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2.
Arch Pathol Lab Med ; 2020 Dec 08.
Artigo em Inglês | MEDLINE | ID: mdl-33290514

RESUMO

CONTEXT.­: Pathology case volume and complexity impact clinical service burden, staffing, and reimbursement, particularly in an academic setting. OBJECTIVE.­: To investigate dermatopathology case complexity by using indicators of challenging cases, which require increased clinical service effort. DESIGN.­: A retrospective review was performed of dermatopathology cases during a 9-year period at a tertiary care academic center. A subset of cases was analyzed for which extractable data were available. Cases requiring the following metrics of complexity were identified: rush processing, consensus agreement, performance of immunohistochemistry, use of special histochemical stains, use of immunofluorescence, examination of additional tissue levels, review of a prior case, addition of an explanatory note, presence of multiple specimen parts, and use of intradepartmental consultation. RESULTS.­: A total of 8173 cases were reviewed. During the same 3-month period of the year, there was a statistically significant increase in use of rush processing/interpretation, consensus review, number of cases requiring immunostains, special stains, levels, and an explanatory note, and cases reviewed by other subspecialists in the department from 2010 to 2019. CONCLUSIONS.­: This study shows an increasing trend in dermatopathology case complexity, suggesting that overall clinical service efforts have increased. These findings may inform clinical service staffing and reimbursement.

3.
Am J Dermatopathol ; 2020 Nov 03.
Artigo em Inglês | MEDLINE | ID: mdl-33156025

RESUMO

Cutaneous reactions surrounding abdominal stoma sites are typically irritant, allergic, infectious, traumatic or pathergic in etiology. Pemphigus, which encompasses a group of vesiculobullous autoimmune skin disorders, is seldom encountered as a peristomal dermatosis. Direct immunofluorescence (DIF) studies of pemphigus generally show continuous intercellular net-like depositions of IgG. However, punctate or dot-like intercellular deposition of IgG can also be seen in cases of pemphigus. The punctuate pattern is underreported in the literature and little is known about its implication. We describe a case of a 58-year-old Caucasian man with a history of bowel obstruction, status postcolostomy, who presented with a sharply demarcated, erythematous, crusted plaque surrounding his abdominal stoma. The patient endorsed persistent pruritus. A punch biopsy of the lesion was performed for clinical suspicion of fungal infection versus irritant dermatitis. Histopathology revealed a predominantly subcorneal acantholytic dermatitis. Periodic acid-Schiff with diastase and Grocott methenamine silver histochemical stains were negative for fungi. DIF was positive for IgG and C3 detected in a punctate intercellular pattern. In conjunction with the patient's clinical presentation and DIF, a diagnosis of peristomal pemphigus foliaceous was rendered. Herein, we describe a case of punctate pemphigus presenting as a peristomal dermatosis and include a review of the literature to raise awareness of this phenomenon.

5.
Neurooncol Adv ; 2(Suppl 1): i117-i123, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32642737

RESUMO

Background: Cutaneous neurofibromas (cNFs) are the most common tumors in people with neurofibromatosis type 1 (NF1) and are associated with reduced quality of life. There is currently no widely accepted standardized language for describing cNFs clinically or histopathologically. The objective of this study was to evaluate interobserver agreement across pathologists in describing and reporting of neurofibromas involving the skin. Methods: Twenty-eight (H&E)-stained slides of cNF were scanned using an Aperio XT scanner. The digital images were reviewed by 6 pathologists, who entered free text of up to a 200 word description for each case into a REDcap database. Responses were analyzed for the most commonly used terms based on frequency, as well as agreement (reported as concordance) between reviewers. Results: A set of the terms most commonly used by pathologists for the histological classification of cNF along with areas of agreement and disagreement have been identified. The study shows that there was strong agreement across reviewers that not all neurofibromas involving the skin are cutaneous neurofibromas and regarding the presence or absence of atypical features and heterologous elements. Areas of less concordance were identified and include cNF subtypes, definition of extension and pattern of growth, as well as the distinction of a cNF from a plexiform without an intraneural component involving skin. Conclusions: This work is the first step towards development of a robust classification system and devising "gold standard" histopathologic diagnostic criteria for cutaneous neurofibromas.

