Evolution of Kaposi sarcoma in the past 30 years in a tertiary hospital of the European Mediterranean basin.

BACKGROUND: The incidence of Kaposi sarcoma (KS) has reduced as a result of the introduction of antiretroviral therapy. It is currently considered a rare disease in developed countries, and there has been a paucity of clinical papers on the subject in recent years in Europe. AIM: To analyse the clinical features and evolution of the different clinical forms of KS in the past 30 years. METHODS: Patients with cutaneous lesions of KS diagnosed during the period 1987-2016 at Bellvitge Hospital (an 800-bed university referral centre in Barcelona, Spain) were enrolled. Data recorded included age, sex, ethnicity, involved site, number of lesions, extracutaneous involvement, leg oedema, treatment, blood haemoglobin level, and blood cell (leucocyte, lymphocyte and CD4) counts. RESULTS: Cutaneous lesions of KS were diagnosed in 191 patients (167 men, 24 women, mean ± SD age 51.95 ± 20.16 years). Clinical forms identified were classic KS (n = 53), acquired immunodeficiency syndrome (AIDS)-associated KS (n = 118), immunosuppression-associated KS (n = 18), and African endemic KS (n = 2). The number of patients diagnosed annually reached a maximum in the 1990s because of the AIDS epidemic, and has decreased since 2000. However, both classic KS and immunosuppression-associated KS doubled from the first to the second half of the analysed period. Cutaneous lesions involved the legs in 137 cases, and extracutaneous lesions were detected in 32 patients. In 46 of 118 patients with AIDS, the diagnosis of KS was simultaneous to the detection of human immunodeficiency virus infection. CONCLUSION: After a decrease in incidence since the middle of the 1990s, AIDS-associated KS continues to occur in Europe, and the number of annual cases of classic KS and immunosuppression-associated KS is increasing.

First-line treatment in lymphomatoid papulosis: a retrospective multicentre study.

BACKGROUND: Data regarding response to treatment in lymphomatoid papulosis (LyP) are scarce. AIM: To assess the daily clinical practice approach to LyP and the response to first-line treatments. METHODS: This was a retrospective study enrolling 252 patients with LyP. RESULTS: Topical steroids, methotrexate and phototherapy were the most common first-line treatments, prescribed for 35%, 20% and 14% of the patients, respectively. Complete response (CR) was achieved in 48% of treated patients. Eczematous lesions significantly increased relative risk (RR) of not achieving CR (RR = 1.76; 95% CI 1.16-2.11). Overall median time to CR was 10 months (95% CI 6-13 months), and 78% of complete responders showed cutaneous relapse; both results were similar for all treatment groups (P > 0.05). Overall estimated median disease-free survival (DFS) was 11 months (95% CI 9-13 months) but DFS for patients treated with phototherapy was 23 months (95% CI 10-36 months; P < 0.03). Having the Type A LyP variant (RR = 2.04; 95% CI 0.96-4.30) and receiving a first-line treatment other than phototherapy (RR = 5.33; 95% CI 0.84-33.89) were significantly associated with cutaneous early relapse. Of the 252 patients, 31 (13%) had associated mycosis fungoides unrelated to therapeutic approach, type of LyP or T-cell receptor clonality. CONCLUSIONS: Current epidemiological, clinical and pathological data support previous results. Topical steroids, phototherapy and methotrexate are the most frequently prescribed first-line treatments. Although CR and cutaneous relapse rates do not differ between them, phototherapy achieves a longer DFS. Presence of Type A LyP and use of topical steroid or methotrexate were associated with an increased risk of early relapse.

Cutaneous leishmaniasis associated with anti-tumour necrosis factor-α drugs: an emerging disease.

Leishmaniasis is endemic in several geographic areas of the world. In each of these areas, particular species of Leishmania with differing aggressiveness to humans predominate. In the European Mediterranean basin, cutaneous leishmaniasis usually presents with discrete, self-healing skin lesions. Although it is known that tumour necrosis factor (TNF) inhibitors may increase the risk of developing infections such as tuberculosis, there is scarce literature on Leishmania infections in patients treated with these drugs. In recent months, we have observed three patients resident in the Catalan coast of Spain who were treated with TNF inhibitors for Crohn disease, and who developed unusually large and persistent cutaneous lesions of leishmaniasis. These lesions responded only to treatment with intravenous liposomal amphotericin B. In countries with a high incidence of infection by aggressive species of Leishmania, serological screening may be indicated to detect a possible latent leishmanial infection before prescription of TNF inhibitors.

