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1.
Reumatismo ; 75(3)2023 Sep 18.
Artigo em Inglês | MEDLINE | ID: mdl-37721352

RESUMO

We report a case of eosinophilic fasciitis in a teenage auto mechanic who was most likely affected by occupational exposure to organic solvents, including the aromatic hydrocarbons benzene, trimethylbenzene, naphthalene, toluene, and xylene. The patient presented with an 8-month history of painful induration of his extremities and an abnormal gait. A deep excisional biopsy of the fascia was obtained, demonstrating subcutaneous fibrosis with perivascular and interstitial inflammation, with lymphocytes and plasma cells spilling into the sclerosed fascia, and focal fibrinoid necrosis. Treatment was begun with intravenous pulse doses of methylprednisolone, prednisone (20 mg daily), and subcutaneous methotrexate (25 mg weekly), and the patient's painful induration had resolved and gait had normalized at the 6-month follow-up. Our case suggests that exposure to organic solvents could be implicated in the pathogenesis of eosinophilic fasciitis and highlights the importance of a thorough occupational history to prevent repeat exposures to potentially causative agents.


Assuntos
Eosinofilia , Fasciite , Adolescente , Humanos , Masculino , Fasciite/induzido quimicamente , Eosinofilia/induzido quimicamente , Tolueno , Solventes
2.
Br J Dermatol ; 185(6): 1221-1231, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34105768

RESUMO

BACKGROUND: Frontal fibrosing alopecia (FFA) has become one of the most common causes of cicatricial alopecia worldwide. However, there is a lack of clear aetiology and robust clinical trial evidence for the efficacy and safety of agents currently used for treatment. OBJECTIVES: To enable data to be collected worldwide on FFA using common criteria and assessment methods. METHODS: A multicentre, international group of experts in hair loss was convened by email to create consensus recommendations for clinical trials. Consensus was defined at > 90% agreement on each recommended part of these guidelines. RESULTS: Standardized diagnostic criteria, severity rating, staging, and investigator and patient assessment of scalp hair loss and other clinical features of FFA were created. CONCLUSIONS: These guidelines should allow the collection of reliable aggregate data on FFA and advance efforts in both clinical and basic research to close knowledge gaps in this condition.


Assuntos
Alopecia , Ensaios Clínicos como Assunto , Guias como Assunto , Líquen Plano , Alopecia/tratamento farmacológico , Cicatriz/tratamento farmacológico , Cicatriz/etiologia , Consenso , Humanos , Líquen Plano/patologia , Couro Cabeludo/patologia
3.
J Eur Acad Dermatol Venereol ; 34(12): 2898-2901, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32531131

RESUMO

BACKGROUND: Comorbidities are associated with higher health care costs, complex management, and poorer health outcomes. Identification and treatment of comorbid conditions in paediatric alopecia areata (AA) patients could provide an opportunity to improve health outcomes. OBJECTIVES: To determine the prevalence of comorbidities among paediatric patients with AA using a large de-identified aggregated patient database. METHODS: This is a cross-sectional study using aggregated health record data through April 1, 2019. Patients ≤18 years of age, with alopecia areata (n = 3510) and without alopecia areata (n = 8 310 710) were identified. The primary outcome was the prevalence of comorbidities among AA patients. RESULTS: Of the 8 314 220 paediatric patients, 3510 (1570 males and 1940 females) had a diagnosis of alopecia areata. The most common comorbidities included atopic dermatitis (17.4% vs. 2.2% controls, OR 9.2, 95% CI 8.55-10.18, P < 0.001), anaemia (7.7% vs. 2.4% controls, OR 3.4, 95% CI 3.06-3.92, P < 0.001), obesity (5.7% vs. 1.1% controls, OR 5.6, 95% CI 4.76-6.34, P < 0.001), vitamin D deficiency (5.1% vs. 0.4% controls, OR 14.7, 95% CI 13.5-18.1, P < 0.001), hypothyroidism (2.6% vs. 0.2% controls, OR 12, 95% CI 10.73-15.9, P < 0.001), vitiligo (1.4% vs. 0.04% controls, OR 32.2, 95% CI 24.01-42.1, P < 0.001), psoriasis (1.4% vs. 0.07% controls, OR 20.6, 95% CI 15.55-27.2, P < 0.001), hyperlipidemia (1.4% vs. 0.2% controls, OR 5.9, 95% CI 4.4-7.7, P < 0.001), and depression (2.6% vs. 0.6% controls, OR 4.8, 95% CI 5.09-9.45, P < 0.001). CONCLUSIONS: Findings from this study suggest that children with AA are more likely to have certain autoimmune and metabolic disorders than the general paediatric population. Paediatric AA patients display a severe burden of autoimmune and metabolic diseases, thus in daily practice, dermatologists might consider multidisciplinary management in these patients.


