Presentación clínica de patologías no malignas que simulan leucemia cutis: a propósito de dos casos / Clinical presentation of non-malignant diseases mimicking leukemia cutis: A report of two cases

La infiltración cutánea por células leucémicas conocida como leucemia cutis es una presentación infrecuente de esta patología y constituye un desafío diagnóstico. Los diagnósticos como infecciones, otras patologías neoplásicas con afectación cutánea y los trastornos histiocíticos, entre otros, constituyen los principales diagnósticos diferenciales, ya que configuran un escenario pronóstico y terapéutico diferente. Se presentan dos pacientes que fueron diagnosticados inicialmente como leucemia cutis, cuyo diagnóstico final fue de patologías no malignas.

The infiltration of leukemia cells into the skin, known as leukemia cutis, is a rare presentation of this disease and accounts for a diagnostic challenge. The main differential diagnoses include infections, other neoplastic diseases with skin involvement and histiocytic disorders, among others, as they entail different prognostic and therapeutic approaches. Here we describe two patients who were initially diagnosed with leukemia cutis, whose final diagnosis was of non-malignant diseases.

Xantogranuloma juvenil gigante: acometimento palpebral difuso e simultâneo / Giant Juvenile Xanthogranuloma: diffuse and simultaneous palpebral involvement

O xantogranuloma juvenil (XGJ) é um tumor benigno e o mais comum do grupo das doenças histiocitárias proliferativas nãoLangerhans. Lesões; 2cm são consideradas XGJ gigantes, com relatos de lesões de até 18cm. Lesões oculopalpebrais podem necessitar de tratamento cirúrgico para controle de sintomas. Esse trabalho relata o caso de um menino de 8 anos que teve as 4 pálpebras acometidas por XGJ gigantes, além do terço médio. Ele foi submetido a 3 ressecções, sendo uma bastante profunda, necessitando enxerto de pele de espessura total diretamente sobre o músculo levantador da pálpebra superior. Posteriormente, 3 procedimentos de lipoenxertia foram realizados, atingindo resultado funcional e estético adequado, sem recorrência lesional.

Juvenile xanthogranuloma (JXG) is the most common benign tumor of the group of non-Langerhans histiocytic proliferative diseases. Lesions >2 cm are considered giant JXG, with reports of lesions of up to 18 cm. Oculopalpebral lesions may require surgical treatment to control symptoms. This study reports a case of an 8-year-old boy who had four eyelids and the middle third of the face affected by giant JXG. He underwent three resections, one of which was of great depth that required a full-thickness skin graft directly on the levator palpebrae superioris muscle. Subsequently, four fat-grafting procedures were performed and adequate functional and

Xantogranuloma juvenil múltiple, a propósito de un caso / Juvenile Multiple Xanthogranuloma, a case report

RESUMEN El xantogranuloma juvenil es la histiocitosis no Langenhans más frecuente en lactantes y niños menores de dos años, generalmente de carácter benigno y autolimitado. Clínicamente se presenta como neoformaciones únicas o múltiples papulonodulares, asintomáticas. Puede asociarse a neurofibromatosis tipo 1 y a leucemia mieloide crónica juvenil. Si bien el compromiso extracutáneo es infrecuente, siempre debe descartarse en lesiones múltiples. Presentamos el caso de un lactante de sexo masculino de 3 meses de edad con diagnóstico de xantogranuloma juvenil con lesiones múltiples y en progresión, por lo que se inicia tratamiento con corticoides tópicos de mediana potencia al que presenta buena respuesta.

ABSTRACT Juvenil xanthogranuloma is the most common non-Langenhans histiocytosis in children under two yeas old, usually benign and self-limited. Clinically presented as single or multiple neoformations like papules and nodules, asymptomatic. Can be associated with neurofibromatosis 1 and juvenile chronic myelogenous leukemia. Although the extracutaneous involvement is infrequent, it should always be discarded in multiple lesions. We present the case of a 3-month old infant with diagnosis of juvenile xanthogranuloma with multiple and increasing lesions, so topical corticoid treatment is initiated showing succesfull clinical response.

