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1.
Artigo em Inglês | MEDLINE | ID: mdl-38712950

RESUMO

OBJECTIVES: Skin changes in acromegaly are often the first sign of the disease. The aim of this study was to describe the cutaneous findings in patients with acromegaly. In addition, a secondary aim was to investigate the possible association of these findings with remission status and concomitant endocrinopathies. DESIGN, PATIENTS, AND MEASUREMENTS: In this prospective multicenter study, 278 patients over the age of 18 years with acromegaly who were followed up in 14 different tertiary healthcare institutions were included. These patients, who were followed up by the Endocrinology Department, were then referred to a dermatologist for dermatological examination. The frequency of skin lesions was investigated by detailed dermatologic examination. Dermatological diagnosis is reached by clinical, dermatological and/or dermoscopic examination, and rarely skin punch biopsy examinations in suspicious cases. The possible association of the skin findings between remitted and nonremitted patients and with concomitant endocrinopathies were evaluated. RESULTS: The most common skin findings in patients with acromegaly in our study were skin tags (52.5%), cherry angiomas (47.4%), seborrhoea (37%), varicose veins (33%), acneiform lesions (28.8%), hyperhidrosis (26.9%) and hypertrichosis (18.3%). Hypertrichosis was significantly more prevalent in patients nonremitted (p: .001), while xerosis cutis was significantly more prevalent in patients remitted (p: .001). The frequency of diabetes mellitus and hypothyroidism was significantly higher in patients with varicose veins and seborrhoeic keratosis than those without. Additionally, the coexistence of hypothyroidism, hyperthyroidism and galactorrhea was significantly higher in patients with Cherry angioma than in those without Cherry angioma (p-values: .024, .034 and .027, respectively). The frequency of hypogonadism in those with xerosis cutis was significantly higher than in those without (p: .035). CONCLUSIONS: Cutaneous androgenization findings such as skin tag, seborrhoea, acne and acanthosis nigricans are common in patients with acromegaly. Clinicians should be aware that skin findings associated with insulin resistance may develop in these patients. It can be said that the remission state in acromegaly has no curative effect on cutaneous findings. Only patients in remission were less likely to have hypertrichosis. This may allow earlier review of the follow-up and treatment of acromegaly patients presenting with complaints of hypertrichosis. Additionally, it can be said that patients with skin findings such as cherry angioma may be predisposed to a second endocrinopathy, especially hypothyroidism. Including dermatology in a multidisciplinary perspective in acromegaly patient management would be beneficial to detect cutaneous findings earlier.

2.
Clin Exp Dermatol ; 2024 Jun 03.
Artigo em Inglês | MEDLINE | ID: mdl-38828627

RESUMO

Dystrophic epidermolysis bullosa (DEB) has long been recognized as a well-known genodermatosis, caused by COL7A1 gene pathogenic variants. Isolated anonychia associated with RSPO4 gene mutation is a recently described non-syndromic autosomal recessive condition. In this family, a 13-year-old girl presented with severe epidermolysis bullosa symptoms. Family history revealed milder but similar complaints in the siblings, and anonychia affecting all nails in the mother and maternal relatives. The father did not have any signs of DEB. Genetic testing revealed heterozygous c.6127G>A (p.Gly2043Arg) variant in the COL7A1 (NM_000094.4) gene, in the proband and her affected siblings. The variant was not detected in the mother or father, prompting investigation into parental mosaicism. Detection of the variant in sperm sample suggested paternal mosaicism. Additionally, RSPO4 gene (NM_001029871.4) was sequenced in the mother and two of her affected sisters for suspected non-syndromic isolated anonychia, revealing homozygous c.79+1G>A variant. Isolated nail disease in the mother was initially thought to be the result of DDEB nails-only subtype and the DEB in the children was inherited from the mother. However, further clinical and genetic investigation showed that the condition in the patient and her siblings arose from gonosomal mosaicism in the father and the nail phenotype in the mother is a separate coincidental condition. This report aims to serve as an example for similar cases and highlight the importance of detailed genetic analysis guided by comprehensive medical history in reaching a diagnosis.

