Oral and Cutaneous Lymphomas other than Mycosis Fungoides and Sézary Syndrome in a Mexican Cohort: Recategorization and Evaluation of International Geographical Disparities.

BACKGROUND: Nonmycosis fungoides/Sézary syndrome (non-MF/SS) primary cutaneous lymphomas (PCL) are currently categorized under the 2005-World Health Organization/European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for PCL. These differ in behavior from secondary cutaneous lymphomas (SCL) and to lymphomas limited to the oral cavity (primary oral lymphomas [POL]) both categorized under the 2016-WHO classification for lymphoid neoplasms. AIMS: This study aims to report the first series of non-MF/SS PCL, SCL, and POL in a Mexican cohort, examine the applicability of current classification systems and compare our findings with those from foreign cohorts. MATERIALS AND METHODS: Eighteen non-MF/SS PCL, four SCL, and two POL with available tissue for morphology and immunophenotypic assessment were reclassified according to the 2005-WHO/EORTC and 2016-WHO classifications. RESULTS: Non-MF/SS PCLs were primarily of T-cell origin (61%) where CD30+ lymphoproliferative disorders predominated, followed by Epstein-Barr virus-induced lymphomas, and peripheral T-cell lymphomas, not otherwise specified. Primary cutaneous B-cell lymphomas (BCL) were primarily of follicle center cell origin followed by postgerminal lymphomas of the diffuse large BCL variety. CONCLUSIONS: Most non-MF/SS PCL, SCL, and POL can be adequately categorized according to the 2005-WHO/EORTC and 2016-WHO classification systems, even when dealing with clinically atypical cases. The relative frequencies in our cohort hold closer similarities to Asian registries than from those of Europe/USA, supporting the concept of individual and/or racial susceptibility, and the notion of geographical variances in the rate of lymphomas. In particular, such disparity may arise from viral-induced lymphomas which might show partial geographical restriction.

Genetic association of IFN-γ +874T/A polymorphism in Mexican patients with drug-induced Stevens-Johnson syndrome/toxic epidermal necrolysis.

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) are rare, but potentially life-threatening diseases, characterized by widespread epidermal necrosis and are predominantly drug induced. There is a paucity of data regarding the role of cytokine and cytokine receptors polymorphisms in the pathoimmunology of SJS/TEN. The aim of this study was to investigate the role of TNF-α-308, IFN-γ +874, IL-10-1082, IL-13 Arg130Gln, and IL-4R Gln551Arg gene polymorphisms in SJS/TEN in Mexican Mestizo patients. Twenty-nine unrelated SJS/TEN patients and 128 unrelated healthy individuals were studied. Genomic extraction was carried out from complete blood samples using the salting out method. The PCR-RFLP method was used to amplify the following polymorphisms: TNF-α-308, IFN-γ +874, IL-10-1082, IL-13 Arg130Gln, and IL-4R Gln551Arg. TNF-α-308, IL-10-1082, IL-13 Arg130Gln, and IL-4R Gln551Arg polymorphisms were not associated with the genetic susceptibility to SJS/TEN. The distribution of TT, TA, AA genotypes of IFN-γ +874 was significantly different in SJS/TEN patients compared with controls (pC = 0.012). TA and AA genotypes were grouped to highlight the differences between patients and controls given by the absence of the AA genotype in the group of patients (pC = 0.03, OR = 3.61 95 % CI 1.20-11.6). This preliminary study suggests that IFN-γ +874 T/A polymorphism is associated with SJS/TEN.

[Dermatoses in patients with acute myeloid and lymphoid leukemia. Cohort follow-up in a tertiary care hospital]. / Dermatosis en pacientes con leucemia aguda mieloide y linfoide. Seguimiento de una cohorte en un hospital de tercer nivel.

INTRODUCTION: Cutaneous manifestations in patients with acute leukemia (AL) cover a broad spectrum, including those due to leukemia per se, to chemotherapy and other drugs and those inherent to hospital care. MATERIAL AND METHODS: This is a cohort study in a tertiary hospital setting where the development of dermatoses was followed for 2 years in 22 patients with the diagnosis of AL. RESULTS: During the study, all patients developed some type of dermatosis, mostly due to chemotherapy.

