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BACKGROUND AND OBJECTIVE: subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma with indolent behavior, mostly present in women and associated with immunological diseases whose pathogenic background is still poorly understood. SPTCL is associated with lupus erythematosus panniculitis (LEP) and histologically misdiagnosed. OBJECTIVES: the aim of our study was to identify mutations affecting the pathogenesis of both SPTCL and LEP. MATERIALS AND METHODS: we studied a total of 10 SPTCL and 10 LEP patients using targeted Next Generation Sequencing and pyrosequencing. Differences in gene expression between molecular subgroups were investigated using NanoString technology. Clinical data were collected, and correlations sought with the molecular data obtained. RESULTS: the mutational profile of SPTCL and LEP is different. We identified fewer pathogenic mutations than previously reported in SPTCL, noting a single HAVCR2-mutated SPTCL case. Interestingly, 40% of our SPTCL cases showed the pathogenic TP53 (p.Pro72Arg) (P72R) variant. Although cases showing HAVCR2 mutations or the TP53 (P72R) variant had more severe symptomatic disease, none developed hemophagocytic syndrome (HPS). Furthermore, TP53 (P72R)-positive cases were characterized by a lower metabolic signaling pathway and higher levels of CD28 expression and Treg signaling genes. In addition, 30% of our cases featured the same mutation (T735C) of the epigenetic modificatory gene DNMT3A. None of the LEP cases showed mutations in any of the studied genes. CONCLUSIONS: the mutational landscape of SPTCL is broader than previously anticipated. We describe, for the first time, the involvement of the TP53 (P72R) pathogenic variant in this subgroup of tumors, consider the possible role of different genetic backgrounds in the development of SPTCL, and conclude that LEP does not follow the same pathogenic pathway as SPTCL.
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BACKGROUND AND OBJECTIVES: Bexarotene has been approved to treat advanced stage cutaneous T-cell lymphomas (CTCL) since 1999. However, very few data have been published on its long-term safety and efficacy profile. The aim of this study is to determine the tolerability to bexarotene and outcomes by collecting the 2nd largest case series to date on its long-term use vs CTCL. MATERIAL AND METHOD: This was a multicenter retrospective review of 216 patients with mycosis fungoides (174), or Sézary syndrome (42) on a 10-year course of bexarotene alone or in combination with other therapies at 19 tertiary referral teaching hospitals. RESULTS: A total of 133 men (62%) and 83 women (38%) were included, with a mean age of 63.5 year (27-95). A total of 45% were on bexarotene monotherapy for the entire study period, 22% started on bexarotene but eventually received an additional therapy, 13% were on another treatment but eventually received bexarotene while the remaining 20% received a combination therapy since the beginning. The median course of treatment was 20.78 months (1-114); and the overall response rate, 70.3%. Complete and partial response rates were achieved in 26% and 45% of the patients, respectively. Treatment was well tolerated, being the most common toxicities hypertriglyceridemia (79%), hypercholesterolemia (71%), and hypothyroidism (52%). No treatment-related grade 5 adverse events were reported. CONCLUSIONS: Our study confirms bexarotene is a safe and effective therapy for the long-term treatment of CTCL.