3.
Photodermatol Photoimmunol Photomed ; 39(5): 428-434, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36967646

RESUMO

BACKGROUND: Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma. Skin-directed therapies, including phototherapy, are the first-line treatment modalities. Psoralen plus ultraviolet A light photochemotherapy (PUVA) is quite effective in controlling the disease; however, long-term adverse effects, particularly carcinogenesis, are the cons of this treatment. OBJECTIVE: There are various studies on the negative impact of PUVA on skin cancer in patients with autoimmune skin diseases. The data on the long-term effects of phototherapy on MF patients are scarce. METHODS: All MF cases that received PUVA alone or combined with other treatments at a single tertiary center were analyzed. This study compared the development of non-melanoma skin cancers, melanoma, and solid organ tumors in MF patients with at least 5-year follow-up data with age- and sex-matched controls. RESULTS: A total of 104 patients were included in the study. Ninety-two malignancies were detected in 16 (15.4%) patients, and six developed multiple malignancies. Skin cancers consisted of 56 basal cell carcinomas, 16 Bowen's disease, four squamous cell carcinomas, three melanomas, two basosquamous cell carcinomas, one Kaposi sarcoma, and one keratoacanthoma were found in nine (8.7%) patients. Eight patients developed three solid cancers and six lymphomas. The risk of developing skin cancer was associated with the total number of PUVA sessions (<250 vs ≥250 sessions; hazard ratio (HR) 4.44, 95% confidence interval (CI) 1.033-19.068; p = .045). 9 (13.2%) of 68 patients who had follow-ups for at least 5 years developed skin cancer. Compared to an age- and sex-matched cohort, the prevalence of new skin cancer was considerably greater (p = .009). CONCLUSIONS: Patients with MF are predisposed to develop secondary malignancies, and continual exposure to PUVA may potentiate this risk. Annual digital dermoscopic follow-up in MF patients treated with UVA is advised for early diagnosis and treatment of secondary cutaneous malignancies.


Assuntos
Micose Fungoide , Fotoquimioterapia , Neoplasias Cutâneas , Humanos , Terapia PUVA/efeitos adversos , Micose Fungoide/tratamento farmacológico , Micose Fungoide/epidemiologia , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/etiologia , Neoplasias Cutâneas/diagnóstico , Fototerapia
4.
Allergy ; 77(1): 258-270, 2022 01.
Artigo em Inglês | MEDLINE | ID: mdl-34519053

RESUMO

BACKGROUND: Vaccines that incorporate multiple SARS-CoV-2 antigens can further broaden the breadth of virus-specific cellular and humoral immunity. This study describes the development and immunogenicity of SARS-CoV-2 VLP vaccine that incorporates the four structural proteins of SARS-CoV-2. METHODS: VLPs were generated in transiently transfected HEK293 cells, purified by multimodal chromatography, and characterized by tunable-resistive pulse sensing, AFM, SEM, and TEM. Immunoblotting studies verified the protein identities of VLPs. Cellular and humoral immune responses of immunized animals demonstrated the immune potency of the formulated VLP vaccine. RESULTS: Transiently transfected HEK293 cells reproducibly generated vesicular VLPs that were similar in size to and expressing all four structural proteins of SARS-CoV-2. Alum adsorbed, K3-CpG ODN-adjuvanted VLPs elicited high titer anti-S, anti-RBD, anti-N IgG, triggered multifunctional Th1-biased T-cell responses, reduced virus load, and prevented lung pathology upon live virus challenge in vaccinated animals. CONCLUSION: These data suggest that VLPs expressing all four structural protein antigens of SARS-CoV-2 are immunogenic and can protect animals from developing COVID-19 infection following vaccination.