6.
Am J Dermatopathol ; 42(8): 557-563, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32701689

RESUMO

Calciphylaxis is a highly morbid disease that is strongly associated with chronic kidney disease (CKD); however, the histologic criteria for diagnosis have not been well established nor have their specificity for calciphylaxis been determined. This retrospective study aimed to examine the prevalence of histologic features commonly associated with calciphylaxis in noncalciphylaxis patients. We also sought to evaluate whether these features may be more prevalent in patients with CKD. To assess this, healthy marginal skin tissue from above-the-knee amputation specimens was compared between patients with CKD (n = 23) and without CKD (n = 47). Intravascular calcification of capillaries or small-to-medium arterioles was detected on von Kossa stain in 40.0% of the entire cohort. Capillary calcification and intravascular thrombosis were more prevalent in patients with CKD. Finely stippled capillary calcification was present in 26.1% of patients with CKD versus 8.5% of patients without CKD (P = 0.0484), and intravascular thrombosis was present in 8.7% of patients with CKD and 0.0% of patients without CKD (P = 0.0403). None of the patients in this study had clinical evidence of calciphylaxis at presentation and in at least a 1-year follow-up period. This study confirms that the histologic features previously associated with calciphylaxis are nonspecific and are more prevalent in patients with CKD.

7.
Clin Appl Thromb Hemost ; 26: 1076029620943671, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32702995

RESUMO

Severe acute respiratory syndrome coronavirus 2 infection (COVID-19) is known to induce severe inflammation and activation of the coagulation system, resulting in a prothrombotic state. Although inflammatory conditions and organ-specific diseases have been shown to be strong determinants of morbidity and mortality in patients with COVID-19, it is unclear whether preexisting differences in coagulation impact the severity of COVID-19. African Americans have higher rates of COVID-19 infection and disease-related morbidity and mortality. Moreover, African Americans are known to be at a higher risk for thrombotic events due to both biological and socioeconomic factors. In this review, we explore whether differences in baseline coagulation status and medical management of coagulation play an important role in COVID-19 disease severity and contribute to racial disparity trends within COVID-19.


Assuntos
Afro-Americanos , Betacoronavirus , Infecções por Coronavirus/etnologia , Pandemias , Pneumonia Viral/etnologia , Trombofilia/etnologia , Tromboembolia Venosa/etnologia , Afro-Americanos/genética , Anemia Falciforme/sangue , Anemia Falciforme/etnologia , Anticoagulantes/uso terapêutico , Proteínas Sanguíneas/análise , Proteínas Sanguíneas/genética , Ensaios Clínicos como Assunto , Comorbidade , Infecções por Coronavirus/sangue , Infecções por Coronavirus/complicações , Fator VIII/análise , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Estudos de Associação Genética , Predisposição Genética para Doença , Disparidades em Assistência à Saúde , Humanos , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/etnologia , Masculino , Seleção de Pacientes , Pneumonia Viral/sangue , Pneumonia Viral/complicações , Polimorfismo de Nucleotídeo Único , Prevalência , Insuficiência Renal Crônica/sangue , Insuficiência Renal Crônica/etnologia , Fatores de Risco , Determinantes Sociais da Saúde , Fatores Socioeconômicos , Trombofilia/sangue , Trombofilia/tratamento farmacológico , Trombofilia/etiologia , Tromboembolia Venosa/sangue , Tromboembolia Venosa/etiologia , Tromboembolia Venosa/prevenção & controle
8.
Nature ; 582(7811): 234-239, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32499652

RESUMO

On average, Peruvian individuals are among the shortest in the world1. Here we show that Native American ancestry is associated with reduced height in an ethnically diverse group of Peruvian individuals, and identify a population-specific, missense variant in the FBN1 gene (E1297G) that is significantly associated with lower height. Each copy of the minor allele (frequency of 4.7%) reduces height by 2.2 cm (4.4 cm in homozygous individuals). To our knowledge, this is the largest effect size known for a common height-associated variant. FBN1 encodes the extracellular matrix protein fibrillin 1, which is a major structural component of microfibrils. We observed less densely packed fibrillin-1-rich microfibrils with irregular edges in the skin of individuals who were homozygous for G1297 compared with individuals who were homozygous for E1297. Moreover, we show that the E1297G locus is under positive selection in non-African populations, and that the E1297 variant shows subtle evidence of positive selection specifically within the Peruvian population. This variant is also significantly more frequent in coastal Peruvian populations than in populations from the Andes or the Amazon, which suggests that short stature might be the result of adaptation to factors that are associated with the coastal environment in Peru.