Subject(s)
Bexarotene , Mycosis Fungoides , Sezary Syndrome , Skin Neoplasms , Tetrahydronaphthalenes , Humans , Bexarotene/therapeutic use , Male , Female , Aged , Retrospective Studies , Middle Aged , Aged, 80 and over , Skin Neoplasms/drug therapy , Adult , Tetrahydronaphthalenes/therapeutic use , Tetrahydronaphthalenes/adverse effects , Mycosis Fungoides/drug therapy , Sezary Syndrome/drug therapy , Spain , Lymphoma, T-Cell, Cutaneous/drug therapy , Treatment Outcome , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/adverse effectsABSTRACT
BACKGROUND AND OBJECTIVES: Bexarotene has been approved to treat advanced stage cutaneous T-cell lymphomas (CTCL) since 1999. However, very few data have been published on its long-term safety and efficacy profile. The aim of this study is to determine the tolerability to bexarotene and outcomes by collecting the 2nd largest case series to date on its long-term use vs CTCL. MATERIAL AND METHOD: This was a multicenter retrospective review of 216 patients with mycosis fungoides (174), or Sézary syndrome (42) on a 10-year course of bexarotene alone or in combination with other therapies at 19 tertiary referral teaching hospitals. RESULTS: A total of 133 men (62%) and 83 women (38%) were included, with a mean age of 63.5 year (27-95). A total of 45% were on bexarotene monotherapy for the entire study period, 22% started on bexarotene but eventually received an additional therapy, 13% were on another treatment but eventually received bexarotene while the remaining 20% received a combination therapy since the beginning. The median course of treatment was 20.78 months (1-114); and the overall response rate, 70.3%. Complete and partial response rates were achieved in 26% and 45% of the patients, respectively. Treatment was well tolerated, being the most common toxicities hypertriglyceridemia (79%), hypercholesterolemia (71%), and hypothyroidism (52%). No treatment-related grade 5 adverse events were reported. CONCLUSIONS: Our study confirms bexarotene is a safe and effective therapy for the long-term treatment of CTCL.
Subject(s)
Bexarotene , Mycosis Fungoides , Sezary Syndrome , Skin Neoplasms , Tetrahydronaphthalenes , Humans , Bexarotene/therapeutic use , Male , Female , Aged , Retrospective Studies , Middle Aged , Aged, 80 and over , Skin Neoplasms/drug therapy , Adult , Tetrahydronaphthalenes/therapeutic use , Tetrahydronaphthalenes/adverse effects , Mycosis Fungoides/drug therapy , Sezary Syndrome/drug therapy , Spain , Lymphoma, T-Cell, Cutaneous/drug therapy , Treatment Outcome , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/adverse effectsABSTRACT
Antecedentes y objetivos Los linfomas cutáneos primarios (LCP) son un conjunto de entidades poco frecuentes. En febrero del 2018 se describieron los resultados del primer año de funcionamiento del Registro de linfomas cutáneos primarios de la AEDV. En el presente trabajo actualizamos los resultados tras 5 años de funcionamiento. Pacientes y métodos Registro de enfermedad de pacientes con LCP. Se recogieron datos prospectivamente de los pacientes, incluyendo diagnóstico, tratamientos, pruebas realizadas y estado actual del paciente. Se realizó un análisis descriptivo. Resultados En diciembre del 2021 se había incluido a un total de 2020 pacientes en el Registro, pertenecientes a 33 hospitales españoles. El 59% fueron hombres, la edad media fue de 62,2 años. Se agruparon en 4grandes grupos diagnósticos: micosis fungoide/síndrome de Sézary (1.112, 55%), LCP de células B (547, 27,1%), trastornos linfoproliferativos de células T CD30+(222, 11%) y otros linfomas T (116, 5,8%). La mayoría presentó estadio T1, encontrándose actualmente casi el 75% en remisión completa (43,5%) o enfermedad estable (EE; 27%). Los tratamientos más usados fueron corticoides tópicos (1.369, 67,8%), fototerapia (890, 44,1%), cirugía (412, 20,4%) y radioterapia (384, 19%). Conclusión Las características del paciente con LCP en España no difieren de otras series. El mayor tamaño del registro permite precisar mejor los datos con respecto a los resultados del primer año. Este registro facilita al grupo de linfomas de la AEDV realizar investigación clínica, surgiendo ya trabajos publicados de dicho registro (AU)
Background and objective Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. Patients and methods RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. Results Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). Conclusion The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data (AU)
Subject(s)
Humans , Male , Female , Middle Aged , Aged , Lymphoma/classification , Lymphoma/epidemiology , Skin Neoplasms/classification , Skin Neoplasms/epidemiology , Diseases Registries/statistics & numerical data , Spain/epidemiology , Academies and InstitutesABSTRACT
