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Calcified cysts in the upper abdomen
A 69-year-old man has been on hemodialysis for about 15 years. Routine plain chest radiographs (Figure 1) show multiple calcified cystic lesions in the upper abdomen. He has no fever or abdominal pain.
Q: Which is the most plausible diagnosis?
- Nephrolithiasis
- Hydatid cyst
- Polycystic kidney and liver disease
- Metastatic calcification
- Intra-abdominal abscesses
- Cystadenoma
A: This patient has polycystic kidney disease. He first came to our hospital 21 years ago because of upper gastrointestinal bleeding, and he was found to have multiple cystic lesions. He was diagnosed as having polycystic kidney disease with multiple hepatic cysts.
About 15 years ago, he had several episodes of gross hematuria with right flank pain, which were attributed to bleeding from the cysts. Because his renal function was deteriorating, he started regular hemodialysis at that time. He underwent parathyroidectomy for secondary hyperparathyroidism 2 years ago.
The differential diagnosis of calcified cystic lesions in the upper abdomen includes infectious diseases (eg, chronic intra-abdominal abscess, echinococcosis, cysticercosis), neoplasms (eg, cystadenomas, cystadenocarcinomas), organized hematomas, liver cysts, renal cysts, gallstones, renal stones, calcified lymph nodes, and vessel calcification.
Autosomal dominant polycystic kidney disease is the most common inherited renal disease.1 Typically, patients with this disease have a positive family history, although about 5% to 10% do not.1,3 (Our patient did not.)
The diagnosis is suggested by family history and imaging, with typical findings including large kidneys and extensive cysts scattered throughout both kidneys.1–3 The history, epidemiologic data, and the character of the cystic lesions help in making the final diagnosis.
In patients at risk, the presence of at least two renal cysts (unilateral or bilateral) at age 15 to 30, of at least two cysts in each kidney at age 30 to 59, and of at least four cysts in each kidney at age 60 or older is regarded as sufficient to establish the diagnosis.1–3
Approximately 80% of patients with autosomal dominant polycystic kidney disease also have hepatic cysts.1,3 Cyst calcifications occur in about 25% of patients, either as a residual effect of intracystic hemorrhage or as a consequence of secondary hyperparathyroidism.4,5
- Braun WE. Autosomal dominant polycystic kidney disease: emerging concepts of pathogenesis and new treatments. Cleve Clin J Med 2009; 76:97–104.
- Torres VE, Bennett WM. Diagnosis of and screening for autosomal dominant polycystic kidney disease. In:Rose BD, editor: UpToDate. Waltham, MA: UpToDate, 2009.
- Grantham JJ. Clinical practice. Autosomal dominant polycystic kidney disease. N Engl J Med 2008; 359:1477–1485.
- Coffin B, Hadengue A, Degos F, Benhamou JP. Calcified hepatic and renal cysts in adult dominant polycystic kidney disease. Dig Dis Sci 1990; 35:1172–1175.
- Levine E, Grantham JJ. Calcified renal stones and cyst calcifications in autosomal dominant polycystic kidney disease: clinical and CT study in 84 patients. AJR Am J Roentgenol 1992; 159:77–81.
A 69-year-old man has been on hemodialysis for about 15 years. Routine plain chest radiographs (Figure 1) show multiple calcified cystic lesions in the upper abdomen. He has no fever or abdominal pain.
Q: Which is the most plausible diagnosis?
- Nephrolithiasis
- Hydatid cyst
- Polycystic kidney and liver disease
- Metastatic calcification
- Intra-abdominal abscesses
- Cystadenoma
A: This patient has polycystic kidney disease. He first came to our hospital 21 years ago because of upper gastrointestinal bleeding, and he was found to have multiple cystic lesions. He was diagnosed as having polycystic kidney disease with multiple hepatic cysts.
About 15 years ago, he had several episodes of gross hematuria with right flank pain, which were attributed to bleeding from the cysts. Because his renal function was deteriorating, he started regular hemodialysis at that time. He underwent parathyroidectomy for secondary hyperparathyroidism 2 years ago.
The differential diagnosis of calcified cystic lesions in the upper abdomen includes infectious diseases (eg, chronic intra-abdominal abscess, echinococcosis, cysticercosis), neoplasms (eg, cystadenomas, cystadenocarcinomas), organized hematomas, liver cysts, renal cysts, gallstones, renal stones, calcified lymph nodes, and vessel calcification.