[Multicentric and localized tineas in immunocompromised patients]. / Tiñas multifocales y localizadas en pacientes inmunosuprimidos.

From the dermatological point of view, multifocal or multicentric tineas are widespread dermatophytic infections affecting two or more anatomical areas. In the immunosuppressed patient, these lesions are frequently atypical and the risk factors are not well established. The aims of this study were: to determine the risk factors associated to multicentric tinea in immunocompromised patients; to evaluate the immune response by trichophytin and candidin skin test, to determine the etiological agent and to quantify some serum interleukines. Thirty-six multicentric tinea and 37 localized tinea patients, both with immunocompromised factors, were included. By means of a questionnaire several risk factors were identified; the trichophytin and candidin skin test was evaluated after 48 hours. Mycological direct examination and culture were performed. The interleukins IL-2, IL-4, IL-10 and interferon gamma were quantified by ELISA. Statistical analysis was made by Chi-square, U Mann Whitney and logistic regression. In disseminated tinea patients a predominance of females (69%) versus localized tinea patients (30%) was observed. Prednisone, azathioprine and cyclophosphamide treatment was associated to multicentric tinea. Trichophytin was negative in all disseminated tinea patients and positive in only three localized tinea cases, candidin was positive in six and eight cases of multicentric and localized tinea respectively. Trichophyton rubrum was the most frequent etiological agent. No differences in interleukin concentrations were found. Female gender and some immunosuppressor treatments were associated with a high probability to develop multicentric tinea. In this study a defect in the cellular immune response was the possible explanation for the extensive reactions.

Immunohistochemistry screening of sebaceous lesions for Muir-Torre syndrome in a 26-year period in a Mexican population.

Muir-Torre syndrome (MTS) is an autosomal dominant genodermatosis defined as the association of rare sebaceous gland skin tumors, keratoacanthomas, and a personal or familial history of malignant visceral tumors. Germline mutations in certain mismatch repair genes (MMR) have been identified in MTS families and their identification is a cornerstone for diagnosis of MTS. We reviewed our series of sebaceous neoplasms and performed immunohistochemistry (IHC) in order to screen for new MTS cases. Sebaceous neoplasms and visceral tumors from the same patient diagnosed between 1980-2006 were included. Immunohistochemistry to determine the presence or absence of MMR gene products in skin and visceral tumors was performed with mouse monoclonal antibodies anti-MSH2, anti-MSH6 and anti-MLH1. Six sebaceous neoplasms were identified in six females. Four patients presented a lack of expression of at least one of the MMR proteins in visceral and cutaneous neoplasms, thus warranting the diagnosis of MTS. Immunohistochemistry is a useful and accessible technique for the characterization of MMR gene expression in patients with sebaceous neoplasms.

Epidemiology of adverse cutaneous drug reactions. A prospective study in hospitalized patients.

BACKGROUND: Drug reactions are commonly present in the skin; however, their frequency in our setting is unknown. METHODS: A 10-month prospective cohort study including all hospitalized patients was designed. Those with adverse cutaneous drug reactions (ACDR) were clinically identified. RESULTS: Thirty five drug reactions (prevalence of 0.7%) were seen among 4785 (2713 females, 2072 males) discharged patients. According to Begaud's imputability criteria, the reactions were most likely attributed to a drug in 4.87%, likely in 41.46% and possible in 53.65%. The most commonly seen dermatoses were morbilliform rash 51.2%, urticaria 12.2% and erythema multiforme 4.9%. Drugs most frequently associated with ACDR were amoxicillin clavulanate (8), amphotericin B (2) and metamizole (4). Expressed as risk by 1000 day-doses (Dd: the risk a patient has of developing an ACDR after receiving 1 day of treatment with the drug): amoxicillin clavulanate Dd 7.7, amphotericin B Dd 4.8 and metamizole Dd 3.7. Immunosuppressed patients were most frequently affected. Notably, patients with systemic lupus erythematosus (SLE) had a 4.68 higher risk (CI 95% 1.794-12.186 p <0.001) of developing an ACDR. AIDS patients showed a risk of 8.68 (CI 95% 2.18-33.19 p <0.001). Non-Hodgkin's lymphoma patients also had an increased risk of developing an ACDR. Six of the 35 identified cases were patients who had been hospitalized due to a severe drug reaction (1.3/1000 patients); one died from complications directly related to the ACDR, representing a 16.6% mortality rate among those admitted for an ACDR and 0.02% among the global mortality. CONCLUSIONS: We have a low prevalence of drug reactions compared to data reported in the literature. Pharmacovigilance with special attention to immunosuppressed SLE or AIDS patients is stressed.