Background and objective Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. Patients and methods RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. Results Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). Conclusion The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data (AU)
Antecedentes y objetivos Los linfomas cutáneos primarios (LCP) son un conjunto de entidades poco frecuentes. En febrero del 2018 se describieron los resultados del primer año de funcionamiento del Registro de linfomas cutáneos primarios de la AEDV. En el presente trabajo actualizamos los resultados tras 5 años de funcionamiento. Pacientes y métodos Registro de enfermedad de pacientes con LCP. Se recogieron datos prospectivamente de los pacientes, incluyendo diagnóstico, tratamientos, pruebas realizadas y estado actual del paciente. Se realizó un análisis descriptivo. Resultados En diciembre del 2021 se había incluido a un total de 2020 pacientes en el Registro, pertenecientes a 33 hospitales españoles. El 59% fueron hombres, la edad media fue de 62,2 años. Se agruparon en 4grandes grupos diagnósticos: micosis fungoide/síndrome de Sézary (1.112, 55%), LCP de células B (547, 27,1%), trastornos linfoproliferativos de células T CD30+(222, 11%) y otros linfomas T (116, 5,8%). La mayoría presentó estadio T1, encontrándose actualmente casi el 75% en remisión completa (43,5%) o enfermedad estable (EE; 27%). Los tratamientos más usados fueron corticoides tópicos (1.369, 67,8%), fototerapia (890, 44,1%), cirugía (412, 20,4%) y radioterapia (384, 19%). Conclusión Las características del paciente con LCP en España no difieren de otras series. El mayor tamaño del registro permite precisar mejor los datos con respecto a los resultados del primer año. Este registro facilita al grupo de linfomas de la AEDV realizar investigación clínica, surgiendo ya trabajos publicados de dicho registro (AU)
Subject(s)
Humans , Male , Female , Middle Aged , Aged , Lymphoma/classification , Lymphoma/epidemiology , Skin Neoplasms/classification , Skin Neoplasms/epidemiology , Diseases Registries/statistics & numerical data , Spain/epidemiology , Academies and InstitutesABSTRACT
BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. PATIENTS AND METHODS: RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. RESULTS: Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). CONCLUSION: The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data.
Subject(s)
Dermatology , Lymphoma, T-Cell, Cutaneous , Mycosis Fungoides , Skin Neoplasms , Venereology , Male , Humans , Middle Aged , Female , Lymphoma, T-Cell, Cutaneous/diagnosis , Lymphoma, T-Cell, Cutaneous/epidemiology , Lymphoma, T-Cell, Cutaneous/therapy , Skin Neoplasms/diagnosis , Skin Neoplasms/epidemiology , Skin Neoplasms/therapy , Registries , Mycosis Fungoides/pathologyABSTRACT
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Subject(s)
Humans , Female , Middle Aged , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/pathology , Skin Diseases/pathology , CD4-Positive T-Lymphocytes/pathology , Immunohistochemistry , Diagnosis, DifferentialABSTRACT
BACKGROUND: Reliable prognostic factors for patients with primary cutaneous anaplastic large cell lymphoma (PCALCL) are lacking. OBJECTIVE: To identify prognostic factors for specific survival in patients with PCALCL. METHODS: Using the convenience sampling method, patients with PCALCL diagnosed from May 1986 to August 2017 in 16 University Departments were retrospectively reviewed. RESULTS: One hundred eight patients were included (57 males). Median age at diagnosis was 58 years. All of them showed T1-3N0M0 stages. Seventy per cent of the cases presented with a solitary lesion, mostly at the limbs. Complete response rate after first-line treatment was 87%, and no advantage was observed for any of them (surgery, radiotherapy, chemotherapy or other approaches). Nodal and visceral progression rate was 11% and 2%, respectively. 5-year specific survival (SSV) reached 93%; 97% for T1 patients and 84% for T2/T3 patients (P = 0.031). Five-year SSV for patients developing early cutaneous relapse was 64%; for those with late or no relapse, 96% (P = 0.001). Estimated median SSV for patients showing nodal progression was 103 months (95% CI: 51-155 months); for patients without nodal progression, estimated SSV did not reach the median (P < 0.001). Nodal progression was an independent predictive parameter for shorter survival (P = 0.011). CONCLUSION: Multiple cutaneous lesions at presentation, early skin relapse and nodal progression portrait worse prognosis in patients with PCALCL.
