Foliculitis pseudolinfomatosa de McNutt (localización nasal) / McNutt's pseudolymphomatous folliculitis (nasal located)

La foliculitis pseudolinfomatosa, descripta por McNutt en 1986, es una afección de etiología desconocida y poco frecuente, que simula un linfoma cutáneo tanto por su clínica como por su histología. Se presenta como una lesión nodular solitaria, eritematosa, de 0,5 hasta 3cm, de crecimiento rápido, sobre todo en la cara, en personas de 40 a 60 años, con una histopatología caracterizada por un infiltrado linfocitario B yT perifocular, y células dendríticas positivas en la inmunohistoquímica para S100yCD1a. Su curso es benigno, muchas veces autolimitado. Se expone el caso de una paciente con una particular forma clínica de pseudolinforma.

Pseudolymphomatous folliculitis, described by McNutt in 1986, is a non-frequent entity of unknown etiology that simulates a cutaneous lymphoma, both clinically and histologically. It shows as a solitary erythematous nodular lesion of 0.5 to 3 cm, with a rapid growth, mainly on the face, in people aged 40 to 60 years, and histopathology characterized by a perifollicular B and T lymphocytic infiltrate, and positive dendritic cells for immunohistochemistry S100 and CD1a. Its course is benign, often self-limited. The case of a patient with a particular clinical form of pseudolymphoma is presented.

Rosettes in T-cell pseudolymphoma: a new dermoscopic finding

Abstract Rosettes are small white structures visible with polarized light dermoscopy, whose exact morphological correlation is not yet defined. These small shiny structures are found in several conditions such as scarring, dermatofibroma, molluscum contagiosum, squamous cell carcinoma, basal cell carcinoma, melanoma, melanocytic nevus, discoid lupus erythematosus, and papulopustular rosacea. In this novel report, the authors describe the presence of rosettes in a T-cell pseudolymphoma lesion.

Estudios de laboratorio como herramienta en la identificación de apendicitis aguda y su diferenciación con hiperplasia linfoide / Laboratory studies as a tool for the differential diagnosis between acute appendicitis and lymphoid hyperplasia

RESUMEN Antecedentes: La apendicitis aguda (AA) es una de las principales patologías quirúrgicas en México y el mundo. A pesar de ser una patología frecuente, el manejo quirúrgico presenta una tasa del 10-20% de apendicitis blancas (AB) o hiperplasia folicular linfoide (HPL) por histopatología. Objetivo: Comparar los hallazgos clínicos, de laboratorio y radiológicos de pacientes con sospecha de AA con su diagnóstico por histopatología. Material y métodos: El estudio es retrospectivo, observacional y transversal. Se analizaron los datos de los pacientes con diagnóstico clínico de apendicitis aguda, desde febrero de 2013 hasta diciembre de 2017, atendidos en un hospital de tercer nivel en Monterrey, México. Se compararon los hallazgos de los pacientes con diagnóstico de AA vs. HPL, AA no complicadas vs. AA complicadas y pacientes pediátricos vs. adultos. Se realizó un análisis descriptivo por medio de frecuencias y porcentajes, y mediana y rango intercuartil (RIC), dada la distribución no paramétrica de estas variables. Se compararon los hallazgos por laboratorio mediante la prueba de Mann-Whitney. Se consideró una p< 0,05 como estadísticamente significativa. Resultados: En nuestro medio, ambos sexos tienen la misma frecuencia de presentación de AA. El tiempo de evolución es significativo en la presentación frecuente de AA complicada. Existe asociación entre leucocitosis, neutrofilia total y porcentual y recuento plaquetario mayor en presencia de AA vs. HPL. Conclusión: Los estudios de laboratorio no muestran cambios significativos en pacientes pediátricos con AA. En adultos con AA no complicada vs. apendicitis complicada, el aumento en WBC, NEU y NEU% son estadísticamente significativos.