Assuntos
COVID-19 , Vacinas de Partículas Semelhantes a Vírus , Animais , Anticorpos Neutralizantes , Anticorpos Antivirais , Vacinas contra COVID-19 , Células HEK293 , Humanos , SARS-CoV-2
5.
Dermatol Ther ; 35(5): e15447, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35289037

RESUMO

Allogeneic hematopoietic stem cell transplantation (AHSCT) is a promising strategy for treatment of heavily pretreated mycosis fungoides/Sezary syndrome (MF/SS). Herein, we aimed to evaluate the outcomes of AHSCT for heavily pretreated patients with MF/SS retrospectively. This analysis included consecutive 19 patients with MF/SS who received 20 AHSCT between 2012-2021 in our transplant center. Eight patients have been previously reported. Fifteen patients had diagnosis of MF and referred to SS in five patients. In our cohort, all cases had advanced disease (stages IIB: n = 1, IIIA: n = 7; IIIB: n = 4, IVA: n = 4, and IVB: n = 3). Nine patients (47.4%) had developed large cell transformation. Only two patients received AHSCT in complete response, one very good partial response and two partial response while the others had progressive disease (n = 15) before transplant. Seven (35%) patients were alive at the time of analysis, with a median follow up of 10.5 months (range, 0.3-113 months) after AHSCT. Nine patients (47.4%) died without disease relapse or progression. Non-relapse mortality was 35.9% at 1 year and 26.9% at 3 years and thereafter. For all patients the probability of overall survival was 48.5% and 32.3% at 1- and 5-year post-transplant, respectively. AHSCT for MF/SS resulted in an estimated progression free survival of 45.4% at 1 year. Given the poor prognosis of patients not receiving transplants and in the absence of curative non-transplantation therapies, our results support that AHSCT is able to effectively rescue 32.3% of the population of transplant eligible, heavily pretreated patients in 5 years.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Linfoma Cutâneo de Células T , Micose Fungoide , Síndrome de Sézary , Neoplasias Cutâneas , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Linfoma Cutâneo de Células T/etiologia , Micose Fungoide/diagnóstico , Micose Fungoide/terapia , Estudos Retrospectivos , Síndrome de Sézary/terapia , Neoplasias Cutâneas/etiologia , Neoplasias Cutâneas/terapia , Transplante Homólogo
6.
Australas J Dermatol ; 62(4): e496-e503, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34642934

RESUMO

BACKGROUND: The differentiation between the pemphigoid diseases is essential for treatment and prognosis. In Turkey, data on the incidence of these diseases are insufficient. Our aim in this study is to determine the incidence, demographics and clinical characteristics associated with diseases of the pemphigoid group. METHODS: We prospectively analysed 295 patients with pemphigoid who visited dermatology clinics of tertiary referral hospitals in 12 different regions of Turkey within a year. The diagnosis was based on clinical, histopathological, direct immunofluorescence (DIF) and serological (multivariant enzyme-linked immunosorbent assay [ELISA], indirect immunofluorescence and mosaic-based BIOCHIP) examinations. Clinical and demographic findings, aetiological factors and concomitant diseases observed in the patients were recorded. RESULTS: A total of 295 (female/male ratio: 1.7/1) patients with pemphigoid were diagnosed in 1-year period. The overall incidence rate of pemphigoid diseases was found to be 3.55 cases per million-years. The ratio of pemphigoid group diseases to pemphigus group diseases was 1.6. The most common pemphigoid type was bullous pemphigoid (BP, 93.2%). The others were epidermolysis bullosa acquisita (3.1%), pemphigoid gestationis (2.4%), linear IgA disease (1%) and mucous membrane pemphigoid (0.3%). The most common (26.8%) possible trigger of the bullous pemphigoid was gliptin derivative drugs. The most common concomitant diseases with pemphigoid were cardiovascular (27.8%) and neurological diseases (23.7%). CONCLUSIONS: This study showed that the increased frequency of bullous pemphigoid reversed the pemphigoid/pemphigus ratio in Turkey. Further studies are warranted regarding the reasons for this increase.