Assuntos
Estatura/genética , Fibrilina-1/genética , Mutação de Sentido Incorreto , Seleção Genética , Feminino , Frequência do Gene , Estudo de Associação Genômica Ampla , Hereditariedade , Humanos , Índios Sul-Americanos/genética , Masculino , Microfibrilas/química , Microfibrilas/genética , Peru
10.
Am J Dermatopathol ; 42(11): 842-847, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32310858

RESUMO

Hypertrophic scars and keloids are fibroproliferative lesions characterized by excessive collagen deposition. It is unclear whether these entities represent distinct disorders or share a common pathogenesis and the molecular underpinnings of these lesions are poorly understood. Accumulating evidence suggests that the Wnt signaling pathway is a key regulator of wound healing. In this study, tissue microarray was used to evaluate the protein expression profile for Wnt3a, phosphorylated glycogen synthase kinase 3 alpha (pGSK-3α), WNT1-inducible-signaling pathway protein 1 (WISP1), and WISP2 in normal skin, scars, hypertrophic scars, and keloids. Analysis revealed significantly increased fibroblast expression of pGSK-3α in scars (27.2%), hypertrophic scars (30.4%), and keloids (57.3%) compared with normal skin (16.4%) (all differences statistically significant; P < 0.01). Analysis of WISP2 showed 94% of fibroblasts in normal skin expressing WISP2 and significantly decreased expression in scars (46.8%), hypertrophic scars (27.0%), and keloids (61.3%) (all differences statistically significant; P < 0.01). The parallel patterns of expression of pGSK-3α and WISP2 in scars and hypertrophic scars and significantly increased expression in keloids may support the notion that keloids are a truly distinct fibrosing disorder and may provide further evidence for targeting the Wnt signaling pathway in the treatment of keloids.

11.
World Neurosurg ; 138: e17-e25, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32142948

RESUMO

BACKGROUND: Eccrine carcinoma involvement of the central nervous system (CNS) is exceedingly rare. The prognosis and response to treatment of this pathology remain poorly characterized. METHODS: A retrospective case series and literature review were conducted. RESULTS: CNS-invading eccrine carcinoma was diagnosed in 3 patients (2 male and 1 female; age range, 60-79 years), including 2 cases of brain metastases and 1 case of brain-invading skull metastasis with subsequent spinal metastasis. The interval from primary tumor to CNS invasion was 18-51 months. All patients received multimodal therapy following diagnosis of CNS involvement. One patient who harbored a NOTCH1 mutation demonstrated a durable oncologic response after treatment with the immune checkpoint inhibitor pembrolizumab and lived 39 months after CNS invasion. The other 2 patients were discharged to hospice care within 1 month after the diagnosis of eccrine carcinoma brain metastasis. Including this case series, 23 cases of eccrine carcinoma invasion or metastasis to the CNS have been reported, with survival after diagnosis of CNS involvement ranging from a few weeks to 4 years. CONCLUSIONS: We present 3 cases of eccrine carcinoma metastatic to the CNS, including the first reported case to our knowledge of eccrine carcinoma treated with immunotherapy. This case, harboring a NOTCH1 mutation, demonstrated the longest durable oncologic response reported in this rare disease. Genomic and molecular testing may play increasingly important roles in the evaluation of these metastases.