ABSTRACT Background: Acute appendicitis (AA) is one of the main surgical pathologies in our country and worldwide. Despite being a surgery that is frequently done, it is still reported a 10-20% of negative appendectomies (NA). Objective: The objective of the study is to compare clinical, laboratory and radiology results with the histopathology diagnosis. Material and methods: A retrospective study was done analyzing the data of patients with clinical diagnosis of AA from February 2013 to December 2017, in a tertiary hospital in Monterrey, Mexico. They were classified by their histopathological results into different groups: AA or NA; and the AA was subdivided into complicated AA and uncomplicated AA. Finally, these groups were also subdivided by ages, into pediatric and adult groups of each category. A descriptive analysis was made using frequencies, percentages, median and the interquartile range. Laboratory results were compared with the Mann-Whitney test. Considering a p-value of p < 0.05 as statistically significant. Results: In our group of patients both genders had AA in a similar frequency, the time between the appearance of symptoms and reaching for medical advice was an important factor for having complicated AA. There is a correlation between leukocytosis, neutrophil count and platelet count elevated in presence of AA against NA. Conclusion: Laboratory studies did not report significant changes in pediatric patients with AA. In adults with uncomplicated AA vs. complicated AA, white blood cell count, and neutrophil count are statistically significant.

Lymphocytoma cutis on the inguinal region: report of a rare case of benign lymphoproliferative disorder

Abstract Lymphocytoma cutis, or benign reactive lymphoid hyperplasia, is an inflammatory skin lesion that mimics clinically and histologically malignant lymphoma. Most cases are idiopathic, but they may also be triggered by multiple factors, such as insect bites, tattoos, injections and herpes zoster. Clinically, the lesions are erythematous, soft papules, plaques or nodules, usually located on the upper limbs and face. The diagnosis is mainly based on histopathology and immunohistochemistry. Corticosteroid injections, cryosurgery, PUVA therapy, radiotherapy and surgery can be therapeutic options in cases requiring immediate treatment. To demonstrate an atypical presentation of this tumor, a case lymphocytoma skin on the groin will be reported, describing its diagnosis and treatment.

Red nodule on the face with "spontaneous" regression

Abstract Pseudolymphomatous folliculitis is a rare entity included among the cutaneous pseudolymphomas. A 32-year-old man, with an unremarkable medical history, presented with a two-month history of an asymptomatic solitary nodule on his left cheek. Histopathological examination demonstrated a dense nodular and diffuse dermal lymphocytic infiltrate with numerous histiocytes and dendritic cells that surrounded hypertrophic hair follicles. Pseudolymphomatous folliculitis commonly presents in the fourth decade of life, with no sex predominance, as an asymptomatic, rapidly growing and solitary red dome-shaped nodule on the face. It has a benign clinical course as the lesions usually resolve with surgical excision or regress spontaneously after incisional biopsy. Although there is no report of pseudolymphomatous folliculitis progressing into lymphoma in the literature, follow-up of these patients is recommended.

Microscopic analysis of patients with chronic diarrhea without macroscopic disease / Análise microscópica de pacientes com diarreia crônica sem doença macroscópica

BACKGROUND: Colonoscopy is part of the current diagnostic armamentarium. However, in some patients with chronic diarrhea, a colonoscopy may show normal mucosa; in these cases, serial biopsies can provide important information for the diagnosis and treatment of patients. AIM: To analyze patients with chronic diarrhea having a macroscopically normal colonoscopy, by evaluating histological changes. METHODS: 30 patients with chronic diarrhea and normal colonoscopy were prospectively evaluated and submitted to serial biopsies of the terminal ileum, ascending colon and rectum. RESULTS: The sample of 30 patients showed a ratio of 18 men (60%) and 12 women (40%). On histological types, it was found that 13 patients (43.3%) had lymphoid hyperplasia, eosinophilic inflammation in 4 (13.3%), nonspecific inflammation in 4 (13.3%), regenerative changes in 3 (10%), lymphocytic colitis in 2 (6.6%) and changes consistent with Crohn's disease in 1 (3.3%). CONCLUSIONS: One can observe that even chronic diarrhea patients, without other associated factors, benefited from colonoscopy with biopsy, because it held the etiologic diagnosis in some cases as also excluded by histopathology. It was noticed that the frequency of patients with altered biopsy and less dragged diarrheal episodes (84.2%) was large, should consider their achievement.

