Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade cutaneous sarcoma with repo... more Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade cutaneous sarcoma with reported at 3 per 1,000,000 individuals. It is a locally aggressive tumor which originates in the dermis and invades deeper structures. Up to 90% of cases of DFSP are associated with a translocation between chromosomes 17 and 22. These tumors have a high risk of local recurrence but very rarely exhibit metastatic spread. Several histologic variants exist, including fibrosarcomatous DFSP, and in this case, pigmented DSFP. Pigmented dermatofibrosarcoma protuberans, otherwise also known as a Bednar tumor, is a rare variant of DFSP first described in 1956 which accounts for less than 5% of all DSFP cases. It is distinguished by the presence of melanin containing dendritic cells within the tumor. Histologically, tumor cells are arranged in compact spindle shaped bundles. These tumors may be derived from neuroectodermal cells although no consensus has been reached. DSFP occurs equally in men and women and most commonly occurs in young to middle aged adults. These cutaneous sarcomas typically present on the trunk, back and shoulders as a slow-growing mass or plaque that may be hyperpigmented. Less that 1% present on genitalia, and only two DSFP cases to date have been reported involving the perianal region.https://scholarlycommons.henryford.com/merf2019caserpt/1085/thumbnail.jp
tory was referred to dermatology by her primary care provider for evaluation of a hematoma on the... more tory was referred to dermatology by her primary care provider for evaluation of a hematoma on the posterior neck that had developed gradually over 5 months. The lesion initially was asymptomatic but more recently had started to be painful and bleed intermittently. The patient denied any personal or family history of skin cancer. Physical examination revealed a large hemorrhagic plaque on the left side of the posterior neck with central brown-yellow crusting. There were few smaller, white, thin, sclerotic plaques with crinkling atrophy at the periphery of and inferolateral to the lesion. A punch biopsy specimen was obtained from the hemorrhagic plaque.
4313 Introduction Iron overload has increasingly been recognized as a cause of morbidity and mort... more 4313 Introduction Iron overload has increasingly been recognized as a cause of morbidity and mortality among allogenic hematopoietic stem cell transplant (HSCT) recipients. Estimation of liver iron is considered the gold standard method that reflects the overall body iron burden. Aim We attempted to correlate the severity of liver iron overload to various clinical parameters in allogenic HSCT recipients. Methods We studied 49 adult allogenic HSCT recipients who had a minimum of one liver biopsy performed after transplant. All patients had been diagnosed, transplanted and followed up in our institution since 1998. Biopsies were performed due to abnormal liver function tests under the initial clinical suspicion of GVHD with a mean of 185 days (range: 23-844 days) post-transplant. Sections from each biopsy were stained with hematoxylin and eosin, Masson trichome and Perl's iron stains. We used the histological semiquantitative grading of liver iron (grade 0-4), evidence of GVHD (sc...
Desmoplastic melanoma (DM) is a rare fibrosing variant of melanoma that can be difficult to diagn... more Desmoplastic melanoma (DM) is a rare fibrosing variant of melanoma that can be difficult to diagnose. One of the diagnostic challenges is its distinction from a melanocytic nevus with desmoplasia. Here we investigate the use of a 23-gene signature, which has previously been shown to distinguish benign from malignant melanocytic neoplasms. We assessed 50 cases with a differential diagnostic consideration of DM that underwent gene expression testing. Hematoxylin and eosin-stained sections were reviewed, and the final cohort included 20 DMs, 5 nondesmoplastic melanomas, and 27 desmoplastic melanocytic nevi. Of the 20 DMs, the gene expression score was positive ("likely malignant") in 15 tumors, indeterminate in 1, and negative ("likely benign") in 4. None of the desmoplastic melanocytic nevi were positive. The gene expression score was negative in 24 of the melanocytic nevi and indeterminate in the remaining 3. Nine DMs were also analyzed cytogenetically by single-n...