Assuntos
Neoplasias Encefálicas/secundário , Carcinoma/secundário , Glândulas Écrinas , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias Cranianas/secundário , Neoplasias da Coluna Vertebral/secundário , Neoplasias das Glândulas Sudoríparas/patologia , Parede Abdominal , Idoso , Anticorpos Monoclonais Humanizados/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/tratamento farmacológico , Neoplasias Encefálicas/genética , Carcinoma/tratamento farmacológico , Carcinoma/genética , Carcinoma Ductal/secundário , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica , Receptor Notch1/genética , Couro Cabeludo , Neoplasias Cranianas/diagnóstico por imagem , Neoplasias da Coluna Vertebral/diagnóstico por imagem
12.
J Cutan Pathol ; 47(7): 654-658, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32119141

RESUMO

Drug-induced subacute cutaneous lupus erythematosus (DI-SCLE) is an uncommon but well-described phenomenon, most often seen in association with antihypertensives and antifungal medications. In recent years, rare reports of DI-SCLE have been described in patients being treated with targeted therapies. Herein, we describe a case of DI-SCLE in association with palbociclib and letrozole treatment for metastatic breast cancer. This report is the first known case of DI-SCLE with positive anti-Ro antibodies in this setting. We also summarize the literature describing DI-SCLE in association with targeted therapies to date and its possible association with dysregulation of the vascular endothelial growth factor pathway.

13.
J Cutan Pathol ; 47(2): 166-170, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31469433

RESUMO

Caspase Recruitment Domain Family Member 9 (CARD9) is an adaptor molecule that drives antifungal activity of macrophages and neutrophils in the skin. Autosomal recessive loss-of-function mutations in CARD9 confer increased susceptibility to invasive disease with select fungi in non-immunosuppressed patients. We report on a patient with X-linked ichthyosis complicated by chronic cutaneous invasive dermatophyte infection. We identified a previously reported c.271T>C (p.Y91H) mutation and a novel intronic c.1269+18G>A mutation in CARD9 underlying recurrent deep dermatophytosis in this patient despite various antifungals for over three decades. Our case highlights susceptibility to invasive dermatophytosis related to autosomal recessive CARD9 deficiency and illustrates the range of CARD9 mutations to be pursued in immunocompetent patients with unexplained deep dermatophyte infections. Further studies are needed to define the best therapeutic regimen.


Assuntos
Proteínas Adaptadoras de Sinalização CARD/genética , Candidíase Mucocutânea Crônica , Doenças Genéticas Ligadas ao Cromossomo X , Mutação com Perda de Função , Tinha do Couro Cabeludo , Adulto , Candidíase Mucocutânea Crônica/genética , Candidíase Mucocutânea Crônica/patologia , Doença Crônica , Doenças Genéticas Ligadas ao Cromossomo X/genética , Doenças Genéticas Ligadas ao Cromossomo X/patologia , Humanos , Ictiose/genética , Ictiose/patologia , Masculino , Tinha do Couro Cabeludo/genética , Tinha do Couro Cabeludo/patologia
14.
J Womens Health (Larchmt) ; 29(4): 550-560, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31687866

RESUMO

Background: Gender-related differences have been found among invited speakers in select professional and medical societies. We examined whether similar disparities existed among keynote speakers, plenary speakers, and invited lecturers in a broad range of medical specialty conferences from 2013 to 2017. Materials and Methods: A cross-sectional study was performed on 27 U.S. medical specialty conferences for which data were available on plenary speakers, keynote speakers, and/or invited lecturers. For each speaker, gender and degree(s) were determined. Fisher's exact test was performed to compare proportions of women among speakers to Association of American Medical Colleges' (AAMC) physician workforce data on gender distribution. Results: In aggregate, we identified 246 women among 984 speakers, significantly lower than expected when compared with 2015 AAMC data (25.0% vs. 34.0%; p < 0.00001). Compared with AAMC data reported in 2013, 2015, and 2017, women were significantly underrepresented in 2013 (p = 0.0064) and 2015 (p = 0.00004). In 2017, the proportion of women among invited speakers trended lower than AAMC active women physicians but did not reach significance (p = 0.309). Analysis of individual conference data stratified by year indicated that, while the representation of women among all speakers improved between 2015 and 2017, the representation of women among keynote speakers, plenary speakers, and invited lectureships was variable (including zero levels some years during the study period) and remained lower than expected as compared with workforce data for specific medical specialties. Conclusions: Evaluating for and improving disparities is recommended to ensure gender equity among invited speakers across all medical specialty conferences.