Juvenile Xanthogranuloma: A Case Report and Review of the Literature / Xantogranuloma Juvenil: Informe de un Caso y Revisión de la Literatura

SUMMARY: Juvenile xanthogranuloma (JXG), is a benign histiocytic proliferation of uncertain histiogenesis which was first described by Adamson in 1905. It is a regressing disorder which occurs in children usually within first year of life. A child of ten months age reported to the Azeezia College of Dental Sciences and Research with a nodular swelling on the right side of the cheek and gave a history of swelling since the age of 5 months with gradual increase in size which was not associated with pain or itching. A provisional diagnosis of Haemangioma was made and excision biopsy of the lesion was done under general anaesthetia. Depending on the histopathologic and immunohistochemical findings a diagnosis of Juvenile Xanthogranuloma was made. The excisional biopsy site healed uneventfully with minimal scar formation. JXG is a benign fibrohistiocytic lesion and a type of granulomatous process. Pathogenesis of the lesion is unknown. It is generally considered to be a reactive lesion. Most common presentation is as solitary cutaneous lesion. Children are affected at a median age of 2 years with a male female ratio of 1.5:1. Classic histopathologic findings include Nodular to diffuse collection of histiocytes with finely vacuolated foamy cytoplasm and round to oval nuclei, Touton giant cells which are the cells with a central wreath of nuclei and peripheral rim of eosinophilic to vacuolated cytoplasm loaded with fat and Inflammatory infiltrate such as lymphocytes and eosinophils. JXG has to be clinically differentiated from Xanthoma, Molluscum contagiosum, Haemangioma and Neurofibroma. Mostly a self-limiting disease which spontaneously resolves. Conservative management is the treatment of choice. Excision may be done due to esthetic and diagnostic reasons. Recurrence is uncommon. JXG is a disease predominantly of early childhood, benign and self-healing.

RESUMEN: El xantogranuloma juvenil (JXG) es una proliferación histiocítica benigna de histiogénesis incierta que fue descrita por primera vez por Adamson en 1905. Es un trastorno regresivo que ocurre en los niños generalmente durante el primer año de vida. Un niño de diez meses de edad consultó al Colegio de Ciencias e Investigación Dental Azeezia por la presencia de hinchazón nodular en el lado derecho de la mejilla y un historial de hinchazón desde la edad de 5 meses con un aumento gradual en el tamaño que no estaba asociado con dolor o comezón. Se realizó un diagnóstico provisional de hemangioma y se realizó una biopsia de escisión de la lesión con GA. A partir de los hallazgos histopatológicos e inmunohistoquímicos, se realizó un diagnóstico de Xantogranuloma Juvenil. El sitio de la biopsia por escisión se curó sin incidentes con una formación de cicatriz mínima. JXG es una lesión fibrohistiocítica benigna y un tipo de proceso granulomatoso. La patogenia de la lesión es desconocida. Generalmente se considera que es una lesión reactiva. La presentación más común es como una lesión cutánea solitaria. Los niños se ven afectados a una edad media de 2 años con una proporción de hombres y mujeres de 1,5:1. Los hallazgos histopatológicos clásicos incluyen colección nodular a difusa de histiocitos con citoplasma espumoso finamente vacuolado y núcleos redondos a ovalados, células gigantes de Touton que son las células con una corona central de núcleos y margen periférico de citoplasma eosinófilo a vacuolado cargado con grasa e infiltrado inflamatorio como linfocitos y eosinófilos. JXG tiene que ser clínicamente diferenciado de Xanthoma, Molluscum contagiosum, Hemangioma y Neurofibroma. Es una enfermedad principalmente autolimitante que se resuelve espontáneamente. El tratamiento conservador es el tratamiento de elección. La escisión puede realizarse por razones estéticas y diagnósticas. La recurrencia es poco común. JXG es una enfermedad predominantemente de la primera infancia, benigna y autocurable.