Sweet syndrome: long-term follow-up of 138 patients.

BACKGROUND: Several studies support a strong association of Sweet syndrome (SS) with malignancy. However, only a few studies analysing the clinical features of malignancy-associated SS have been published in recent years. AIM: To retrospectively study the clinical features of SS that could predict the development of associated malignancies and to analyse the development of malignant neoplasia during long-term follow-up of patients with SS. METHODS: Clinical features of the patients diagnosed with SS syndrome between 1987 and 2013 at Bellvitge Hospital (Barcelona, Spain) were retrospectively analysed. RESULTS: In total, 138 patients were included in the study (66 male, 72 female, mean ± SD age 51.24 ± 14.11 years). SS was associated with haematological malignancy in 31 cases, infection in 23, inflammatory bowel disease in 12, inflammatory systemic disease in 8, and solid tumours in 4. It was drug-induced in 5 cases and idiopathic in 54. In four patients, an underlying haematological disease that was considered related to SS was diagnosed between 4 and 16 months after SS presentation. Variables significantly associated with malignancy in multivariate logistic regression analysis were age (OR = 1.08 for each increasing year, P = 0.01), anaemia (OR = 9.38, P = 0.001), thrombocytopenia (OR = 16.10, P < 0.01) and absence of arthralgia (OR = 11.13, P < 0.01). CONCLUSIONS: Patients with older age, anaemia or thrombocytopenia, and without arthralgia are more likely to have malignancy-associated SS. We recommend that patients with SS without clear aetiology should be followed up for at least 16 months to exclude a possible underlying haematological malignancy.

Necrobiosis lipoidica: a descriptive study of 35 cases.

INTRODUCTION AND OBJECTIVES: Necrobiosis lipoidica (NL) is a chronic idiopathic granulomatous disease considered to occur in association with diabetes mellitus. Data on the frequency of this association, however, are inconsistent. Our aim was to retrospectively analyze the clinical characteristics of patients diagnosed with NL at our hospital and to investigate the association with diabetes mellitus and other diseases. MATERIAL AND METHODS: We performed a chart review of all patients with a clinical and histologic diagnosis of NL treated and followed in the dermatology department of Hospital de Bellvitge in Barcelona, Spain between 1987 and 2013. RESULTS: Thirty-five patients (6 men and 29 women with a mean age of 47.20 years) were diagnosed with NL in the study period. At the time of diagnosis, 31 patients had pretibial lesions. Thirteen patients (37%) had a single lesion at diagnosis, and the mean number of lesions was 3.37. Twenty-three patients (65.71%) had diabetes mellitus (type 1 in 10 cases and type 2 in 13). In 20 patients, onset of diabetes preceded that of NL by a mean of 135.70 months. The 2 conditions were diagnosed simultaneously in 3 patients. None of the 35 patients developed diabetes mellitus during follow-up. Six patients had hypothyroidism, and 4 of these also had type 1 diabetes. CONCLUSIONS: NL is frequently associated with type 1 and 2 diabetes. Although diabetes tends to develop before NL, it can occur simultaneously.

Association between infliximab concentrations and clinical response in psoriasis: a prospective cohort study.