Assuntos
Congressos como Assunto/estatística & dados numéricos , Médicas/estatística & dados numéricos , Sociedades Médicas/organização & administração , Estudos Transversais , Feminino , Humanos , Fatores Sexuais , Estados Unidos , Recursos Humanos/estatística & dados numéricos
15.
Mod Pathol ; 33(6): 1092-1103, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-31857679

RESUMO

Microcystic adnexal carcinoma is a locally aggressive sweat gland carcinoma characterized by its infiltrative growth and histopathologic overlap with benign adnexal tumors, often posing challenges to both diagnosis and management. Understanding the molecular underpinnings of microcystic adnexal carcinoma may allow for more accurate diagnosis and identify potential targetable oncogenic drivers. We characterized 18 microcystic adnexal carcinomas by targeted, multiplexed PCR-based DNA next-generation sequencing of the coding sequence of over 400 cancer-relevant genes. The majority of cases had relatively few (<8) prioritized somatic mutations, and lacked an ultraviolet (UV) signature. The most recurrent mutation was TP53 inactivation in four (22%) tumors. Frame-preserving insertions affecting the kinase domain of JAK1 were detected in three (17%) cases, and were nonoverlapping with TP53 mutations. Seven (39%) cases demonstrated copy number gain of at least one oncogene. By immunohistochemistry, p53 expression was significantly higher in microcystic adnexal carcinomas with TP53 mutations compared with those without such mutations and syringomas. Similarly, phospho-STAT3 expression was significantly higher in microcystic adnexal carcinomas harboring JAK1 kinase insertions compared with those with wild-type JAK1 and syringomas. In conclusion, microcystic adnexal carcinomas are molecularly heterogeneous tumors, with inactivated p53 or activated JAK/STAT signaling in a subset. Unlike most other nonmelanoma skin cancers involving sun-exposed areas, most microcystic adnexal carcinomas lack evidence of UV damage, and hence likely originate from a relatively photo-protected progenitor population in the dermis. These findings have implications for the biology, diagnosis, and treatment of microcystic adnexal carcinomas, including potential for therapeutic targeting of p53 or the JAK/STAT pathway in advanced tumors.

16.
Am J Dermatopathol ; Publish Ahead of Print2020 Dec 29.
Artigo em Inglês | MEDLINE | ID: mdl-33395040

RESUMO

ABSTRACT: Distinguishing hypertrophic lichen planus (HLP) and squamous cell carcinoma (SCC) can be diagnostically challenging because of overlapping clinical and histopathological features. This study characterizes histopathological features in HLP and SCC, assessing their utility in diagnosing atypical squamous proliferations. We compared 12 histopathological features of 15 HLP and 11 SCC biopsies from the lower extremities. We then reviewed 16 cases that were diagnosed as atypical squamous proliferations with differential diagnoses of HLP versus SCC. Clinical follow-up allowed for retrospective categorization of these difficult cases as HLP or SCC. HLP showed significant differences in hyperorthokeratosis (P = 0.04), wedge-shaped hypergranulosis (P = 0.0033), and irregular psoriasiform hyperplasia (P = 0.004), whereas parakeratosis (P = 0.001), solar elastosis (P = 0.001), deep extension (P = 0.02), and perforating elastic fibers (P = 0.0001) were significant for SCC. A scoring system based on these significant differences was devised to aid the classification of difficult cases. 56% of the difficult cases received an "indeterminate" score. A score favoring HLP had a sensitivity of 44% and a specificity of 71%. Although significant differences were identified between cases of definitive HLP and SCC, these histopathological features were unable to distinguish difficult cases, highlighting the need for clinicopathological correlation in patients with atypical squamous proliferations of the lower extremities. Many difficult cases had histologic features that could not be evaluated because of the superficial nature of the biopsy. Therefore, obtaining a deep wedge or punch biopsy may facilitate a diagnosis in cases with a differential diagnosis of HLP and SCC.