Relevancia del xantogranuloma juvenil en pacientes con neurofibromatosis tipo 1: Revisión de un caso clínico / The relevance ofjuvenile xanthogranuloma in neurofibromatosis type i patients. Aclinical case

El xantogranuloma juvenil es una forma de histiocitosis de células no Langerhans que suele afectar a los niños y niñas dentro de los primeros años de vida. Su principal relevancia radica en la posible asociación a neurofibromatosis tipo 1, ya que su presentación conjunta conlleva un mayor riesgo de desarrollo de leucemia mielomonocítica crónica juvenil. Se presenta el caso de un lactante diagnosticado con neurofibromatosis tipo 1 en que se detectan lesiones múltiples compatibles con xantogranuloma juvenil.

Juvenile xanthogranuloma is a form of non-Langerhans cell histiocytosis which usually affects boys and girls in their early years. Its importance stemsfrom its possible association with neurofibromatosistype 1 asthe combined presentation brings a heightened risk of developing chronic juvenile myelomonocytic leukemia. We present the case of an infant diagnosed with neurofibromatosistype I who was found to have multiple lesions compatible with the diagnosis of juvenile xanthogranuloma.

Radiological characteristics of intracranial juvenile xanthogranuloma:report of two cases / 医疗卫生装备

Objective To explore the relationship between the radiological characteristics and clinical and pathological manifestations of intracranial juvenile xanthogranuloma. Methods The radiological and clinical characteristics of two intracranial juvenile xanthogranuloma cases were analyzed respectively. In combination with reviewing literatures, the radiological characteristics and discipline of intracranial juvenile xanthogranuloma disease were also analyzed. Results Intracranial juvenile xanthogranuloma disease was accidently detected in one case. The other case was detected with headache and numbness in face. No abnormal sign was found in skin. On plain CT isodensity signals appeared and on contrast-enhanced CT homogeneous enhancement was found.On MR imaging,masses showed isointense signals on T1WI and iso-or hypo-intensity signals on T2WI, and enhanced homogeneously. Lesions were solitary or multiple in the external of brain. After operation intracranial juvenile xanthogranuloma was confirmed by pathology, and no recurrence occurred. Conclusion Intracranial juvenile xanthogranuloma is a kind of rarely encountered disease with typically radiological characteristics, which is difficult to detect before surgery and can be confirmed by post-operative pathological and immunohistochemical examinations.[Chinese Medical Equipment Journal,2018,39(5):71-74]

Xantogranuloma juvenil solitario congénito: Caso clínico / Solitary and congenital juvenile xanthogranuloma: Case report

El xantogranulomajuvenil es una patología benigna y representa la forma más común de histiocitosis de células no Langerhans. Está caracterizado por la presencia de pápulas o nodulos firmes de coloración rosada o amarillo amarronada, que comprometen, principalmente, la piel y, de forma excepcional, otros órganos. Es una entidad autolimitada con una involución espontánea en los primeros 5 años de vida. Presentamos a una paciente de 1 mes de vida con una lesión congénita en el abdomen, cuya histopatología mostró la presencia de células gigantes multinucleadas de Touton, características de esta patología. Destacamos la presentación infrecuente de este tipo de lesión y la importancia de los múltiples diagnósticos diferenciales que se deben tener en cuenta debido a la edad de la paciente y a sus características.

Juvenile xanthogranuloma is a bening pathology and it represents the most common form of non-Langerhans cell histiocytosis. It is characterized by the presence of papules or firm nodules of a pinkish or yellow-brownish nature, which mainly compromise the skin and, exceptionally, other organs. It is a self-limited entity having a spontaneous regression during the first five years of life. We report the case of a one-month-old patient who presented a congenital tumor in the abdomen, whose histopathology showed the presence of multinucleated giant Touton cells, which are typical of this pathology. We emphasize the rare occurrence of this type of lesion and the importance of the multiple differential diagnosis to be taken into account due to the age of the patient and the characteristics of the lesion.