Diagnostic challenge of glucagonoma: case report and literature review.

OBJECTIVE: To report the diagnostic difficulties encountered in a case of glucagonoma. METHODS: We provide a literature review and present the clinical findings, pertinent laboratory data, and results of related studies in a patient with a glucagonoma. RESULTS: A 54-year-old-man, with no relevant history of endocrine disorders, presented to the hospital with a 5-year history of recurrent stomatitis and glossitis, a more recent weight loss of 11.5 kg, and recurrent pruritic maculae on the scalp in conjunction with raised erythematous maculae in the scrotal region and perineum that gradually migrated to the distal extremities, becoming bullous and painful. The patient was hospitalized, and because of the dermatologic findings suggestive of necrolytic migratory erythema, the presence of a glucagonoma was suspected. His blood glucose levels were in the normal range. Glucagon levels were found to be elevated, and imaging studies confirmed the presence of an enlarged mass in the pancreatic tail, without evidence of extension to surrounding structures. Liver metastatic lesions were also excluded. After surgical removal of the tumor, the skin and oral mucosal lesions disappeared spontaneously. The histologic appearance and immunohistochemical staining results confirmed the diagnosis of a glucagonoma. Subsequently, all related symptoms resolved, and the glucagon levels normalized. CONCLUSION: The diagnosis of glucagonoma is often delayed. Clinicians should be aware of the unusual initial manifestations of this tumor and the potential for less than a full spectrum of the characteristic features of the glucagonoma syndrome.

Incidence and risk factors for cutaneous adverse drug reactions in an intensive care unit / Incidencia y factores de riesgo en las reacciones adversas medicamentosas de tipo cutáneo en la unidad de cuidados intensivos

Objective. To evaluate the incidence of adverse cutaneous drug reactions in intensive care unit patients. Design. Cohort study. Setting. General adult intensive care unit of an institutional tertiary care hospital. Patients. Patients in the intensive care unit during a consecutive 8-month period were examined for adverse cutaneous drug reactions. Results. Patients in the intensive care unit have an incidence of 11.6% of adverse cutaneous drug reactions. Associated risk factors were female gender, obesity, age over 60 and immune dysregulation (systemic lupus erythematosus, dysthyroidism, and antiphospholipid antibodies syndrome). Few patients had previous history of adverse cutaneous drug reactions. Antimicrobials were the main drug involved. Morbilliform rash followed by urticary were the most frequently observed reactions. Interestingly, over 50% of patients with massive edema -independent of etiology- died. Conclusions. Intensive care unit patients are particularly at risk for developing an adverse cutaneous drug reaction.

Se realizó un estudio de cohorte en la Unidad de Terapia Intensiva (UTI) de un hospital de tercer nivel para evaluar la incidencia de reacciones cutáneas adversas a drogas. Se examinaron todos los pacientes internados en dicha unidad durante un periodo consecutivo de ocho meses. Observamos una incidencia de reacciones adversas a medicamentos de 11.6%. Los factores de riesgo asociados fueron sexo femenino, obesidad, edad mayor a 60 años y alteraciones inmu-nológicas (lupus eritematoso sistémico, distiroidismo y síndrome de antifosfolípido). Los antimicrobianos fueron los principales medicamentos involucrados. La erupción morbiliforme y la urticaria fueron las reacciones más frecuentes. Un hallazgo interesante fue que más de 50% de los pacientes con anasarca fallecieron. Concluimos que los pacientes internados en la UTI son particularmente susceptibles para desarrollar una reacción adversa cutánea a medicamentos.