INTRODUÇÃO: A colonoscopia faz parte do arsenal de diagnóstico atual. Porém, em alguns pacientes com diarreia crônica, a colonoscopia pode evidenciar mucosa normal; nesses casos biópsias seriadas podem trazer informações importantes para o diagnóstico e tratamento dos pacientes. OBJETIVO: Analisar pacientes com diarreia crônica submetidos à colonoscopia macroscopicamente normal, avaliando assim histologicamente as alterações. MÉTODOS: Análise prospectiva da histologia 30 pacientes com diarreia crônica e colonoscopias normais, submetidos a biópsias seriadas de íleo terminal, cólon ascendente e reto. RESULTADOS: A amostra de 30 pacientes mostrou uma proporção de 18 homens (60%) e 12 mulheres (40%). Sobre os tipos de alterações histológicas, foi verificado que 13 pacientes (43,3%) apresentaram hiperplasia linfóide, inflamação eosinofílica em 4 (13,3%), inflamação inespecífica em 4 (13,3%), alterações regenerativas em 3 (10%), colite linfocítica em 2 (6,6%) e alterações compatíveis com Doença de Crohn em 1 (3,3%). CONCLUSÕES: Observou-se que mesmo pacientes com diarreia crônica, sem outros fatores associados, beneficiaram-se da colonoscopia com biópsia, pois a mesma realizou o diagnóstico etiológico em alguns casos como também o excluiu através da histopatologia. Verificou-se que a frequência de pacientes com biópsia alterada e quadros diarreicos menos arrastados (84,2%) foi grande, devendo-se considerar a realização do exame.

Possibly drug-induced palpable migratory arciform erythema

Palpable migratory arciform erythema is an entity of unknown etiology, with few published cases in the literature. The clinical and histopathological features of this disease are difficult to be distinguished from those of Jessner’s lymphocytic infiltration of the skin, lupus erythematous tumidus and the deep erythema annulare centrifugum. We describe here the first two Brazilian cases of palpable migratory arciform erythema. The patients presented with infiltrated annular plaques and erythematous arcs without scales. These showed centrifugal growth before disappearing without scarring or residual lesions after a few days. They had a chronic course with repeated episodes for years. In addition, these cases provide evidence of a drug-induced etiology.

T-cell-predominant lymphoid hyperplasia in a tattoo

Cutaneous lymphoid hyperplasia (CLH) can be idiopathic or secondary to external stimuli, and is considered rare in tattoos. The infiltrate can be predominantly of B or T-cells, the latter being seldom reported in tattoos. We present a case of a predominantly T CLH, secondary to the black pigment of tattooing in a 35-year-old patient, with a dense infiltrate of small, medium and scarce large T-cells. Analysis of the rearrangement of T-cells receptor revealed a polyclonal proliferation. Since the infiltrate of CLH can simulate a T lymphoma, it is important to show that lesions from tattoos can have a predominance of T-cells.

