed and directed treatment initiated, including intradialytic sodium thiosulfate. Histology findings were compatible with APD and also excluded findings suggestive of vasculitis or calciphylaxis. Soon after, difficult-to-treat cellulitis of the left hand and forearm progressed to critical ischemia and amputation. Microbiology analysis revealed Serratia marcescens as the causative agent. DISCUSSION: To our knowledge, there are no previously described cases of APD-related cellulitis. Its treatment can be particularly challenging, as lesions can persist and relapse, and chronic scars can develop. S. marcescens behaves as an opportunistic and difficult-to-treat pathogen, complicating the prognosis. CONCLUSION: APD can be associated with cellulitis and all of its complications in patients with underlying severe vasculopathy. Awareness of this complication in APD with early referral and aggressive treatment might improve the outcomes and quality of life of such patients.

Gaucher disease and Lupus: A rare association? / Gaucher y lupus: ¿una rara asociación?

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[Obesity and the kidney]. / O risco renal da obesidade.

Obesity represents an important risk factor for the development of chronic kidney disease (CKD), due to its known strong association with diabetes mellitus and hypertension, the two major causes of CKD, but also as an independent renal risk factor. This direct relationship between obesity and kidney injury has been undervalued. The aim of this revisión is to point out the mechanisms of kidney injury induced by obesity, underline the importance of this association and alert for the prevention, education and treatment of the obese patient, as a way to control this heavy modifiable risk factor.

Severe Relapsing Goodpasture's Disease Successfully Treated with Mycophenolate Mofetil.

Goodpasture's disease is a rare autoimmune disorder characterised by the development of antiglomerular basement membrane autoantibodies, which typically presents with rapidly progressive crescentic glomerulonephritis and pulmonary haemorrhage. Even with aggressive nonspecific immunosuppression and plasma exchange, mortality remains high. We report a case of life-threatening Goodpasture's disease with relapsing pulmonary haemorrhage refractory to conventional therapy. Second line treatment was based on mycophenolate mofetil 1 g every 12 hours and prednisolone 60 mg/day. In this case, the use of a low-dose mycophenolate mofetil regimen turned out to be insufficient. However, in our opinion higher mycophenolate mofetil doses should be considered an alternative treatment, mainly in relapsing disease, due to its mechanism of action and current insufficient therapeutic weapons.

Elderly patients on chronic hemodialysis with hyperparathyroidism: increase of hemoglobin level after intravenous calcitriol.

In patients on chronic hemodialysis (CHD), hyperparathyroidism (HPTH) is associated with anemia and resistance to erythropoietin (EPO). In the last few years, calcitriol intravenously (IV) has been used with success in the treatment of the HPTH, secondary to chronic renal failure. However, the effects of calcitriol on the hematological parameters of these patients have never been well evaluated. This study included 11 elderly CHD patients (f = 6, m = 5; mean age = 73.6 years, mean time on CHD = 42.8 months) with HPTH under EPO therapy (IV). They were treated for 12 months with calcitriol IV (mean dose = 2.33 mcg/pt/week). Patients with iron deficiency anemia (ferritin < 200 ng/ml) were excluded. The patients were compared before and after 12 months of calcitriol treatment, with respect to several laboratory parameters and with respect to EPO dose. A paired t-test was used. After treatment, we found a decrease of PTH (634 vs. 418 pg/ml, P = 0.029); the serum calcium increased (8.8 vs. 9.9 mg/dl, P = 0.002); no differences were noted in the plasma levels of alkaline phosphatase, phosphorous, BUN, creatinine, Na and K. Mean levels of Hb (10.2 vs. 11.4 g/dl, P = 0.004) and the Hct (30 vs. 34.3, P = 0.004) increased after 12 months of calcitriol; the levels of serum iron (70 vs. 78 microg/dl, P = ns) and ferritin (531 vs. 785 ng/ml, P = ns) and the EPO dose (105 vs. 100 U/kg/week, P = ns) were similar before and after treatment. Our data show that the treatment of HPTH in CHD elderly patients with calcitriol can increase Hb level without increasing EPO dose.

Elderly patients on chronic hemodialysis: effect of the secondary hyperparathyroidism on the hemoglobin level.

In patients on chronic hemodialysis (CHD) hyperparathyroidism (HPTH) is associated with anemia and resistance to erythropoietin (EPO). This study included 86 CHD elderly pts (mean age 74.8 y, mean time on CHD = 50.5 mos); they were divided into two groups: I (n = 31) - PTH > 250 pg/mL and II (n = 55) - PTH < 250 pg/mL. All these patients had been on CHD for > 6 mos. No differences were found between groups in respect to age, sex distribution and time on CHD. The levels of creatinine, BUN, Ca, Al, Fe, albumin and ferritin were similar. Group I had a higher P level (5.4 vs 4.3 mg/dL, p = 0.001) and Ca x P (53.5 vs 43.7, p = 0.009). Also the Hct (31 vs 33.5%, p = 0.008) and the Hb (10.4 vs 11.2 g/dL, p = 0.009) values were lower in Group I. The EPO dose (88 vs 85 U/kg/week, p = ns) was similar in the two groups. Our data showed that elderly patients with HPTH have lower Hct and Hb levels than do younger patients on a similar EPO dose. We believe these patients will need a more aggressive therapy with calcitriol.