Assuntos
Alopecia em Áreas , Dermatite Atópica , Vitiligo , Alopecia em Áreas/epidemiologia , Criança , Comorbidade , Estudos Transversais , Dermatite Atópica/epidemiologia , Feminino , Humanos , Masculino , Vitiligo/epidemiologia
4.
Br J Dermatol ; 181(1): 166-174, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30613954

RESUMO

BACKGROUND: High-mobility group box 1 (HMGB1) is a damage-associated molecular-pattern protein. Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) are serious, immune-mediated skin-blistering conditions. OBJECTIVES: To determine serum and/or blister-fluid total HMGB1 levels in SJS/TEN cohorts, and HMGB1 expression in formalin-fixed, paraffin-embedded (FFPE) SJS/TEN skin vs. healthy and maculopapular exanthema (MPE) skin. Methods Serum HMGB1 was quantified in Malawian nevirapine-induced hypersensitivity, Taiwanese SJS/TEN and Spanish SJS/TEN cohorts. FFPE skin (healthy skin, MPE, SJS/TEN) was stained and assessed for HMGB1 expression. RESULTS: Serum total HMGB1 was not significantly elevated in patients with nevirapine-induced SJS/TEN (3·98 ± 2·17 ng mL-1 ), MPE (3·92 ± 2·75 ng mL-1 ) or drug reaction with eosinophilia and systemic symptoms (4·73 ± 3·00 ng mL-1 ) vs. tolerant controls (2·97 ± 3·00 ng mL-1 ). HMGB1 was significantly elevated in Taiwanese patients with SJS/TEN, highest during the acute phase (32·6 ± 26·6 ng mL-1 ) vs. the maximal (19·7 ± 23·2 ng mL-1 ; P = 0·007) and recovery (24·6 ± 25·3 ng mL-1 ; P = 0·027) phases. In blister fluid from Spanish patients with SJS/TEN, HMGB1 (486·8 ± 687·9 ng mL-1 ) was significantly higher than in serum (8·8 ± 7·6 ng mL-1 ; P <0·001). Preblistered SJS/TEN skin showed decreased epidermal nuclear HMGB1 expression in upper epidermis vs. healthy or MPE skin but retained basal/suprabasal expression. CONCLUSIONS: Epidermal HMGB1 expression was decreased in SJS/TEN skin. Retained basal/suprabasal epidermal HMGB1 expression may exacerbate localized injury in SJS/TEN.


Assuntos
Vesícula/patologia , Epiderme/patologia , Proteína HMGB1/análise , Síndrome de Stevens-Johnson/diagnóstico , Adulto , Idoso , Biomarcadores/análise , Biomarcadores/metabolismo , Biópsia , Feminino , Proteína HMGB1/metabolismo , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Síndrome de Stevens-Johnson/sangue , Síndrome de Stevens-Johnson/patologia , Adulto Jovem
5.
J Eur Acad Dermatol Venereol ; 32(8): 1385-1390, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29524253

RESUMO

BACKGROUND: Treatment for frontal fibrosing alopecia (FFA) is challenging, and its treatment regimen often mirrors other lymphocytic-predominant cicatricial alopecia. 5-alpha-reductase inhibitor (5ARI) has been reported with some treatment success in severe cases of FFA. OBJECTIVE: To carry out evidence-based analysis of articles published on treatment efficacy and safety of 5-alpha-reductase inhibitor for the treatment of FFA. METHODS: Articles published on the use of 5ARI to treat FFA between 2005 and 2017 were reviewed, analysed and graded according to the American College of Physicians outcome study grading system. RESULTS: There were two studies with moderate level of evidence that described the efficacy of 5ARI for the treatment of FFA. 5ARI was commonly used as adjunctive therapy with positive results in recalcitrant disease. Mild to moderate hair regrowth was reported in one grade 2 and three lower grade (one grade 3 and two grade 4) studies. There is limited evidence on the safety aspects of this medication in most studies that were analysed. LIMITATIONS: Database studies might not fully account for confounders and are subjected to variations in methodology and data collection. CONCLUSION: This review demonstrated that FFA patients treated with 5ARI could achieve either disease stability or reduction in the rate of progression in selected cases. A well-designed randomized, double-blind, controlled study would strengthen the role of 5ARI as part of treatment armamentarium for FFA.