Hepatobiliary & Pancreatic Diseases International, 2011
Nonsteroidal anti-inflammatory drugs (NSAIDs) have been reported to induce liver injury. Patterns... more Nonsteroidal anti-inflammatory drugs (NSAIDs) have been reported to induce liver injury. Patterns of the injury usually range from mild elevations of liver enzymes to sometimes severe fulminant hepatic failure. Likewise, naproxen is a propionic acid derivative NSAID that was introduced in 1980 and has been available as an over-the-counter medication since 1994, but has rarely been reported to cause liver injury. We treated a 30-year-old woman with jaundice and intractable pruritus that developed shortly after taking naproxen. We reviewed the medical history and liver histopathology of the patient as well as all previously published case reports of naproxen-associated liver toxicity in the English language literature. The liver biochemical profile of the patient revealed a mixed cholestasis and hepatitis pattern. Consecutive liver biopsies demonstrated focal lobular inflammation, hepatocyte drop-out, and a progressive loss of the small interlobular bile ducts (ductopenia). The biopsy performed two years after onset of the disease showed partial recovery of a small number of bile ducts; however, 10 years passed before the biochemical profile returned to near normal. Naproxen-associated liver toxicity remains a rare entity, but should be considered in any patient presenting with cholestasis shortly after its use. Liver injury is most commonly seen in a mixed pattern characterized by cholestasis and hepatitis. The resulting liver damage may take years to resolve.
Peritoneal dialysis-associated peritonitis due to fungi of the class Zygomycetes occurs very rare... more Peritoneal dialysis-associated peritonitis due to fungi of the class Zygomycetes occurs very rarely. A case of fungal continuous ambulatory peritoneal dialysis peritonitis due to Cunninghamella bertholletiae is reported in a 39-year-old Aboriginal woman with end-stage renal failure and diabetes mellitus. This isolate was found to be resistant in vitro to amphotericin B, 5-fluorocytosine, fluconazole, itraconazole, ketoconazole and voriconazole. However, this patient was successfully treated with voriconazole and removal of the Tenckhoff dialysis catheter. Zygomycoses are an emerging threat among immunocompromised patients, including those with chronic renal failure. Zygomycosis due to C. bertholletiae is frequently fatal and is often non-responsive to systemic antifungal therapy. This is believed to be the first reported case of C. bertholletiae causing peritonitis in humans and one of the minority of cases involving this organism with a successful outcome.
Pandoraea species are considered emerging pathogens in cystic fibrosis (CF) patients, but few dat... more Pandoraea species are considered emerging pathogens in cystic fibrosis (CF) patients, but few data exist regarding outcomes of patients colonized with these organisms. We report a case of Pandoraea sputorum colonization in a CF patient under consideration for lung transplantation and review five cases of lung transplantation involving Pandoraea species.
Gastroenterology, Volume 138, Issue 5, Pages S-808, May 2010, Authors:Sharif Ali; Jason D. Piment... more Gastroenterology, Volume 138, Issue 5, Pages S-808, May 2010, Authors:Sharif Ali; Jason D. Pimentel; Veena Shah; Javier Munoz; Nalini Janakiraman. ... Sharif Ali ,; Jason D. Pimentel ,; VeenaShah ,; Javier Munoz ,; Nalini Janakiraman. Abstract; PDF. No full text is available. ...
Infection due to Streptococcus agalactiae or Group B streptococcus may be acquired during parturi... more Infection due to Streptococcus agalactiae or Group B streptococcus may be acquired during parturition or gestation. Discordant twin gestational Group B streptococcus infections are rarely reported. We describe two cases of fatal discordant gestational Group B streptococcus infection in both a monochorionic/diamniotic pregnancy and in a dichorionic/diamniotic pregnancy. In both instances, cultures, examination of the placentae, and autopsy findings demonstrated infection by Group B streptococcus in only the presenting fetus. Despite the ubiquity of this organism, this is the first documented case of discordant gestational Group B streptococcus infection in monochorionic/diamniotic twins and only the third case documented in dichorionic/diamniotic twins.