Assuntos
Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/epidemiologia , Pênfigo/diagnóstico , Pênfigo/epidemiologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Distribuição por Sexo , Turquia/epidemiologia , Adulto Jovem
7.
Dermatol Ther ; 33(4): e13585, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32410323

RESUMO

Folliculotropic mycosis fungoides (FMF) is characterized by a broad clinical spectrum and worse prognosis compared to classical MF. This study aimed to evaluate the clinical characteristics, treatment modalities and long-term outcome and risk factors for progression and survival of FMF patients. We conducted a single-center retrospective study and reviewed 53 patients diagnosed with FMF between 1990 to 2019 in a referral center at Ankara University, Turkey. Regarding to stage at diagnosis, 24 patients (45.3%) had advanced-stage disease (≥IIB). Follicular papules was observed in 66% and alopecia in 49.1% of the cases. Forty-three patients (81.1%) suffered from pruritus. The majority of the patients (92.4%) had at least one systemic therapy. Complete remission was achieved in 24.5% of the patients. The median time of overall survival (OS) was 50 months (range 9-324 months) and 5-year and 10-year OS was 83% and 69%, respectively. Twenty-eight (52.3%) patients progressed to more advanced stages and seven (13.2%) patients died due to MF during the follow-up period. FMF is associated with a progressive course and in most patients, skin-directed therapies were found to be inefficient to control the disease and multiple systemic therapeutic agents were required to control the disease.


Assuntos
Micose Fungoide , Neoplasias Cutâneas , Humanos , Micose Fungoide/diagnóstico , Micose Fungoide/terapia , Estudos Retrospectivos , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Centros de Atenção Terciária , Turquia
8.
Dermatol Ther ; 32(5): e13003, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31237104

RESUMO

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disorder that causes a significant decline in quality of life. There are numerous treatment options; however, real-life data on the efficacy of these treatments is limited. This study was performed in two centers to describe clinical characteristics and assess treatment outcome in a cohort of 139 patients with HS. Data on demographic and clinical characteristics, Hurley stage and comorbidities were collected from patient charts and evaluated retrospectively. Treatment response was measured with HS clinical response index (HISCR). Mean body mass index was 27.8±4.88. Inflammatory comorbidities were present in 23%. Among first-line drugs systemic doxycycline resulted in 60% HISCR followed by rifampicin-clindamycin combination (46.4%). Isotretinoin had the lowest HISCR (30.7%) in this group. For second-line therapies, all acitretin treated patients achieved response and patients treated with tumor necrosis factor alpha (TNF-α) inhibitors had the highest HISCR. Currently recommended first-line therapies have moderate efficacy in HS. Acitretin appears to be a reasonable alternative for the highly effective TNF-α inhibitors in patients with severe and resistant HS. Overall, these results support that excessive inflammatory response play an important role in pathogenesis of HS.


Assuntos
Acitretina/uso terapêutico , Antibacterianos/uso terapêutico , Fatores Biológicos/uso terapêutico , Hidradenite Supurativa/diagnóstico , Adulto , Quimioterapia Combinada , Feminino , Hidradenite Supurativa/tratamento farmacológico , Humanos , Ceratolíticos/uso terapêutico , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento , Adulto Jovem
9.
J Allergy Clin Immunol ; 142(1): 246-257, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29155101