Pseudolinfoma cutâneo: relato de caso / Cutaneous pseudolymphoma: case report

Os pseudolinfomas cutâneos representam um grupo heterogêneo de reações linfoproliferativas benignos que podem simular clinica e histologicamente linfomas cutâneos. O objetivo deste estudo foi relatar a apresentação anatomoclínica de um caso de pseudolinfoma cutâneo para o aperfeiçoamento do seu diagnóstico diferencial com linfomas cutâneos. Paciente do gênero masculino, 66 anos procurou atendimento médico relatando história de nódulos em região superior do dorso há seis anos. Ao exame físico foram observados sete nódulos com aproximadamente 0,5 cm de diâmetro e sem sinais de linfonodomegalias. A biópsia de pele mostrava um denso infiltrado de linfócitos e histiócitos na derme. A análise imuno-histoquímica revelou uma população mista de linfócitos B (CD 20) e linfócitos T (CD 3). O diagnóstico diferencial entre as lesões benignas e malignas é a principal prioridade nesses casos. Tanto o pseudolinfoma, quanto o linfoma cutâneo se manifestam como nódulos solitários, pápulas e placas decorrentes de infiltração linfocitária. Para o diagnóstico diferencial, estão indicadas a avaliação imuno-histoquímica e técnicas de biologia molecular...

Cutaneous pseudolymphoma represents heterogeneous groups of benign lymphoproliferative reactions that may simulate clinically and histologically cutaneous lymphomas. The aim of this study was to report a case of cutaneous pseudolymphoma. Male patient, 66-year-old, came to us reporting a 6-year history of nodules on the upper back. On physical examination we noticed 7 nodules with 0.5 cm of diameter and no sign of adenopathy. The skin biopsy showed a dense infiltrate of lymphocytes and histiocytes in the dermis. The immunohistochemical analysis revealed a mixed population of B lymphocytes (CD 20) and T lymphocytes (CD 3). Differential diagnosis between benign and malignant lesions is the main concern in these cases. In some cases, pseudolymphoma manifests as solitary nodules, papules and plaques that are clinically indistinguishable from cutaneous lymphomas. The differentiation process can be further facilitated by immunohistochemical and molecular biological techniques...

Acral pseudolymphomatous angiokeratoma: case report and literature review / Angioqueratoma pseudolinfomatoso acral: relato de caso e revisao da literatura

The authors describe a case of a female patient with Acral Pseudolymphomatous Angiokeratoma of Children, known as APACHE. It is a rare benign cutaneous disease, of unknown etiology, characterized by multiple, asymptomatic erythematous-violaceous papules and nodules, usually located unilaterally with acral distribution. Today, this denomination is questionable, since there are published reports of this disease in adults and in different locations. Clinically, it is similar to an angiokeratoma, whereas hystologically, it corresponds to a distinct type of pseudolymphoma. The immunohistochemical study is required to distinguish APACHE from cutaneous lymphoma.

Relata-se o caso de uma paciente com diagnóstico de angioqueratoma pseudolinfomatoso acral, conhecido por sua sigla em inglês APACHE - Acral Pseudolymphomatous Angiokeratoma of Children. É uma doença cutânea benigna, rara, de etiologia desconhecida, caracterizada por múltiplas pápulas e nódulos eritêmato-violáceos assintomáticos, de localização geralmente unilateral e acral. Atualmente, questiona-se esta denominação, já que há relatos na literatura do quadro em adultos e em outras localizações. Clinicamente, é similar a um angioqueratoma, porém, histologicamente, corresponde a um tipo distinto de pseudolinfoma. O estudo imuno-histoquímico é necessário para diferenciação dos linfomas cutâneos.

Lymphocytoma cutis - case report / Linfocitoma cutis - relato de caso

We describe a clinical case involving a 62-year-old white male, diagnosed with lymphocytoma cutis (Spiegler-Fendt sarcoid) in the cephalic segment. The diagnosis was carried out by pathological study and confirmed by immunohistochemical panel: evidence of polyclonality. Phototherapy sessions were suggested as treatment (13 PUVA sessions, with an accumulated dose of 58.65 J/cm2 ). The improvement was partial. Thus, infiltration of triamcinolone was opted for (one intralesional infiltration every 3 weeks). After 5 sessions, satisfactory improvement was observed: regression of nearly all the lesions.

