Nephrogenic fibrosing dermopathy (NFD) and calciphylaxis are rare conditions that are associated with chronic kidney disease. Histopathologic changes, including dystrophic dermal calcification, often in association with elastic fibers have been observed in NFD and calciphylaxis. A pattern of dermal elastic fiber calcification that mimics pseudoxanthoma elasticum (PXE) has been previously reported as an incidental finding in the setting of calciphylaxis. Despite a shared association with renal disease and abnormal calcium deposits, however, NFD and calciphylaxis are discrete pathologic processes with distinct clinical and histopathologic features. Criteria for each are reviewed through case presentation of a patient meeting the clinical and histopathologic criteria for both NFD and calciphylaxis with histologic features mimicking PXE.
We propose the term 'spiradenocylindrocarcinoma' to describe malignant tumors with features of both a spiradenoma and a cylindroma. In conjunction with histological features of malignancy, p53 and Ki-67 immunohistochemical staining may provide helpful clues in determining the malignant potential of this tumor. Further studies are needed to clarify the biologic behavior of such lesions.
The clinical and histopathologic features of this case are consistent with a diagnosis of linear NC with EHK. Lesions of NC suspected by clinical exam should be examined microscopically to look for features of EHK. If present, patients should be educated about the risk, albeit rare, of passing on a more severe form of the disorder to subsequent generations.
Viral associated trichodysplasia spinulosa (VATS) is a rare cutaneous eruption characterized by folliculocentric papules, keratin spicules, and alopecia associated with trichodysplasia spinulosa-associated polyomavirus (TSPyV) infection. We report a case of a 6-year-old male child who presented with a generalized papular eruption during chemotherapy for acute lymphoblastic leukemia. The papules were tested for human papillomavirus (HPV) DNA by nested polymerase chain reaction (PCR) and TSPyV using PCR and gene sequencing studies. The lesions were positive for TSPyV by PCR combined with sequencing and showed high copy number with real-time PCR, and beta-papillomavirus was identified by PCR and sequencing. Immunohistochemistry revealed inner root sheath keratinocytes expressing nuclear HPV L1 capsid antigen. To our knowledge, this is the first case of concomitant productive HPV and TSPyV infection in a VATS-affected patient. The presence of HPV may be coincidental, however, further studies are needed to establish whether specific HPV genotypes influence the development of abnormal inner root sheath trichohyalin granules found in VATS.
A previous study of 100 karyotyped metaphase cells has demonstrated the utility of a graphic arts tool in deriving chromosome measurements for relative length determination. In the present study we utilize this same approach to address the question: "What are the average differences in relative lengths between apparently normal homologous chromosomes?" Normal standards derived from this study will be useful for testing specific hypotheses involving heteromorphic differences between homologs. No such data on normal controls could readily be found in either An International System for Human Cytogenetic Nomenclature (1985) or elsewhere.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.