RESUMO

BACKGROUND: Pathological inflammatory syndromes of unknown etiology are commonly observed in ataxia telangiectasia (AT) and Artemis deficiency. Similar inflammatory manifestations also exist in patients with STING-associated vasculopathy in infancy (SAVI). OBJECTIVE: We sought to test the hypothesis that the inflammation-associated manifestations observed in patients with AT and Artemis deficiency stem from increased type I IFN signature leading to neutrophil-mediated pathological damage. METHODS: Cytokine/protein signatures were determined by ELISA, cytometric bead array, or quantitative PCR. Stat1 phosphorylation levels were determined by flow cytometry. DNA species accumulating in the cytosol of patients' cells were quantified microscopically and flow cytometrically. Propensity of isolated polymorhonuclear granulocytes to form neutrophil extracellular traps (NETs) was determined using fluorescence microscopy and picogreen assay. Neutrophil reactive oxygen species levels and mitochondrial stress were assayed using fluorogenic probes, microscopy, and flow cytometry. RESULTS: Type I and III IFN signatures were elevated in plasma and peripheral blood cells of patients with AT, Artemis deficiency, and SAVI. Chronic IFN production stemmed from the accumulation of DNA in the cytoplasm of AT and Artemis-deficient cells. Neutrophils isolated from patients spontaneously produced NETs and displayed indicators of oxidative and mitochondrial stress, supportive of their NETotic tendencies. A similar phenomenon was also observed in neutrophils from healthy controls exposed to patient plasma samples or exogeneous IFN-α. CONCLUSIONS: Type I IFN-mediated neutrophil activation and NET formation may contribute to inflammatory manifestations observed in patients with AT, Artemis deficiency, and SAVI. Thus, neutrophils represent a promising target to manage inflammatory syndromes in diseases with active type I IFN signature.


Assuntos
Ataxia Telangiectasia/imunologia , Armadilhas Extracelulares/imunologia , Síndromes de Imunodeficiência/imunologia , Interferon Tipo I/imunologia , Ataxia Telangiectasia/patologia , Proteínas de Ligação a DNA , Endonucleases/deficiência , Endonucleases/imunologia , Humanos , Síndromes de Imunodeficiência/genética , Proteínas de Membrana/genética , Ativação de Neutrófilo , Neutrófilos/imunologia , Neutrófilos/patologia , Proteínas Nucleares/deficiência , Proteínas Nucleares/imunologia , Vasculite/genética , Vasculite/imunologia , Vasculite/patologia
10.
Cytogenet Genome Res ; 151(4): 186-190, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28554179

RESUMO

H syndrome (OMIM 602782) is a very rare autosomal recessive genodermatosis with multisystem involvement. Hallmarks of this disorder are juvenile onset and progressive, hyperpigmented, hypertrichotic lesions with histiocytic infiltration. Associated systemic manifestations form a long list, and there is high variability between patients. In some patients, dysmorphic and other systemic features may be so subtle that the disorder may readily be mistaken as an acquired skin disease and treated as such. Herein, we report a novel homozygous c.1339G>A (p.Glu447Lys) mutation in the SLC29A3 gene in a patient with skin-dominant presentation of H syndrome. Additionally, due to the present case, double superior vena cava can be added to the list of possible cardiovascular manifestations of H syndrome.


Assuntos
Mutação/genética , Proteínas de Transporte de Nucleosídeos/genética , Dermatopatias/genética , Pele/patologia , Adolescente , Feminino , Homozigoto , Humanos , Fenótipo , Dermatopatias/patologia , Síndrome
13.
Dermatol Pract Concept ; 12(1): e2022036, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-35223180