Relatamos um caso de um paciente de 62 anos, branco e com diagnóstico de linfocitoma cutis (Sarcoide de Spigler-Fendt) em segmento cefálico. O diagnóstico foi confirmado pelo histopatológico e reafirmado pelo painel imuno-histoquímico: evidência de policlonalidade. Sessões de fototerapia foram propostas como tratamento: 13 sessões de PUVA cuja dose acumulada foi de 58.65 J/cm2); a melhora foi parcial. Optado, então, por infiltrações de triancinolona (uma infiltração intralesional a cada 3 semanas). Na quinta sessão, satisfatória melhora já podia ser evidenciada: regressão de quase todas as lesões.

Fibroma osificante periférico asociado a resorción ósea: revisión de la literatura y presentación de un caso / Peripheral ossifying fibroma associated with bone resorption: literature review and presentation of a case

El Fibroma Osificante Periférico (FOP) es una hiperplasia reactiva del tejido conjuntivo que contiene áreas de hueso. Se origina a partir del ligamento periodontal. Se presenta con frecuencia en la segunda y tercera década de vida, con mayor frecuencia en el género femenino. Se observa como una lesión sésil o pediculada, circunscrita, lisa o ulcerada, firme y del mismo color de la mucosa, asintomático. Se localiza en la encía, por lo general en papilas interdentales. En gran parte de los casos no se lesiona el hueso subyacente. Se reporta un caso de un paciente masculino de 47 años de edad, quien es referido por su odontólogo tratado por una lesión en el maxilar. El paciente refiere inicio de la enfermedad hace aproximadamente un año, que comienza con un aumento de volumen de la encía, asintomático, sangrante y asociada a dientes con movilidad dentaria. Al examen extrabucal se observa un aumento de volumen de la mejilla izquierda que produce asimetría facial. Intrabucalmente se observa una lesión de naturaleza tumoral que se desprende de la encía vestibular distal al 25 al diente 28, de aproximadamente 3cm de diámetro, circunscrita, roja, sangrante y de superficie ulcerada. El examen radiográfico revela una gran resorción ósea del hueso alveolar que produjo movilidad de los dientes 25, 27 y 28. Se planteó como diagnóstico provisional: Lesión Periférica de Células Gigantes y como diagnósticos diferenciales: Fibroma Osificante Periférico, Granuloma Piogénico. La conducta a seguir fue extirpación quirúrgica de la lesión para posterior estudio histopatológico con exodoncias de los dientes 25, 27 y 28. Se destaca la importancia de este caso por su presentación clínica de gran tamaño y la destrucción ósea que produjo, condición que determinó las exodoncias de los dientes involucrados, hecho que es inusual para este tipo de patologías

Peripheral ossifying fibroma (POF) is a reactive hyperplasia of connective tissue containing areas of bone. It originates from the periodontal ligament. It occurs frequently in the second and third decades, predominantly in females. It appears as a sessile or pedunculated lesion, circumscribed, smooth or ulcerated, firm, and the color of the mucosa, asymptomatic. Located in the gum, usually in interdental papillae. In most cases no underlying bone is injured. We report a case of a male patient aged 47 who are referred by your dentist treated for an injury to the jaw. The patient reported onset of illness for about a year, which begins with an enlargement of the gums, asymptomatic, bleeding and teeth associated with tooth mobility. Extra-oral examination showed an increase in volume of the left cheek that produces facial asymmetry. The oral examination shows tumoral lesion of nature that emerges from the buccal gingiva distal to the tooth 28 to 25, approximately 3 cm in diameter, circumscribed, red, bleeding and ulcerated surface. Radiographic examination reveals a large alveolar bone resorption resulting in tooth mobility 25, 27 and 28. Was raised as a provisional diagnosis: Peripheral giant cell lesion as a differential diagnosis: peripheral ossifying fibroma, pyogenic granuloma. The action to take was surgical removal of the lesion for subsequent histopathological study extractions of teeth 25, 27 and 28. The importance of this case the clinical presentation and the large bone destruction that occurred, a condition that determined the extractions of the teeth involved, a fact which is unusual for this type of pathology

Hiperplasia cutánea linfoide (Linfocitoma cutis) secundaria a fármacos: hallazgos clínicos y morfológicos / Cutaneous lymphoid hyperplasia (lymphocytoma cutis) due to drugs: clinical and morphological findings