Autosomal dominant polycystic kidney disease is the most common inherited renal disease.1 Typically, patients with this disease have a positive family history, although about 5% to 10% do not.1,3 (Our patient did not.)
The diagnosis is suggested by family history and imaging, with typical findings including large kidneys and extensive cysts scattered throughout both kidneys.1–3 The history, epidemiologic data, and the character of the cystic lesions help in making the final diagnosis.
In patients at risk, the presence of at least two renal cysts (unilateral or bilateral) at age 15 to 30, of at least two cysts in each kidney at age 30 to 59, and of at least four cysts in each kidney at age 60 or older is regarded as sufficient to establish the diagnosis.1–3
Approximately 80% of patients with autosomal dominant polycystic kidney disease also have hepatic cysts.1,3 Cyst calcifications occur in about 25% of patients, either as a residual effect of intracystic hemorrhage or as a consequence of secondary hyperparathyroidism.4,5
A 69-year-old man has been on hemodialysis for about 15 years. Routine plain chest radiographs (Figure 1) show multiple calcified cystic lesions in the upper abdomen. He has no fever or abdominal pain.
Q: Which is the most plausible diagnosis?
- Nephrolithiasis
- Hydatid cyst
- Polycystic kidney and liver disease
- Metastatic calcification
- Intra-abdominal abscesses
- Cystadenoma
A: This patient has polycystic kidney disease. He first came to our hospital 21 years ago because of upper gastrointestinal bleeding, and he was found to have multiple cystic lesions. He was diagnosed as having polycystic kidney disease with multiple hepatic cysts.
About 15 years ago, he had several episodes of gross hematuria with right flank pain, which were attributed to bleeding from the cysts. Because his renal function was deteriorating, he started regular hemodialysis at that time. He underwent parathyroidectomy for secondary hyperparathyroidism 2 years ago.
The differential diagnosis of calcified cystic lesions in the upper abdomen includes infectious diseases (eg, chronic intra-abdominal abscess, echinococcosis, cysticercosis), neoplasms (eg, cystadenomas, cystadenocarcinomas), organized hematomas, liver cysts, renal cysts, gallstones, renal stones, calcified lymph nodes, and vessel calcification.
Autosomal dominant polycystic kidney disease is the most common inherited renal disease.1 Typically, patients with this disease have a positive family history, although about 5% to 10% do not.1,3 (Our patient did not.)
The diagnosis is suggested by family history and imaging, with typical findings including large kidneys and extensive cysts scattered throughout both kidneys.1–3 The history, epidemiologic data, and the character of the cystic lesions help in making the final diagnosis.
In patients at risk, the presence of at least two renal cysts (unilateral or bilateral) at age 15 to 30, of at least two cysts in each kidney at age 30 to 59, and of at least four cysts in each kidney at age 60 or older is regarded as sufficient to establish the diagnosis.1–3
Approximately 80% of patients with autosomal dominant polycystic kidney disease also have hepatic cysts.1,3 Cyst calcifications occur in about 25% of patients, either as a residual effect of intracystic hemorrhage or as a consequence of secondary hyperparathyroidism.4,5
- Braun WE. Autosomal dominant polycystic kidney disease: emerging concepts of pathogenesis and new treatments. Cleve Clin J Med 2009; 76:97–104.
- Torres VE, Bennett WM. Diagnosis of and screening for autosomal dominant polycystic kidney disease. In:Rose BD, editor: UpToDate. Waltham, MA: UpToDate, 2009.
- Grantham JJ. Clinical practice. Autosomal dominant polycystic kidney disease. N Engl J Med 2008; 359:1477–1485.
- Coffin B, Hadengue A, Degos F, Benhamou JP. Calcified hepatic and renal cysts in adult dominant polycystic kidney disease. Dig Dis Sci 1990; 35:1172–1175.
- Levine E, Grantham JJ. Calcified renal stones and cyst calcifications in autosomal dominant polycystic kidney disease: clinical and CT study in 84 patients. AJR Am J Roentgenol 1992; 159:77–81.