Assuntos
Inibidores de 5-alfa Redutase/uso terapêutico , Alopecia/tratamento farmacológico , Alopecia/patologia , Couro Cabeludo/patologia , Inibidores de 5-alfa Redutase/efeitos adversos , Neoplasias da Mama/induzido quimicamente , Depressão/induzido quimicamente , Dutasterida/uso terapêutico , Medicina Baseada em Evidências , Feminino , Fibrose , Finasterida/uso terapêutico , Testa , Cabelo/efeitos dos fármacos , Humanos , Disfunções Sexuais Fisiológicas/induzido quimicamente
11.
J Eur Acad Dermatol Venereol ; 30(4): 667-76, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26676524

RESUMO

BACKGROUND: Female pattern hair loss (FPHL) is a common non-scarring alopecia characterized by widening of the midline hair part at the crown (vertex). In 1977, Ludwig developed a scale that graded the degree of visible vertex hair thinning from I (least severe) to III (most severe). However, by the time patients exhibit the full manifestations of 'Ludwig I', they have already lost a significant volume of hair. Although current therapies may realistically halt progression of hair loss, improvements in hair density is often more limited. Identification and grading of FPHL at an earlier stage is desirable to institute appropriate therapy before significant hair loss has occurred and to enable monitoring over time. AIM: To generate consensus guidance for the recognition and quantification of FPHL that can be used in the clinic. METHODS: Nine clinicians from Europe, North America and Australia experienced in the management of FPHL developed this scale by consensus. RESULTS: We propose a three-point severity scale (termed the FPHL Severity Index (FPHL-SI)) that combines validated measures of hair shedding, midline hair density and scalp trichoscopy criteria to produce a total FPHL-SI score (maximum score = 20). The score is designed to grade FPHL severity over time, while being sufficiently sensitive to identify early disease. A score of 0-4 makes FPHL unlikely; a score of 5-9 would indicate early-stage FPHL, with higher scores indicating greater disease severity. CONCLUSIONS: As a starting point for further public debate, we employ criteria already used in clinical practice to generate a pragmatic FPHL grading system (FPHL-SI) of sufficient sensitivity to identify and monitor early FPHL changes. This may have to be further optimized after systematic validation in clinical practice.


Assuntos
Alopecia/diagnóstico , Alopecia/classificação , Feminino , Humanos , Índice de Gravidade de Doença
14.
Am J Transplant ; 13(10): 2750-64, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23919328

RESUMO

In December of 2008, our institution performed a near total face transplant. The patient was monitored for signs of rejection assessed by paired skin and mucosa biopsies. The results of histological review of 120 biopsies collected during the first 4 years posttransplant are discussed. All biopsies were stained with hematoxylin and eosin, periodic acid-Schiff, immunohistochemical and TUNEL assays and graded using the Banff 2007 classification. Grade III rejection was diagnosed clinically at weeks 45 and 66, posttransplant; week 45 was determined as folliculitis while the erythema episode at week 66 confirmed an acute rejection (AR) that required hospitalization. The mucosa frequently showed interface inflammation without clinical signs of rejection and was not present in skin biopsies. In all, 34 of the 45 mucosal biopsies (75%) showed these interface changes. Clinical symptoms concurred with skin pathology in two grade III rejections. The mucosa showed histologic signs of rejection more frequently, which may indicate: increased mucosal sensitivity to rejection, a different type or subtype of AR that is specific to the mucosa, or a nonspecific process such as a drug effect. With more data and world experience, the diagnosis of face transplant rejection will be better defined and the Banff classification enhanced.


Assuntos
Transplante de Face , Rejeição de Enxerto/patologia , Sobrevivência de Enxerto , Feminino , Rejeição de Enxerto/classificação , Rejeição de Enxerto/imunologia , Humanos , Pessoa de Meia-Idade , Prognóstico
16.
Dermatol Ther ; 21(4): 264-7, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18715296

RESUMO

Central centrifugal cicatricial alopecia (CCCA) is a common but poorly understood cause of hair loss in African American women. A photographic scale was developed that captures the pattern and severity of the central hair loss seen with CCCA in order to help identify this problem in the general community and to potentially correlate clinical data with hair loss. The utility and reproducibility of this photographic scale was determined in a group of 150 African American women gathered for a health and beauty day who were evaluated by both four investigators experienced in the diagnosis of hair disorders and by the subjects themselves.