RESUMO

INTRODUCTION: The COVID-19 pandemic drastically changed the priorities in healthcare services; outpatient management of acne has changed during this period. OBJECTIVES: We aimed to investigate treatment practices, outcomes and identify modified follow-up schedules applied during the pandemic. METHODS: The patients who were admitted to dermatology outpatient clinic between March 13 and July 13, 2020, were included. Patients who were admitted between March 13 and July 13, 2019, were served as controls for the study. For each patient, age, gender, treatment protocols, treatment intervals, compliance with the treatment, treatment modifications, and adverse events were recorded. RESULTS: The total number of acne patients admitted to dermatology outpatient clinics during the pandemic period was 278 and consisted of 12.3% (278) of all admissions. Isotretinoin treatment was started in only 16 (5.8%) of the patients. The proportion of patients who were under follow-up was significantly higher during the pandemic period (P < 0.005). There was no difference between the pandemic period and the non-pandemic period in terms of starting isotretinoin treatment (P > 0.05). During pandemic period, 79% of the patients who used isotretinoin were followed-up every two or more months. Extended follow-up intervals showed no difference for detecting side effects (P > 0.05). CONCLUSIONS: Acne patients constitute an important part of dermatology outpatient clinics. During the pandemic period, majority of acne patients came for follow-up. Extended follow-up periods were adopted by physicians and were found safe and effective in the current study. Thus, isotretinoin treatment seems efficacious and safe during pandemic period.

14.
J Drugs Dermatol ; 10(4): 403-8, 2011 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-21455551

RESUMO

OBJECTIVE: Long-term survival for advanced stages of mycosis fungoides (MF) may be beneficially affected by the use of multimodality therapy. We aim to evaluate the activity of vorinostat in combination with interferon (IFN) alpha and extracorporeal photopheresis (ECP) with persistent, progressive advanced stage MF and Sezary syndrome (SS). PATIENTS AND METHODS: Three patients with stage IIB-IVA MF/SS were treated with vorinostat 400 mg/day/po. Vorinostat was added to ongoing ECP and IFN-alpha-2a therapies in all three patients. RESULTS: The patient with stage IIB MF achieved a complete response. The patient with SS showed a stable disease of less than 50 percent improvement in body surface area with reduction in the sizes of axillary and inguinal lymph nodes. A partial remission was maintained for 24 weeks in the patient with stage IVA MF, followed by rapid disease progression under treatment which led to cessation of vorinostat treatment due to study criteria as well as serious side effects. CONCLUSION: Our experience in this case series is suggestive of the synergistic effect of vorinostat in combination with IFN and ECP and supports the efficacy of vorinostat in inducing prolonged responses in patients with progressive disease and/or stable disease in otherwise progressive and treatment refractory late stage MF/SS.


Assuntos
Ácidos Hidroxâmicos/uso terapêutico , Interferon-alfa/uso terapêutico , Micose Fungoide/tratamento farmacológico , Fotoferese , Síndrome de Sézary/tratamento farmacológico , Adulto , Antineoplásicos/efeitos adversos , Antineoplásicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Progressão da Doença , Feminino , Inibidores de Histona Desacetilases/efeitos adversos , Inibidores de Histona Desacetilases/uso terapêutico , Humanos , Ácidos Hidroxâmicos/efeitos adversos , Interferon alfa-2 , Interferon-alfa/efeitos adversos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/patologia , Proteínas Recombinantes , Índice de Gravidade de Doença , Síndrome de Sézary/patologia , Resultado do Tratamento , Vorinostat
15.
J Clin Rheumatol ; 17(5): 278-80, 2011 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-21778904

RESUMO

We describe a patient with cutaneous T-cell lymphoma (CTCL), in association with concurrent ankylosing spondylitis (AS), who achieved a long-lasting complete remission of both diseases after 3 cycles of extracorporeal photochemotherapy (ECP). The combination of CTCL with AS is very rare. In our patient, the CTCL was refractory to psoralen-UVA. He was unable to continue with administration of interferon α because of exacerbation of his back, anterior chest, and bilateral heel pain. The Bath AS Functional Index and Bath AS Disease Activity Index scores were determined to be 2.2 and 5.8, respectively. Extracorporeal photochemotherapy has been shown to be effective in the treatment of CTCL consisting of reinfusion of 3 to 9 × 10 leukocytes, taken from the patient by leukopheresis and treated in an extracorporeal system with 8-methoxypsoralen and UVA. There is a strong suggestion that ECP as a monotherapy can provide a significant benefit for other T-cell-mediated diseases including rheumatoid arthritis and psoriatic arthritis. This is the first report describing an effective treatment of AS in which pain, inflammatory response, Bath AS Disease Activity Index, and Bath AS Functional Index improved after initiation of ECP.