- Braun WE. Autosomal dominant polycystic kidney disease: emerging concepts of pathogenesis and new treatments. Cleve Clin J Med 2009; 76:97–104.
- Torres VE, Bennett WM. Diagnosis of and screening for autosomal dominant polycystic kidney disease. In:Rose BD, editor: UpToDate. Waltham, MA: UpToDate, 2009.
- Grantham JJ. Clinical practice. Autosomal dominant polycystic kidney disease. N Engl J Med 2008; 359:1477–1485.
- Coffin B, Hadengue A, Degos F, Benhamou JP. Calcified hepatic and renal cysts in adult dominant polycystic kidney disease. Dig Dis Sci 1990; 35:1172–1175.
- Levine E, Grantham JJ. Calcified renal stones and cyst calcifications in autosomal dominant polycystic kidney disease: clinical and CT study in 84 patients. AJR Am J Roentgenol 1992; 159:77–81.
Multiple huge bullae after renal transplant
A 56-year-old woman presents with multiple huge bullae and crusted erosions in her left sixth to eighth cervical and first thoracic dermatomes (Figure 1), accompanied by severe, sharp, lancinating pain. She underwent renal transplantation 3 months ago for end-stage diabetic kidney disease and is now taking immunosuppressants, including tacrolimus (Prograf) (trough serum level 8–10 ng/dL), mycophenolate mofetil (CellCept) 500 mg twice a day, and prednisolone 5 mg per day.
Q: What is the most likely diagnosis?
- Contact dermatitis
- Herpes zoster
- Herpes simplex
- Pemphigus
- Bullous pemphigoid
- Graft-vs-host disease
A: The correct answer is herpes zoster (shingles), which represents reactivation of varicella-zoster virus.
The diagnosis of herpes zoster is usually based solely on the clinical presentation. It is typically characterized in immunocompetent patients by a unilateral vesicular eruption with a well-defined dermatomal distribution. But occasionally, as in this patient on immunosuppressant drugs, it presents with atypical skin lesions such as multiple huge bullae involving multiple dermatomes.1,2
Patients treated with immunosuppressive agents after organ transplantation are at high risk of herpes zoster. A recent published retrospective study of adult kidney transplant recipients showed an average incidence of approximately 28 per 1,000 person-years.3
Treatment involves analgesics and sometimes antiviral drugs, and the decisions should take into account the patient’s age and immune status.1
This patient was admitted to the hospital and was put in a private room. The lesions were protected from further breakdown and secondary bacterial infection. We discontinued mycophenolate mofetil and prescribed acyclovir (Zovirax) 250 mg intravenously every 8 hours (dose adjusted according to her renal function) for 7 days. Antibiotics needed to be given later for cellulitis that developed as a complication. She had no sign of ophthalmic involvement, visceral involvement, or other complication. She was discharged with healing skin after 42 days of hospitalization (Figure 2) and is free from postherpetic neuralgia.
- Nagel MA, Gilden DH. The protean neurologic manifestations of varicella-zoster virus infection. Cleve Clin J Med 2007; 74:489–504.
- Albrecht MA. Clinical manifestations of varicella-zoster virus infection: Herpes zoster. InRose BD, editor: UpToDate. Waltham, MA: UpToDate, 2008.
- Arness T, Pedersen R, Dierkhising R, Kremers W, Patel R. Varicella zoster virus-associated disease in adult kidney transplant recipients: incidence and risk-factor analysis. Transpl Infect Dis 2008; 10:260–268.
A 56-year-old woman presents with multiple huge bullae and crusted erosions in her left sixth to eighth cervical and first thoracic dermatomes (Figure 1), accompanied by severe, sharp, lancinating pain. She underwent renal transplantation 3 months ago for end-stage diabetic kidney disease and is now taking immunosuppressants, including tacrolimus (Prograf) (trough serum level 8–10 ng/dL), mycophenolate mofetil (CellCept) 500 mg twice a day, and prednisolone 5 mg per day.
Q: What is the most likely diagnosis?
- Contact dermatitis
- Herpes zoster
- Herpes simplex
- Pemphigus
- Bullous pemphigoid
- Graft-vs-host disease
A: The correct answer is herpes zoster (shingles), which represents reactivation of varicella-zoster virus.