BACKGROUND: Infliximab (IFX) trough concentrations (Cmin) have been linked to treatment efficacy in psoriatic patients. Inter-individual IFX Cmin variability and factors influencing IFX pharmacokinetics could explain differences in treatment response. OBJECTIVE: To evaluate the association between IFX Cmin and clinical outcomes in psoriatic patients. METHODS: Prospective study of 33 patients with moderate to severe psoriasis receiving IFX at Bellvitge University Hospital, between October 2013 and November 2016. IFX Cmin and antibodies toward infliximab (ATI) were measured. RESULTS: We collected 155 IFX Cmin and ATI values (mean age, 46 (14) years; 11 (33.3%) women). Mean IFX Cmin was 2.5 (2.4) mg/L and ATIs were detected in six patients, resulting in undetectable IFX Cmin. IFX Cmin was significantly associated with ATI and body mass index (BMI) (ß -2.51, 95% CI -3.56 to -1.4 and ß -0.05, 95% CI -0.09 to -0.01). PASI score and PASI 90/100 response were significantly associated with IFX Cmin (IRR 0.80, 95% CI 0.70 to 0.92; OR 1.79, 95% CI 1.18 to 2.71 and OR 1.79, 95% CI 1.14 to 2.81). CONCLUSION: IFX Cmin significantly influences PASI 90/100 response rates. IFX Cmin wa significantly associated with ATI and BMI. The observed inter-individual variability in IFX Cmin supports the need for IFX drug monitoring.

Regresión completa espontánea del tumor primario en el carcinoma de células de Merkel / Complete Spontaneous Regression of the Primary Tumor in Merkel Cell Carcinoma

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Necrobiosis lipoídica. Estudio descriptivo de 35 pacientes / Necrobiosis Lipoidica: A Descriptive Study of 35 Cases

Introducción y objetivos: La necrobiosis lipoídica (NL) es una enfermedad granulomatosa idiopática de curso crónico que se considera asociada a la diabetes mellitus (DM). Sin embargo, existen resultados contradictorios respecto a la frecuencia de esta asociación. Nuestro objetivo fue analizar retrospectivamente las características clínicas de nuestros pacientes con NL y su relación con la DM y otras enfermedades. Material y métodos: Todos los pacientes diagnosticados clínica e histológicamente de NL que han sido tratados y controlados en el Servicio de Dermatología del Hospital de Bellvitge de Barcelona fueron incluidos en el estudio. Resultados: Treinta y cinco pacientes fueron diagnosticados de NL entre 1987 y 2013 (6 varones y 29 mujeres, edad media 47,20 años). En el momento del diagnóstico de la NL 31 pacientes presentaban lesiones a nivel pretibial. Trece pacientes (37%) presentaban una única lesión al ser diagnosticados y el número medio de lesiones fue de 3,37. La NL se asoció a DM en 23 pacientes (65,71%) (10 tipo 1, 13 tipo 2). En 20 casos la DM precedió al inicio de las lesiones de NL con un tiempo medio de 135,70 meses. En 3 casos la DM y la NL se diagnosticaron simultáneamente. Ninguno de nuestros pacientes con NL desarrolló DM durante el tiempo de seguimiento. Seis pacientes tenían hipotiroidismo, 4 de los cuales tenían también DM tipo 1. Conclusiones: La NL está frecuentemente asociada a DM, tanto tipo 1 como tipo 2, y aunque esta suele precederla en años, en algún caso la aparición es simultánea (AU)

Introduction and objectives: Necrobiosis lipoidica (NL) is a chronic idiopathic granulomatous disease considered to occur in association with diabetes mellitus. Data on the frequency of this association, however, are inconsistent. Our aim was to retrospectively analyze the clinical characteristics of patients diagnosed with NL at our hospital and to investigate the association with diabetes mellitus and other diseases. Material and methods: We performed a chart review of all patients with a clinical and histologic diagnosis of NL treated and followed in the dermatology department of Hospital de Bellvitge in Barcelona, Spain between 1987 and 2013. Results: Thirty-five patients (6 men and 29 women with a mean age of 47.20 years) were diagnosed with NL in the study period. At the time of diagnosis, 31 patients had pretibial lesions. Thirteen patients (37%) had a single lesion at diagnosis, and the mean number of lesions was 3.37. Twenty-three patients (65.71%) had diabetes mellitus (type 1 in 10 cases and type 2 in 13). In 20 patients, onset of diabetes preceded that of NL by a mean of 135.70 months. The 2 conditions were diagnosed simultaneously in 3 patients. None of the 35 patients developed diabetes mellitus during follow-up. Six patients had hypothyroidism, and 4 of these also had type 1 diabetes. Conclusions: NL is frequently associated with type 1 and 2 diabetes. Although diabetes tends to develop before NL, it can occur simultaneously (AU)