Assuntos
Micose Fungoide/terapia , Fotoferese , Espondilite Anquilosante/terapia , Comorbidade , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/epidemiologia , Índice de Gravidade de Doença , Espondilite Anquilosante/epidemiologia , Resultado do Tratamento
16.
Transfus Apher Sci ; 43(3): 353-359, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-21035398

RESUMO

Pemphigus vulgaris (PV) and epidermolysis bullosa acquisita (EBA) sometimes resist treatments. In drug-resistant cases, adjuvant treatment with extracorporeal photochemotherapy (ECP) has been reported to induce remission. However, limited numbers of patients have been reported up to date. Eleven patients with drug-resistant autoimmune bullous diseases have been treated with ECP (8 patients with PV, 3 patients with EBA). The introduction of ECP to systemic therapies of the patients with PV resulted in complete response (CR) after a limited cycle (2-6 cycles) in all, except one patient. Prednisolone doses could be tapered in all patients. ECP resulted in CR in two patients while improvement was partial in one patient with EBA after 3-6 cycles. Our patients demonstrate the efficacy of long-term ECP to be tried in the treatment of aggressive autoimmune bullous disorders. The treatment has produced a remarkable corticosteroid-sparing effect while inducing clinical remission.


Assuntos
Epidermólise Bolhosa Adquirida/terapia , Pênfigo/terapia , Fotoferese/métodos , Adulto , Doenças Autoimunes/terapia , Resistência a Medicamentos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prednisolona/uso terapêutico , Indução de Remissão/métodos , Estudos Retrospectivos
17.
J Drugs Dermatol ; 9(8): 1014-6, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20684154

RESUMO

There are several reports of patients who developed extracutaneous lymphoma after they started bexarotene treatment. The authors report a case in which the initiation of bexarotene therapy for Sézary syndrome was temporally associated with the development of Hodgkin's lymphoma despite improvement in cutaneous signs and symptoms. It is possible that bexarotene may contribute to the development of extracutaneous lymphoma. Although bexarotene therapy may relieve symptoms and signs of cutaneous T-cell lymphoma, careful examination of the lymph nodes during treatment is recommended.


Assuntos
Anticarcinógenos/efeitos adversos , Doença de Hodgkin/induzido quimicamente , Tetra-Hidronaftalenos/efeitos adversos , Adulto , Anticarcinógenos/uso terapêutico , Bexaroteno , Humanos , Masculino , Síndrome de Sézary/tratamento farmacológico , Síndrome de Sézary/patologia , Tetra-Hidronaftalenos/uso terapêutico
18.
Turk J Haematol ; 27(2): 91-8, 2010 Jun 05.
Artigo em Inglês | MEDLINE | ID: mdl-27263450

RESUMO

OBJECTIVE: We aimed to evaluate the clinical features of sclerodermatous chronic graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (AHSCT). METHODS: We retrospectively analyzed 423 patients who underwent AHSCT. We assessed age, sex, pre-transplant diagnosis, conditioning regimen, GVHD prophylaxis, and occurrence of acute GVHD (aGVHD), chronic lichenoid and chronic systemic GVHD, and clinical properties of sclerodermatous GVHD. RESULTS: Sclerotic skin lesions developed in 22 patients after a mean of 752±647 days (median 480). aGVHD appeared in 17 patients, with hepatic involvement in 2, gastrointestinal tract involvement in 2 and skin involvement in 13 of these patients. Extensive chronic GVHD (liver, pulmonary, skin and oral mucosa) developed in 12 patients. Sclerosis was generalized in 19 patients (86.4%) and localized in 3 patients (13.6%). Leopard skin eruption appeared in 8 (36.4%) of the 19 patients with generalized sclerodermatous changes. In most cases, sclerotic lesions appeared on the trunk, and distal parts of the extremities were spared. Eight patients (36.4%) progressed from lichenoid to sclerodermatous lesions, 2 (9.1%) with lichenoid and sclerodermatous phases together and 12 (55.5%) with de novo sclerodermatous lesions. Five patients died because of late transplant-related complications. CONCLUSION: Sclerodermatous GVHD has a late onset and may be quite disabling. Unlike scleroderma, acral involvement is seen rarely. Although most lesions do not disappear in the course of the disease, most patients have a good prognosis.