Background: Psoriasis is a chronic, inflammatory disease affecting 2-3% of the worldwide populati... more Background: Psoriasis is a chronic, inflammatory disease affecting 2-3% of the worldwide population, and it may worsen with HIV or be detected as HIV cutaneous manifestation. HIV-related psoriasis shows a severe and prolonged clinical course with more frequent exacerbations. The management of this condition is challenging because im-munomodulating and immunosuppressant agents may have variable and partial efficacy, and therefore, antiretroviral treatment represents a potential adjunctive therapeutic option. Results: In the case we report, the HIV test was shown to be crucial for driving the therapeutic approach. Indeed, antiretroviral agents have been proven to be effective in the treatment of HIV+ psoriasis as first-line therapy. Conclusion: The HIV test should be considered in high-risk patients affected by severe psoriasis and resistant to conventional and biological treatments.
Archives of Pathology & Laboratory Medicine, 2012
Context.—Patients who undergo hematopoietic stem cell transplant are at an increased risk of deve... more Context.—Patients who undergo hematopoietic stem cell transplant are at an increased risk of developing iron overload. Objectives.—To describe the effect of hepatic iron overload on hematopoietic stem cell transplant recipients and to validate the utility of histologic scoring system of iron granules in the liver. Design.—Records of 154 post allogeneic hematopoietic stem cell transplant patients were reviewed. Forty-nine patients underwent liver biopsy. Histologic hepatic iron overload was defined as a score of 2 or greater (scale, 0–4). Results.—Twenty-eight of 49 patients (57%) evaluated by liver biopsy had hepatic iron overload; 17 had moderate to severe hepatic iron overload (score, 3 or 4). In multivariate analysis, a significant correlation was discovered between hepatic iron overload and the number of transfusions (P < .001), posttransplant serum ferritin levels (P = .004), lactate dehydrogenase levels (P = .03), and the development of blood stream infections (P = .0...
Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade cutaneous sarcoma with repo... more Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade cutaneous sarcoma with reported at 3 per 1,000,000 individuals. It is a locally aggressive tumor which originates in the dermis and invades deeper structures. Up to 90% of cases of DFSP are associated with a translocation between chromosomes 17 and 22. These tumors have a high risk of local recurrence but very rarely exhibit metastatic spread. Several histologic variants exist, including fibrosarcomatous DFSP, and in this case, pigmented DSFP. Pigmented dermatofibrosarcoma protuberans, otherwise also known as a Bednar tumor, is a rare variant of DFSP first described in 1956 which accounts for less than 5% of all DSFP cases. It is distinguished by the presence of melanin containing dendritic cells within the tumor. Histologically, tumor cells are arranged in compact spindle shaped bundles. These tumors may be derived from neuroectodermal cells although no consensus has been reached. DSFP occurs equally in men and women and most commonly occurs in young to middle aged adults. These cutaneous sarcomas typically present on the trunk, back and shoulders as a slow-growing mass or plaque that may be hyperpigmented. Less that 1% present on genitalia, and only two DSFP cases to date have been reported involving the perianal region.https://scholarlycommons.henryford.com/merf2019caserpt/1085/thumbnail.jp
tory was referred to dermatology by her primary care provider for evaluation of a hematoma on the... more tory was referred to dermatology by her primary care provider for evaluation of a hematoma on the posterior neck that had developed gradually over 5 months. The lesion initially was asymptomatic but more recently had started to be painful and bleed intermittently. The patient denied any personal or family history of skin cancer. Physical examination revealed a large hemorrhagic plaque on the left side of the posterior neck with central brown-yellow crusting. There were few smaller, white, thin, sclerotic plaques with crinkling atrophy at the periphery of and inferolateral to the lesion. A punch biopsy specimen was obtained from the hemorrhagic plaque.