Histoplamosis cutánea en nueve pacientes con SIDA / Cutaneous histoplasmosis in nine patients with AIDS

El diagnóstico de histoplasmosis diseminada en pacientes con SIDA representa un reto. Cuando la histoplasmosis se manifiesta inicialmente en piel, el diagnóstico conlleva mayor dificultad por presentar un cuadro clínico inespecífico. Presentamos el curso de nueve pacientes con SIDA e histoplasmosis cutánea atendidos de 1987 a 1998. El diagnóstico se estableció por el estudio histopatológico y el asilamiento del hongo de la piel. Las lesiones cutáneas más frecuentes fueron pápulas (6/9) combinadas con nódulos o pústulas en 6/9 casos; (1/9), placas y nódulos (1/9). La dermatosis, pero todos cursaron con fiebre, 7/9 con hepatomegalia y esplenomegalia, y 5/9 con pérdida de peso. La cuenta de linfocitos CD4+ al diagnóstico de histoplasmosis fue baja (media de 47.7 células µL). El tratamiento fue con anfotericina B e intraconazol para manejo supresivo y de mantenimiento. Cinco pacientes murieron. Uno, un día posterior al diagnóstico y cuatro a los 5, 8, 11 y 12 meses del inicio del tratamiento. Cuatro están vivos a los 3, 3, 19 y 26 meses de seguimiento. Conclusiones. La histoplasmosis cutánea en pacientes con SIDA se debe considerar cuando existan pápulas o placas en cara y tronco con fiebre y hepato y esplenomegalia. El estudio histopatológico y el cultivo de piel o de médula ósea fueron procedimientos útiles para el diagnóstico, pero la biopsia de piel permitió un diagnóstico más rápido

Two cases of psychogenic purpura

La púrpura psicógena, conocida también como hematomas dolorosos recurrentes o síndrome de autosensibilización eritrocitaria, se asocia usualmente a pacientes con alteraciones emocionales. Es una entidad difícil para el binomio médico-paciente, ya que aún los estudios clínicos y paraclínicos más extensos no orientan al diagnóstico. Presentamos dos mujeres en la tercera década de la vida con hematomas recurrentes dolorosos y diversos síntomas acompañantes que iniciaron posterior a un traumatismo físico. Los psiquiatras diagnosticaron un trastorno de personalidad tipo limítrofe (borderline) en una paciente, y un trastorno facticio en la otras. Las biopsias de piel revelaron un infiltrado inflamatorio perivascular, extravasación de eritrocitos y ausencia de vasculitis. Las alteraciones hemorrágicas psicógenas son poco frecuentes pero deben ser incluidas en el diagnóstico diferencial de las púrpuras para evitar tratamientos mutilantes y agresivos. En su patogenia se implican mecanismos autoinmunes y actividad fibrinolítica cutánea aumentada, pero se requieren más estudios

Sarcoidosis cutánea en tres pacientes mexicanos: análisis clínico-patológico de las variantes papular, nodular y en placa / Cutaneous sarcoidosis in three Mexican patients: clinical pathologic analysis of papullar nodular and in plaques varieties

La sarcoidisis es una afección clínico-patológica de causa desconocida que se caracteriza por granulomas sin necrosis. El cuadro clínico descrito con mayor frecuencia es de linfanopatía hiliar bilateral, infiltrados pulmonares y lesiones cutáneas. Al revisar la literatura mexicana de 1964 a 1995 al parecer sólo se han publicado siete artículos de sarcoidosis y en dos selectivamente se describen las manifestaciones cutáneas. Se comunican los halllazgos clínicos e histopatológicos de tres pacientes mexicanos con sarcoidosis, cuyo diagnóstico se sospechó por las lesiones en la piel. Los tres pacientes tuvieron manifestaciones intratorácicas; el diagnóstico de sarcoidisis se confirmó por biopsia y todos respondieron satisfactoriamente al tratamiento con esteroides. Una paciente desarrolló enfermedad de addison como complicación endocrina infrecuente de la sarcoidosis.