19.
Int J Dermatol ; 59(1): 127-133, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31633200

RESUMO

BACKGROUND AND OBJECTIVE: Less than 5% of cases of mycosis fungoides (MF) present with a cytotoxic/suppressor CD8+ phenotype. This study aimed to evaluate the clinical characteristics, treatment modalities, and clinical course in CD8+ MF patients. METHODS: In a retrospective analysis of 353 MF patients in a referral center at Ankara University, Turkey, 29 patients that were diagnosed with CD8+ MF were included in the study. RESULTS: CD8+ MF cases constituted 8.2% of all MF patients. The age at the time of diagnosis ranged between 6 and 81 years with a median value of 46 years. The female-to-male ratio was 1.41. Patients presented with erythematous scaly (69%), hyperpigmented (58.6%), poikilodermic (17.2%), and hypopigmented (17.2 %) patches/plaques. The most common sites of involvement were the trunk and lower extremities. The most common comorbidity was hypertension (24.1%, n: 7) with 13 patients (44.8%) having a history of at least one autoimmune disease. At the time of diagnosis, 93.2% of the patients had early-stage disease, and 6.8% of the patients had advanced stage. The mean follow-up period was 6.68 ± 6.04 years (range 1-28 years). Most of the patients were treated with skin-directed therapies. Complete remission was achieved in 17 (58.6%) patients, eight (27.6%) patients had partial remission, and four (13.8%) patients had stable disease. CONCLUSIONS: We concluded that CD8+ MF is associated with an indolent course and in most patients, skin-directed therapies were found to be efficient to control the disease.


Assuntos
Linfócitos T CD8-Positivos/imunologia , Micose Fungoide/diagnóstico , Micose Fungoide/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/imunologia , Estudos Retrospectivos , Adulto Jovem
20.
Dermatology ; 218(3): 226-30, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19060465

RESUMO

BACKGROUND: Mastocytosis is a disorder characterized by the accumulation of mast cells in various organs, most commonly in the skin. Cutaneous mastocytosis (CM) can be classified as nodular CM with solitary or multiple lesions, diffuse CM (erythroderma), and maculopapular CM including the papular/plaque variant, urticaria pigmentosa (UP) and telangiectasia macularis eruptiva perstans (TMEP). OBJECTIVE: To evaluate the dermatoscopic features of cutaneous mastocytosis. METHODS: We reviewed the dermatoscopic images of 6 patients who had different variants of cutaneous mastocytosis and who attended the Departments of Dermatology at the Medical University of Ankara, Turkey, and the Medical University of Vienna, Austria. RESULTS: In UP and in the papular variant of CM the most common structures seen by dermatoscopy were brown reticular lines (pigment network). In TMEP we observed telangiectatic vessels arranged in a reticular pattern. CONCLUSION: Skin lesions of mastocytosis may exhibit a pigment network, a dermatoscopic feature said to be characteristic of melanocytic lesions. We were also able to identify a new dermatoscopic feature, a reticular vascular pattern that is characteristic of a clinical variant of mastocytosis known as TMEP. This feature may help to differentiate TMEP from other variants of mastocytosis and from other exanthematous skin diseases.


Assuntos
Dermoscopia , Mastocitose Cutânea/patologia , Adolescente , Adulto , Pré-Escolar , Feminino , Humanos , Masculino , Pele/patologia
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