4313 Introduction Iron overload has increasingly been recognized as a cause of morbidity and mort... more 4313 Introduction Iron overload has increasingly been recognized as a cause of morbidity and mortality among allogenic hematopoietic stem cell transplant (HSCT) recipients. Estimation of liver iron is considered the gold standard method that reflects the overall body iron burden. Aim We attempted to correlate the severity of liver iron overload to various clinical parameters in allogenic HSCT recipients. Methods We studied 49 adult allogenic HSCT recipients who had a minimum of one liver biopsy performed after transplant. All patients had been diagnosed, transplanted and followed up in our institution since 1998. Biopsies were performed due to abnormal liver function tests under the initial clinical suspicion of GVHD with a mean of 185 days (range: 23-844 days) post-transplant. Sections from each biopsy were stained with hematoxylin and eosin, Masson trichome and Perl's iron stains. We used the histological semiquantitative grading of liver iron (grade 0-4), evidence of GVHD (sc...
Desmoplastic melanoma (DM) is a rare fibrosing variant of melanoma that can be difficult to diagn... more Desmoplastic melanoma (DM) is a rare fibrosing variant of melanoma that can be difficult to diagnose. One of the diagnostic challenges is its distinction from a melanocytic nevus with desmoplasia. Here we investigate the use of a 23-gene signature, which has previously been shown to distinguish benign from malignant melanocytic neoplasms. We assessed 50 cases with a differential diagnostic consideration of DM that underwent gene expression testing. Hematoxylin and eosin-stained sections were reviewed, and the final cohort included 20 DMs, 5 nondesmoplastic melanomas, and 27 desmoplastic melanocytic nevi. Of the 20 DMs, the gene expression score was positive ("likely malignant") in 15 tumors, indeterminate in 1, and negative ("likely benign") in 4. None of the desmoplastic melanocytic nevi were positive. The gene expression score was negative in 24 of the melanocytic nevi and indeterminate in the remaining 3. Nine DMs were also analyzed cytogenetically by single-n...
Hepatobiliary & Pancreatic Diseases International, 2011
Nonsteroidal anti-inflammatory drugs (NSAIDs) have been reported to induce liver injury. Patterns... more Nonsteroidal anti-inflammatory drugs (NSAIDs) have been reported to induce liver injury. Patterns of the injury usually range from mild elevations of liver enzymes to sometimes severe fulminant hepatic failure. Likewise, naproxen is a propionic acid derivative NSAID that was introduced in 1980 and has been available as an over-the-counter medication since 1994, but has rarely been reported to cause liver injury. We treated a 30-year-old woman with jaundice and intractable pruritus that developed shortly after taking naproxen. We reviewed the medical history and liver histopathology of the patient as well as all previously published case reports of naproxen-associated liver toxicity in the English language literature. The liver biochemical profile of the patient revealed a mixed cholestasis and hepatitis pattern. Consecutive liver biopsies demonstrated focal lobular inflammation, hepatocyte drop-out, and a progressive loss of the small interlobular bile ducts (ductopenia). The biopsy performed two years after onset of the disease showed partial recovery of a small number of bile ducts; however, 10 years passed before the biochemical profile returned to near normal. Naproxen-associated liver toxicity remains a rare entity, but should be considered in any patient presenting with cholestasis shortly after its use. Liver injury is most commonly seen in a mixed pattern characterized by cholestasis and hepatitis. The resulting liver damage may take years to resolve.
Peritoneal dialysis-associated peritonitis due to fungi of the class Zygomycetes occurs very rare... more Peritoneal dialysis-associated peritonitis due to fungi of the class Zygomycetes occurs very rarely. A case of fungal continuous ambulatory peritoneal dialysis peritonitis due to Cunninghamella bertholletiae is reported in a 39-year-old Aboriginal woman with end-stage renal failure and diabetes mellitus. This isolate was found to be resistant in vitro to amphotericin B, 5-fluorocytosine, fluconazole, itraconazole, ketoconazole and voriconazole. However, this patient was successfully treated with voriconazole and removal of the Tenckhoff dialysis catheter. Zygomycoses are an emerging threat among immunocompromised patients, including those with chronic renal failure. Zygomycosis due to C. bertholletiae is frequently fatal and is often non-responsive to systemic antifungal therapy. This is believed to be the first reported case of C. bertholletiae causing peritonitis in humans and one of the minority of cases involving this organism with a successful outcome.