Subject(s)
Lymphoma, Primary Cutaneous Anaplastic Large Cell/mortality , Lymphoma, Primary Cutaneous Anaplastic Large Cell/pathology , Disease Progression , Female , Humans , Lymphatic Metastasis , Male , Middle Aged , Neoplasm Staging , Prognosis , Retrospective Studies , Spain , Survival RateABSTRACT
BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year. PATIENTS AND METHODS: Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma. A descriptive analysis was performed. RESULTS: In December 2017, the registry contained data on 639 patients (60% male) from 16 university hospitals. The most common diagnoses, in order of frequency, were mycosis fungoides/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30+ T-cell lymphoproliferative disorder (CD30+ CLPD) (70 cases, 11%), and other types of T-cell lymphoma (37 cases, 5%). In total, 105 (16.5%) of the cases recorded were incident cases. The most common diagnosis in the MF/SS group was classic MF (77.3%). Half of the patients with MF had stage IA disease when diagnosed, and the majority were either in partial remission (32.5%) or had stable disease (33.1%). The most widely used treatments were topical corticosteroids (90.8%) and phototherapy. The most common form of primary CBCL was marginal zone lymphoma (50%). Almost all of the patients had cutaneous involvement only and nearly half had stage T1a disease. Most (76.1%) were in complete remission. The main treatments were surgery (55.4%) and radiotherapy (41.9%). The most common diagnosis in patients with CD30+ CLPD was lymphomatoid papulosis (68.8%). Most of the patients (31.4%) had stage T3b disease and half were in complete remission. The most common treatments were topical corticosteroids (68.8%) and systemic chemotherapy (32.9%). CONCLUSION: The characteristics of patients with primary cutaneous lymphoma in Spain do not differ from those described in other series in the literature. The registry will facilitate clinical research by the AEDV's lymphoma group.
Subject(s)
Lymphoma, B-Cell/epidemiology , Lymphoma, T-Cell, Cutaneous/epidemiology , Registries , Skin Neoplasms/epidemiology , Databases, Factual , Humans , Lymphoma, B-Cell/diagnosis , Lymphoma, B-Cell/therapy , Lymphoma, Large-Cell, Anaplastic/epidemiology , Lymphoma, T-Cell, Cutaneous/diagnosis , Lymphoma, T-Cell, Cutaneous/therapy , Lymphomatoid Papulosis/diagnosis , Lymphomatoid Papulosis/epidemiology , Prospective Studies , Spain/epidemiologyABSTRACT
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.
Subject(s)
Antimetabolites, Antineoplastic/therapeutic use , Lymphomatoid Papulosis/drug therapy , Methotrexate/therapeutic use , Phototherapy , Skin Neoplasms/drug therapy , Steroids/therapeutic use , Administration, Topical , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Disease-Free Survival , Female , Humans , Infant , Lymphomatoid Papulosis/mortality , Lymphomatoid Papulosis/therapy , Male , Middle Aged , Mycosis Fungoides/mortality , Neoplasms, Multiple Primary , Receptors, Antigen, T-Cell , Retrospective Studies , Skin Neoplasms/mortality , Skin Neoplasms/therapy , Young AdultABSTRACT
BACKGROUND: Intravenous rituximab is a safe and effective option for the treatment of systemic non-Hodgkin B-cell lymphoma. The effectiveness of intralesional rituximab (ILR) in primary cutaneous B-cell lymphomas (PCBL) has been described in a small number of patients. OBJECTIVES: To evaluate the effectiveness, tolerance and adverse effects of ILR in patients with follicle centre (FCL) and marginal zone (MZL) PCBL. METHODS: This was an epidemiological observational multicentre study of patients with PCBL treated with ILR. RESULTS: Seventeen patients with MZL and 18 with FCL PCBL were included. The median number of lesions treated was two per patient. The treatment regimen used in 74% of the patients was a course of three injections in a single week at 1-month intervals. The dose per lesion and day of treatment was 10 mg in 71% of the patients. The median cumulative dose of rituximab per lesion was 60 mg (range 13-270) and per patient was 150 mg (range 20-360 mg). Complete response (CR) and partial response were achieved in 71% and 23% of patients, respectively. The median time to CR in patients who received 10 mg of ILR per lesion was 8 weeks. Similar response rates were observed in MZL and FCL. Median disease-free survival was 114·1 weeks. No parameters that significantly predicted CR were identified. Adverse reactions were recorded in 19 patients; the most frequent was localized pain at the injection site. Median follow-up was 21 months. CONCLUSIONS: Intralesional rituximab is a well-tolerated and effective treatment for FCL and MZL PCBL. It should be considered a useful alternative in patients with recurrent lesions and in which the sequelae of radiotherapy or surgery would be significant.