18.
Nature ; 575(7783): 519-522, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31666702

RESUMO

Immunosuppression increases the risk of cancers that are associated with viral infection1. In particular, the risk of squamous cell carcinoma of the skin-which has been associated with beta human papillomavirus (ß-HPV) infection-is increased by more than 100-fold in immunosuppressed patients2-4. Previous studies have not established a causative role for HPVs in driving the development of skin cancer. Here we show that T cell immunity against commensal papillomaviruses suppresses skin cancer in immunocompetent hosts, and the loss of this immunity-rather than the oncogenic effect of HPVs-causes the markedly increased risk of skin cancer in immunosuppressed patients. To investigate the effects of papillomavirus on carcinogen-driven skin cancer, we colonized several strains of immunocompetent mice with mouse papillomavirus type 1 (MmuPV1)5. Mice with natural immunity against MmuPV1 after colonization and acquired immunity through the transfer of T cells from immune mice or by MmuPV1 vaccination were protected against skin carcinogenesis induced by chemicals or by ultraviolet radiation in a manner dependent on CD8+ T cells. RNA and DNA in situ hybridization probes for 25 commensal ß-HPVs revealed a significant reduction in viral activity and load in human skin cancer compared with the adjacent healthy skin, suggesting a strong immune selection against virus-positive malignant cells. Consistently, E7 peptides from ß-HPVs activated CD8+ T cells from unaffected human skin. Our findings reveal a beneficial role for commensal viruses and establish a foundation for immune-based approaches that could block the development of skin cancer by boosting immunity against the commensal HPVs present in all of our skin.


Assuntos
Carcinoma de Células Escamosas/imunologia , Carcinoma de Células Escamosas/prevenção & controle , Papillomaviridae/imunologia , Infecções por Papillomavirus/imunologia , Infecções por Papillomavirus/virologia , Neoplasias Cutâneas/prevenção & controle , Neoplasias Cutâneas/virologia , Simbiose , Idoso , Idoso de 80 Anos ou mais , Animais , Linfócitos T CD8-Positivos/imunologia , Carcinogênese/efeitos da radiação , Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/virologia , Feminino , Humanos , Hospedeiro Imunocomprometido/imunologia , Masculino , Camundongos , Pessoa de Meia-Idade , Oncogenes , Papillomaviridae/genética , Papillomaviridae/patogenicidade , RNA Viral/análise , RNA Viral/genética , Neoplasias Cutâneas/imunologia , Neoplasias Cutâneas/patologia , Raios Ultravioleta
19.
Dermatopathology (Basel) ; 6(3): 189-194, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31616659

RESUMO

Langerhans cell histiocytosis is a rare and clinically heterogeneous group of dendritic histiocytic disorders with typical onset in the neonatal period or infancy, although it can present at any age. Histiocytes accumulate in one or more organs, leading to a variable clinical presentation of disease. We report a case of biopsy-proven Langerhans cell histiocytosis in a newborn and discuss the workup and management of this disease, along with reviewing its clinical variants.

20.
PLoS One ; 14(6): e0218155, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31194797

RESUMO

Calciphylaxis is a rare and life-threatening disease that classically manifests with painful skin lesions. It occurs mainly in patients with end-stage renal disease (ESRD) treated with dialysis, has poor outcomes, and has no FDA-approved treatment. Our cohort study aims to examine the clinical and pathological features of calciphylaxis and investigates the correlation between cutaneous clinical manifestations and histopathological findings. Data from 70 calciphylaxis patients who were evaluated at the Massachusetts General Hospital between January 2014 and April 2018 were collected from the institutional electronic database. The median age was 58 years (interquartile range [IQR]: 49-69 years), 60% were women, and 73% were of white race. Most (74%) patients reported severe pain at the time of calciphylaxis diagnosis with a median pain intensity score of 8/10 (IQR: 6-10) on a 0-10 pain scale. The median time from symptom onset to clinical diagnosis was 9 weeks (IQR: 6-16 weeks). The majority (87%) of patients presented with open necrotic wounds (advanced stage lesion) at the time of diagnosis. Common cutaneous clinical features included ulceration (79%), induration (57%), and erythema (41%), while common pathological features included cutaneous microvascular calcification (86%) and necrosis (73%). The presence of fibrin thrombi in skin biopsies was associated with pain severity (p = 0.04). The stage of a skin lesion positively correlated with the presence of necrosis on histological analyses (p = 0.02). These findings have implications for improving understanding of calciphylaxis origins and for developing novel treatments.


Assuntos
Calciofilaxia/patologia , Dermatopatias/patologia , Pele/patologia , Idoso , Biópsia/métodos , Estudos de Coortes , Feminino , Humanos , Falência Renal Crônica/patologia , Masculino , Massachusetts , Pessoa de Meia-Idade , Necrose/patologia , Diálise Renal/métodos
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