Pandoraea species are considered emerging pathogens in cystic fibrosis (CF) patients, but few dat... more Pandoraea species are considered emerging pathogens in cystic fibrosis (CF) patients, but few data exist regarding outcomes of patients colonized with these organisms. We report a case of Pandoraea sputorum colonization in a CF patient under consideration for lung transplantation and review five cases of lung transplantation involving Pandoraea species.
Gastroenterology, Volume 138, Issue 5, Pages S-808, May 2010, Authors:Sharif Ali; Jason D. Piment... more Gastroenterology, Volume 138, Issue 5, Pages S-808, May 2010, Authors:Sharif Ali; Jason D. Pimentel; Veena Shah; Javier Munoz; Nalini Janakiraman. ... Sharif Ali ,; Jason D. Pimentel ,; VeenaShah ,; Javier Munoz ,; Nalini Janakiraman. Abstract; PDF. No full text is available. ...
Infection due to Streptococcus agalactiae or Group B streptococcus may be acquired during parturi... more Infection due to Streptococcus agalactiae or Group B streptococcus may be acquired during parturition or gestation. Discordant twin gestational Group B streptococcus infections are rarely reported. We describe two cases of fatal discordant gestational Group B streptococcus infection in both a monochorionic/diamniotic pregnancy and in a dichorionic/diamniotic pregnancy. In both instances, cultures, examination of the placentae, and autopsy findings demonstrated infection by Group B streptococcus in only the presenting fetus. Despite the ubiquity of this organism, this is the first documented case of discordant gestational Group B streptococcus infection in monochorionic/diamniotic twins and only the third case documented in dichorionic/diamniotic twins.
Background: Psoriasis is a chronic, inflammatory disease affecting 2-3% of the worldwide populati... more Background: Psoriasis is a chronic, inflammatory disease affecting 2-3% of the worldwide population, and it may worsen with HIV or be detected as HIV cutaneous manifestation. HIV-related psoriasis shows a severe and prolonged clinical course with more frequent exacerbations. The management of this condition is challenging because im-munomodulating and immunosuppressant agents may have variable and partial efficacy, and therefore, antiretroviral treatment represents a potential adjunctive therapeutic option. Results: In the case we report, the HIV test was shown to be crucial for driving the therapeutic approach. Indeed, antiretroviral agents have been proven to be effective in the treatment of HIV+ psoriasis as first-line therapy. Conclusion: The HIV test should be considered in high-risk patients affected by severe psoriasis and resistant to conventional and biological treatments.
Archives of Pathology & Laboratory Medicine, 2012
Context.—Patients who undergo hematopoietic stem cell transplant are at an increased risk of deve... more Context.—Patients who undergo hematopoietic stem cell transplant are at an increased risk of developing iron overload. Objectives.—To describe the effect of hepatic iron overload on hematopoietic stem cell transplant recipients and to validate the utility of histologic scoring system of iron granules in the liver. Design.—Records of 154 post allogeneic hematopoietic stem cell transplant patients were reviewed. Forty-nine patients underwent liver biopsy. Histologic hepatic iron overload was defined as a score of 2 or greater (scale, 0–4). Results.—Twenty-eight of 49 patients (57%) evaluated by liver biopsy had hepatic iron overload; 17 had moderate to severe hepatic iron overload (score, 3 or 4). In multivariate analysis, a significant correlation was discovered between hepatic iron overload and the number of transfusions (P < .001), posttransplant serum ferritin levels (P = .004), lactate dehydrogenase levels (P = .03), and the development of blood stream infections (P = .0...
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