The diagnosis of herpes zoster is usually based solely on the clinical presentation. It is typically characterized in immunocompetent patients by a unilateral vesicular eruption with a well-defined dermatomal distribution. But occasionally, as in this patient on immunosuppressant drugs, it presents with atypical skin lesions such as multiple huge bullae involving multiple dermatomes.1,2
Patients treated with immunosuppressive agents after organ transplantation are at high risk of herpes zoster. A recent published retrospective study of adult kidney transplant recipients showed an average incidence of approximately 28 per 1,000 person-years.3
Treatment involves analgesics and sometimes antiviral drugs, and the decisions should take into account the patient’s age and immune status.1
This patient was admitted to the hospital and was put in a private room. The lesions were protected from further breakdown and secondary bacterial infection. We discontinued mycophenolate mofetil and prescribed acyclovir (Zovirax) 250 mg intravenously every 8 hours (dose adjusted according to her renal function) for 7 days. Antibiotics needed to be given later for cellulitis that developed as a complication. She had no sign of ophthalmic involvement, visceral involvement, or other complication. She was discharged with healing skin after 42 days of hospitalization (Figure 2) and is free from postherpetic neuralgia.
A 56-year-old woman presents with multiple huge bullae and crusted erosions in her left sixth to eighth cervical and first thoracic dermatomes (Figure 1), accompanied by severe, sharp, lancinating pain. She underwent renal transplantation 3 months ago for end-stage diabetic kidney disease and is now taking immunosuppressants, including tacrolimus (Prograf) (trough serum level 8–10 ng/dL), mycophenolate mofetil (CellCept) 500 mg twice a day, and prednisolone 5 mg per day.
Q: What is the most likely diagnosis?
- Contact dermatitis
- Herpes zoster
- Herpes simplex
- Pemphigus
- Bullous pemphigoid
- Graft-vs-host disease
A: The correct answer is herpes zoster (shingles), which represents reactivation of varicella-zoster virus.
The diagnosis of herpes zoster is usually based solely on the clinical presentation. It is typically characterized in immunocompetent patients by a unilateral vesicular eruption with a well-defined dermatomal distribution. But occasionally, as in this patient on immunosuppressant drugs, it presents with atypical skin lesions such as multiple huge bullae involving multiple dermatomes.1,2
Patients treated with immunosuppressive agents after organ transplantation are at high risk of herpes zoster. A recent published retrospective study of adult kidney transplant recipients showed an average incidence of approximately 28 per 1,000 person-years.3
Treatment involves analgesics and sometimes antiviral drugs, and the decisions should take into account the patient’s age and immune status.1
This patient was admitted to the hospital and was put in a private room. The lesions were protected from further breakdown and secondary bacterial infection. We discontinued mycophenolate mofetil and prescribed acyclovir (Zovirax) 250 mg intravenously every 8 hours (dose adjusted according to her renal function) for 7 days. Antibiotics needed to be given later for cellulitis that developed as a complication. She had no sign of ophthalmic involvement, visceral involvement, or other complication. She was discharged with healing skin after 42 days of hospitalization (Figure 2) and is free from postherpetic neuralgia.
- Nagel MA, Gilden DH. The protean neurologic manifestations of varicella-zoster virus infection. Cleve Clin J Med 2007; 74:489–504.
- Albrecht MA. Clinical manifestations of varicella-zoster virus infection: Herpes zoster. InRose BD, editor: UpToDate. Waltham, MA: UpToDate, 2008.
- Arness T, Pedersen R, Dierkhising R, Kremers W, Patel R. Varicella zoster virus-associated disease in adult kidney transplant recipients: incidence and risk-factor analysis. Transpl Infect Dis 2008; 10:260–268.
- Nagel MA, Gilden DH. The protean neurologic manifestations of varicella-zoster virus infection. Cleve Clin J Med 2007; 74:489–504.
- Albrecht MA. Clinical manifestations of varicella-zoster virus infection: Herpes zoster. InRose BD, editor: UpToDate. Waltham, MA: UpToDate, 2008.
- Arness T, Pedersen R, Dierkhising R, Kremers W, Patel R. Varicella zoster virus-associated disease in adult kidney transplant recipients: incidence and risk-factor analysis. Transpl Infect Dis 2008; 10:260–268.