Subject(s)
Antibodies, Monoclonal, Murine-Derived/administration & dosage , Antineoplastic Agents/administration & dosage , Lymphoma, B-Cell/drug therapy , Skin Neoplasms/drug therapy , Adult , Aged , Aged, 80 and over , Disease-Free Survival , Female , Humans , Injections, Intralesional , Male , Middle Aged , Rituximab , Treatment OutcomeABSTRACT
BACKGROUND: In a small number of cases of childhood atopic dermatitis, topical therapy is ineffective, necessitating prolonged use of systemic immunosuppressants. Over the last few years, a better understanding of the metabolic pathways involved in azathioprine breakdown has enabled us to use this drug more safely. In this study, we evaluated the toxicity of azathioprine treatment adjusted to thiopurine methyltransferase activity in children with severe atopic dermatitis. MATERIAL AND METHODS: We performed a retrospective study of the side effects of azathioprine therapy adjusted to thiopurine methyltransferase activity in children aged under 14 years with atopic dermatitis who were treated in the dermatology department of Hospital Universitario Insular de Gran Canaria in Gran Canaria, Spain. Side effects were evaluated by analysis of leukocyte count and transaminase levels at baseline, after 1 month of treatment, and every 3 months thereafter. RESULTS: During the last 4 years, 7 children (mean age, 10 years) with severe atopic dermatitis received azathioprine in our department. Mean duration of treatment was 12 months (range, 1 to 38 months). Only 2 patients presented mild transient leukopenia that did not require treatment to be suspended. DISCUSSION: Our experience shows that, when adjusted to thiopurine methyltransferase activity, azathioprine is a safe drug for the treatment of children with severe atopic dermatitis. However, clinical trials should be performed to compare the risk-benefit ratios of the different immunosuppressants used to treat these patients.
Subject(s)
Azathioprine/adverse effects , Dermatitis, Atopic/drug therapy , Dermatitis, Atopic/enzymology , Immunosuppressive Agents/adverse effects , Methyltransferases/metabolism , Adolescent , Azathioprine/therapeutic use , Child , Female , Humans , Immunosuppressive Agents/therapeutic use , Male , Retrospective Studies , Severity of Illness IndexABSTRACT
Introducción: Existe un pequeño número de casos de dermatitis atópica (DA) infantil donde la terapia tópica es insuficiente para el control de la enfermedad y es necesario el uso prolongado de agentes inmunosupresores sistémicos. En los últimos años el mejor conocimiento de las vías metabólicas implicadas en la catabolización de la azatioprina (AZT) parece ofrecernos un uso más seguro de este fármaco. Hemos evaluado la toxicidad de la AZT según los niveles de tiopurina metiltransferasa (TPMT) en niños con DA grave. Material y métodos: Estudio retrospectivo de los efectos secundarios de la AZT ajustada a los niveles de TPMT en menores de 14 años con DA que han sido tratados en el Servicio de Dermatología del Hospital Universitario Insular de Gran Canaria. La evaluación de los efectos secundarios se realizó mediante controles analíticos con recuento leucocitario y de transaminasemia en la visita basal, al mes y cada tres meses durante el tratamiento. Resultados: En los últimos 4 años, 7 niños (media de 10 años) con DA grave han recibido AZT en nuestro Servicio. La duración media del tratamiento fue de 12 meses (rango 138); sólo dos pacientes presentaron una leucopenia leve transitoria que no requirió la suspensión del tratamiento. Discusión: Según nuestra experiencia la AZT, ajustada a los niveles de TPMT, se muestra como un fármaco seguro en el tratamiento de niños con DA grave, aunque son precisos ensayos clínicos que comparen la relación riesgo/beneficio entre los inmunosupresores empleados en estos pacientes (AU)