Pseudolinfoma cutáneo es el término utilizado para indicar un grupo de trastornos linfoproliferativos benignos de la piel, siendo el linfocitoma cutis una forma de seudolinfoma de células B. Clínicamente se manifiesta como nódulos o placas solitarias, generalmente localizados en la cara de mujeres jóvenes. Su etiopatogenia estaría relacionada con una respuesta inmunológica local exagerada ante diversos desencadenantes tales como, picadura de insectos, acupuntura, vacunas, tatuajes, medicamentos e infecciones. Se presentan dos casos de pacientes con diagnóstico de linfocitoma cutis realizado en la Unidad de Anatomía Patológica del Hospital Hernán Henríquez de Temuco.

Cutaneous pseudolymphoma is the term used to indicate a group of benign lymphoproliferative disorders of the skin, being the cutaneous lymphomas a form of B-cell pseudolymphoma. Clinically it manifests as solitary nodules or plaques, usually located on the face of young women. Its pathogenesis could be related to an exaggerated local immune response to various causes such as insect bites, acupuncture, vaccinations, tattoos, drugs and infections. We present two cases of patients diagnosed with skin linfocitoma made in the Pathology Unit in Hernán Henríquez Hospital of Temuco.

Síndrome de Dress y Síndrome de Steven Johnson / Dress Syndrome and Steven Johnson Syndrome

Las enfermedades de la piel muchas veces se asocian a medicamentos que pueden resultar en consecuencias fatales si no se detecta su causa a tiempo. El síndrome de Dress (Drug reaction with eosinophilia and systemic symptoms) es una enfermedad caracterizada por la triada rash, compromiso sistémico y visceral y en la mayor¡a de los casos se ha reportado la presencia de eosinofilia; que se asocia en la mayoría de las veces al uso de antiepilépticos aunque no de manera exclusiva. El síndrome de Steven Johnson, es una reacción mucocutánea que se caracteriza por un rash macular en cara y tronco, con fiebre los primeros tres días y culmina con la presencia de bulas que son el resultado de un desacoplamiento de la epidermis y la dermis. Ambas enfermedades están asociadas a infecciones o al uso de medicamentos y mejoran con el diagnóstico oportuno y la suspención inmediata del medicamento...

Manejo clínico y quirúrgico del granuloma periférico de células gigantes / Clinical and surgical management of peripheral giant cell granuloma

El granuloma periférico de células gigantes es una lesión exofítica de la cavidad bucal, pertenece a las "hiperplasias reactivas" En la literatura solamente se han reportado 12 casos en pacientes infantiles, su crecimien-to es rápido, alcanza gran tamaño y se com-porta de manera agresiva ya que absorbe el hueso alveolar, produciendo movilidad dental e interfiere con la erupción. En este artículo se presenta el caso de un paciente masculino de 9 años, 6 meses de edad, el cual mostraba un crecimiento gingival en la zona de premolares superio-res derechos de color rojo, suave, forma irregular de aproximadamente 4 cm. de diámetro con una úlcera en la superficie oclusal, asintomático y de corta evolución. Refiere recidiva en menos de 6 meses; el diagnóstico histopatológico fue de granulo-ma periférico de células gigantes, el trata-miento fue quirúrgico, consistiendo en una toma de biopsia excisional y realizando el curetaje abierto . El objetivo de este trabajo fue identificar oportunamente al granuloma periférico de células gigantes para evitar destrucción del hueso alveolar, evitando así la pérdida de los dientes permanentes...(AU)