Assuntos
Alopecia/patologia , Negro ou Afro-Americano , Feminino , Humanos , Fotografação
17.
Int J Womens Dermatol ; 4(3): 180-184, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30175224

RESUMO

BACKGROUND: Lichen planopilaris (LPP) is a rare, cicatricial, lymphocyte-mediated alopecia that is thought to have an autoimmune pathogenesis and possibly related to other autoimmune diseases. However, data are limited and studies that examine comorbid conditions are lacking. OBJECTIVES: We sought to determine the prevalence of systemic comorbid conditions, nutritional deficiencies, psychological problems, and skin cancers in patients with LPP. METHODS: We identified 334 patients with LPP who were seen in the Department of Dermatology at the Cleveland Clinic Foundation between 2000 and 2016. Patients with LPP were compared with 78 control patients with a diagnosis of seborrheic dermatitis. RESULTS: There were more female patients with LPP compared with the controls (93.1% vs. 79.5%; p < .001) but the average age did not differ (54.77 ± 12.83 vs. 52.19 ± 15.37; p = .12). Conditions positively associated with LPP were Hashimoto's thyroiditis (6.3% vs. 0%; p = .023), hypothyroidism (24.3% vs. 12.8%; p = .028), and hirsutism (11.4% vs. 1.3%; p = .006). Negatively associated conditions were allergic rhinitis (15% vs. 24.4%; p = .046), diabetes mellitus type II (11.7% vs. 21.8%; p = .019), hyperlipidemia (38.6% vs. 52.6%; p = .024), vitamin D deficiency (50% vs. 65.4%; p = .014), depression (15.6% vs. 28.9%; p = .018), and sleep problems (7.5% vs. 29.5%; p < .001). CONCLUSIONS: Our study further emphasizes that dermatologists should screen patients with LPP for autoimmune disorders that are associated with LPP and complete a full metabolic workup to avoid missing other abnormalities. The importance of atopy, autoimmune disorders, endocrine disorders, nutritional deficiencies, psychological problems, and skin cancers in patients with scarring alopecia should be better understood.

18.
Case Rep Pathol ; 2018: 4039545, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29651355

RESUMO

Cylindromas are benign epithelial neoplasms derived from cutaneous eccrine adnexal structures. These tumors are most commonly encountered on the head, neck, and scalp of older women. In rare instances, solitary cylindromas may arise at other body sites. In the current case, a cylindroma of the skin of the breast was diagnosed by complete excision. Immunohistochemical studies confirmed the tumor cells to be immunoreactive with cytokeratin AE1/3, cytokeratin 5/6, cytokeratin 7, p63, and SOX10. The neoplastic cells were also noted to be immunoreactive with markers typically expected to be positive in ductal epithelium of the breast including GATA3, mammaglobin, and E-cadherin. The case emphasizes the importance of correlating clinical setting, imaging studies, patient history, and careful microscopic evaluation in arriving at an accurate diagnosis. This case also illustrates the point that not all "breast" tumors that are confirmed to be positive for GATA3, mammaglobin, and E-cadherin are derived from mammary ducts.

20.
J Invest Dermatol ; 105(3): 352-6, 1995 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-7665911

RESUMO

The Ehlers-Danlos syndrome has been classified into nine phenotypic presentations. Type IV is a variant of particular importance because people affected with this genodermatosis are at great risk of spontaneous hemorrhage from vascular rupture or bowel perforation. Recent molecular advances have identified mutations in the gene for type III procollagen as responsible for Ehlers-Danlos syndrome type IV. We report a case of a 14-year-old male with a typical presentation of the type IV variant who was found to have markedly dilated fibroblast cisternae and varying collagen fibril diameter on ultrastructural study. A novel genetic defect was noted by polymerase chain reaction and DNA sequencing of genetic material isolated from skin fibroblast cultures. Analysis of the gene for type III procollagen revealed a single base mutation in the last nucleotide of exon 34. The mutation led to abnormal RNA splicing and skipping of exon 34 on the mRNA level.


Assuntos
Síndrome de Ehlers-Danlos/genética , Éxons , Adolescente , Sequência de Bases , DNA/genética , Síndrome de Ehlers-Danlos/classificação , Humanos , Masculino , Dados de Sequência Molecular , Mutação , Sondas de Oligonucleotídeos/genética , Fenótipo , Reação em Cadeia da Polimerase , Pró-Colágeno/genética , Splicing de RNA
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