Pytirosporum ovale en dermatitis seborreica. Estudio en 26 pacientes con SIDA y 21 controles / Pytirosporum ovale in seborrheic dermatitis. a study of 26 patients with AIDS and 21 controls

La dermatitis seborreica (DS) ha sido informada como una entidad frecuente en pacientes con SIDA. En este estudio evaluamos 26 pacientes con SIDA para conocer la prevalencia de DS y su relación con la cuenta de Pytirosporum ovale mediante tinción de Gram y cinta adhesiva transparente, en comparación con controles hospitalizados por enfermedad no relacionadas con el SIDA ni neurológicas. Los resultados fueron semejantes en ambos grupos: la cuenta de P. ovale correlacionó con la forma clínica de la DS; no hubo diferencia entre pacientes con SIDA y controles, ni en los que tomaban ketoconazol. La cuenta de P. ovale fue semejante en las áreas corporales en que se tomaron las muestras. Las técnicas de Gram y cinta adhesiva transparente fueron comparables para cuenta de P. ovale. Conclusiones. En nuestro medio, la DS no es un hallazgo frecuente en población general ni en pacientes con SIDA, y no hay diferencias en el número de P. ovale encontrado en comparación con controles; estos resultados pueden sugerir que la DS, para expresarse, requiere de factores raciales y que se trata de una entidad diferente en la que el P. ovale no es un factor importante en la fisiopatogenia de la enfermedad

Síndrome de hipersensibilidad por alopurinol: un caso de necrosis hepática masiva / Allopurinol hypersernsitivity syndrome: a case with massive hepatic necrosis

Los efectos o reacciones adversas a medicamentos, dependiendo del órgano blanco, tienen diversas manifestaciones clínicas que varían de grado e intensidad. Debido a que la piel es un órgano visible y fácil de explorar, las dermatosis por medicamentos son una complicación frecuente y en ocasiones forman parte de un padecimiento sistémico. Se discute el caso de una mujer de 72 años con antecedente de automedicación con alopurinol a dosis no especificadas, dos semanas previas al inicio de los síntomas. El cuadro clínico se caracterizó por dermatosis generalizada con daño renal y hepático. Posteriormente la enfermedad desarrolló síndrome de Stevens-Johnson, insuficiencia renal, hepatitis tóxica con necrosis masiva corroborada por biopsia transyugular; además cursó con choque cardiogénico y hemorragia del tubo digestivo como eventos finales. La paciente falleció y no se realizó el estudio postmórtem. El curso clínico y las complicacione en nuestra paciente corresponden al síndrome de hipersensibilidad al alopurinol. La relevancia del caso se basa en la extensión del daño hepático, el cual es un efecto tóxico infrecuente por este medicamento. El síndrome de hipersensibilidad al alopurinol es una complicación impredecible y grave

Eritrodermia, estudio retrospectivo de 23 casos: desarrollo de un algoritmo de estudio / Erythrodermia, retrospective study of 23 cases, and algorithm development

Se llevó a cabo un estudio retrospectivo de 23 pacientes con eritrodermia (ED) vistos en el Instituto Nacional de la Nutrición durante un periodo de 9 años. Las características clínicas fueron similares a otras series publicadas en la literatura. Las causas más frecuentes fueron: Psoriasis 22 por ciento, dermatitis atópica 17 por ciento, micosis fungoides 17 por ciento, eritrodermia idiopática 17 por ciento, reacción medicamentosa 13 por ciento y otros 21.6 por ciento. En un promedio de 22 meses después del diagnóstico el 78 por ciento de los casos se encontraban en remisión, siendo la prednisona la droga más usada en el tratamiento (21 casos). Cinco enfermos murieron, tres de ellos por infecciones asociadas al tratamiento. Los datos de mayor utilidad para el diagnóstico diferencial fueron la historia de dermatosis previa, o exposición a medicamentos, la presencia de adenomegalia, eosinofilia, la biopsia de piel y en algunos casos la de ganglio. Se propone un algoritmo de estudio de ED a partir de la historia clínica y de los resultados de laboratorio e histopatología

Pustulosis exantemática generalizada: dermatosis diferente al eritema polimorfo / Acute generalized exanthematous (AGEP) a specific entity, different to erythema multiforme

Informamos el caso de una mujer con pustulosis exantemática aguda generalizada (PEAG). Generalmente secundaria a medicamentos, se caracteriza por curso agudo, fiebre, múltiple pústulas puntiformes y resolución espontánea rápida. En vista de sus características particulares clínico-patológicas, ésta debe considerarse como una entidad específica