The peripheral giant cell granuloma is an exophytic lesion developing in the oral ca-vity; it is one of the "reactive hyperplasias" The etiology is unknown In the literature only 12 cases have been reported in chil-dren, in these patients, it achieved rapid growth, behaving in an aggressive manner as it absorbed alveolar bone, leading to tooth mobility and interferes with eruption. This article presents the case of a male 9 years, 6 months old patient, who showed a red, soft, irregularly shaped gingival swelling involving the right maxillary premolars of 4cm in diameter with an ulcer in the oclusal side, asymptomatic and with short evolution. Recurrence appeared in less than 6 months; histopathologic diag-nosis was peripheral giant cell granuloma, the treatment was surgical. The purpose of this study was to identify the peripheral giant cell granuloma to avoid destruction of the alveolar bone, thus avoiding the loss of permanent teeth...(AU)

Reações aos diferentes pigmentos de tatuagens: relato de dois casos / Reactions to the different pigments in tattoos: a report of two cases

A tatuagem é definida como deposição de pigmento intencional ou acidental na pele. Os pigmentos têm sido associados a diversas dermatoses, como a dermatite de contato alérgica, a dermatite liquenoide e as reações fotoinduzidas, granulomatosas, sarcoídeas e pseudolinfomatosas. Enfocam-se os diversos tipos de reações aos pigmentos e a importância de reconhecê-los clinicamente. São relatados dois casos: um de dermatite liquenoide sobre o pigmento vermelho e outro de pseudolinfoma sobre os pigmentos vermelho e lilás e de reação fotoinduzida sobre o amarelo. A remoção geralmente requer múltiplos tratamentos, e a maioria não retira as cores completamente.

Tattoos are defined as the intentional or accidental deposit of pigment into the skin. These pigments have been associated with various dermatoses such as allergic contact dermatitis, lichenoid dermatitis, photoinduced reactions, and granulomatous, sarcoid and pseudolymphomatous reactions. The objective of this report was to describe the various types of reactions to pigments and the importance of recognizing them clinically. Two cases are reported: one of lichenoid dermatitis resulting from a reaction to the red pigment of a tattoo and the other of a pseudolymphoma resulting from a reaction to red and lilac pigments and a photo-induced reaction to a yellow pigment. Removal generally requires multiple forms of treatment, most of which fail to remove the colors completely.

Compromiso pulmonar en síndrome de Sjõgren: caso clínico: [revisión] / Pulmonary involvement in primary Sjõgren's syndrome: a case report: [review]

Se presenta el caso de una mujer de 53 años con historia de aumento de volumen y dolor articular, pérdida de peso y síntomas sicca. El estudio de laboratorio mostró VHS elevada, anemia, linfopenia, hipocomplementemia y autoanticuerpos contra SSA/Ro y SSB/La, compatibles con un síndrome de Sjõgren (SS). Además, la radiografía de tórax mostró múltiples nódulos pulmonares, lo que fue confirmado por una TAC de tórax. El estudio histológico de los nódulos, plasmocitosis y proliferación linfocitaria atípica no demostró linfoma. Es ampliamente conocido que el SS confiere un mayor riesgo de desarrollar sindromes linfoproliferativos, lo que aumenta cuando hay presencia de vasculitis, hipo-complementemia y linfopenia. En este caso clínico el diagnóstico diferencial de la etiología de los nódulos pulmonares fue particularmente difícil. Dado lo anterior, se discute y revisa la literatura disponible acerca de compromiso pulmonar en SS.

We report the case of a 53-year old woman with a history of joint swelling and pain, weight loss and sicca symptoms. The laboratory showed a high ESR, anemia, lymphopenia, low complements and circulating auto-antibodies against SSA/Ro and SSB/La consistent with Sjõgren´s Syndrome (SS). Interestingly, the chest x-ray revealed multiple nodules in both lungs, which were corroborated by CT scan. The histological study of the pulmonary nodules showed plasmocytosis and atypical lymphocytes, but failed to demonstrate lymphoma. It is widely acknowledged that SS confers a high risk of developing lymphoproliferative syndromes, which is increased when vasculitis, low complements and lymphopenia are present. In this case, it was particularly difficult to elucidate the differential diagnosis of lung nodules. Accordingly, we discuss and review the available literature regarding pulmonary involvement in SS.