Background: In a small number of cases of childhood atopic dermatitis, topical therapy is ineffective, necessitating prolonged use of systemic immunosuppressants. Over the last few years, a better understanding of the metabolic pathways involved in azathioprine breakdown has enabled us to use this drug more safely. In this study, we evaluated the toxicity of azathioprine treatment adjusted to thiopurine methyltransferase activity in children with severe atopic dermatitis. Material and methods: We performed a retrospective study of the side effects of azathioprine therapy adjusted to thiopurine methyltransferase activity in children aged under 14 years with atopic dermatitis who were treated in the dermatology department of Hospital Universitario Insular de Gran Canaria in Gran Canaria, Spain. Side effects were evaluated by analysis of leukocyte count and transaminase levels at baseline, after 1 month of treatment, and every 3 months thereafter. Results: During the last 4 years, 7 children (mean age, 10 years) with severe atopic dermatitis received azathioprine in our department. Mean duration of treatment was 12 months (range, 1 to 38 months). Only 2 patients presented mild transient leukopenia that did not require treatment to be suspended. Discussion: Our experience shows that, when adjusted to thiopurine methyltransferase activity, azathioprine is a safe drug for the treatment of children with severe atopic dermatitis. However, clinical trials should be performed to compare the risk-benefit ratios of the different immunosuppressants used to treat these patients (AU)
Subject(s)
Humans , Male , Female , Child , Adolescent , Azathioprine/classification , Azathioprine/pharmacokinetics , Azathioprine/therapeutic use , Dermatitis, Atopic/complications , Dermatitis, Atopic/diagnosis , Dermatitis, Atopic/therapy , Immunosuppressive Agents/metabolism , Immunosuppressive Agents/pharmacokinetics , Immunosuppressive Agents/therapeutic useABSTRACT
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Subject(s)
Humans , Female , Adult , Alopecia/complications , Alopecia/diagnosis , Alopecia/therapy , Fibrosis/complications , Fibrosis/diagnosis , Hair/pathology , Hair Diseases/complications , Hair Preparations/adverse effects , Minoxidil/therapeutic use , Dermis/pathology , Diagnosis, Differential , Anti-Inflammatory Agents/therapeutic use , Adrenal Cortex Hormones/therapeutic use , Immunosuppressive Agents/therapeutic use , Tetracyclines/therapeutic useABSTRACT
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Subject(s)
Humans , Female , Middle Aged , Lupus Erythematosus, Cutaneous/drug therapy , Antibodies, Monoclonal/therapeutic use , /therapeutic use , Adrenal Cortex Hormones/therapeutic useABSTRACT
BACKGROUND: Neonatal lupus erythematosus (NLE) is a disease associated with the transplacental transfer of maternal anti-Ro/SSA. The histopathologic characteristics of neonatal lupus have been described as compatible with cutaneous lupus based on isolated cases. METHODS: We retrospectively review the available literature and compare them with findings obtained in seven biopsies of five cases. RESULTS: Erythematous-desquamative lesions and urticaria-like lesions were observed in our series. Two cases showed both type of lesions. Vacuolar alterations at the dermoepidermal interface and adnexal structures were the histopathologic findings on erythematous-desquamative lesions, and a superficial and deep perivascular and periadnexal lymphocytic infiltrate was the major pattern in urticaria-like lesions. One case showed prevalence of eosinophils in the inflammatory infiltrate. Sixty cases have been reported previously. Sixty-five percent presented erythematous-desquamative and 29% urticaria-like lesions. Pathologic findings of erythematous-desquamative lesions were similar to those found in our series, but epidermal vacuolar changes were the predominant histopathologic finding in urticaria-like lesions of cases reported in the literature. CONCLUSIONS: The majority of cases of NLE show vacuolar alteration at the dermoepidermal interface and adnexal structures. Some cases exhibit a superficial and deep perivascular and periadnexal lymphocytic infiltrate without epidermal alteration, and rare cases may have eosinophils in the infiltrate.