A Case of Multiple Juvenile Xanthogranuloma with Involvement of the Glans Penis / 대한피부과학회지

No abstract available.

Xantogranuloma juvenil del adulto: Reporte de un caso clínico y revisión de la literatura / Juvenile xanthogranuloma in adult: A case report and review

El xantoganuloma juvenil es una enfermedad benigna, poco frecuente en el adulto, forma parte del grupo de las histiocitosis de células no Langerhans, representando el 90 % de las mismas y el 15 % de éstas, se presentan en el adulto. El compromiso de otros órganos es excepcional. La evolución suele ser benigna con autoresolución de las lesiones. Presentamos el caso de una paciente de sexo femenino de 27 años de edad, que presentaba lesiones papulares de 15 días de evolución, en zona periocular, asintomáticas, las que fueron biopsiadas con diagnóstico histopatológico de xantogranuloma juvenil.

Juvenile Xanthogranuloma (JX) is an uncommon benign disease in adults, is part of the group of non-Langerhans cells histiocytosis, representing 90 % of them and 15% of these occur in adults. The involvement of other organs is exceptional. The evolution is usually benign with resolution of disease. We present a case of a female patient of 27 years who had papules with 15 days of evolution, in periocular area, asymptomatic, which were biopsied with histopathologic diagnosis of juvenile xanthogranuloma.

Xantogranuloma juvenil múltiple: variante inusual en un lactante / Multiple juvenile xanthogranuloma: unusual variant in an infant

El Xantogranuloma Juvenil (XJ) es un tumor benigno de la infancia, constituido por histiocitos progresivamente lipidizados, en ausencia de anomalías metabólicas. Se reporta un caso de XJ múltiple en un lactante. Paciente masculino de 1 año y 11 meses de edad, sin antecedentes mórbidos. Consultó por lesiones de 15 meses de evolución en cuero cabelludo. Al examen físico destacaban dos pápulas amarillentas y tres máculas anaranjadas en cuero cabelludo. A la dermatoscopía se apreciaba un color amarillonaranja homogéneo. La biopsia incisional demostró infiltrado de histiocitos en dermis y células de Touton. A la inmunohistoquímica, los histiocitos fueron CD68 positivo, S100 negativo y CD1a negativo. Los exámenes de laboratorio y la evaluación por oftalmología no evidenciaron anormalidades. El XJ es un tumor benigno, siendo la forma más común de Histiocitosis no Langerhans. El 90% de las veces ocurre en etapas tempranas de la vida. Se caracteriza por una pápula o nódulo asintomático, amarillo-anaranjado, que compromete cabeza, cuello y/o tronco superior. Hasta el 82% de los casos se manifiesta de manera única. El ojo es el sitio extracutáneo más frecuentemente comprometido. La dermatoscopía describe un patrón de "puesta de sol". Su pronóstico es bueno y tiende a involucionar a los 3-6 años desde su aparición. Se presenta este caso dado que el XJ múltiple es menos frecuente de observar que su forma única y para recordar que su asociación con neurofibromatosis tipo 1 predispone a un mayor riesgo de desarrollar una leucemia mieloide crónica infantil.

The Juvenile Xanthogranuloma (JX) is a benign tumor of childhood, composed of progressively lipidized histiocytes in the absence of metabolic abnormalities. A case of multiple JX is reported in an infant. Male patient of 1 year and 11 months, without morbid history. Checking injuries in scalp of 15 months of evolution. At physical examination highlights two yellow papules and three orange macules in the scalp. Dermatoscopy shown a homogeneous yellow-orange color. The incisional biopsy showed infiltration of histiocytes in the dermis and Touton cells. At immunohistochemistry, histiocytes were CD68 positive, S100 negative and CD1a negative. Laboratory tests and evaluation by Ophthalmology showed no abnormalities. The JX is a benign tumor, been the most common form non- Langerhans histiocytosis. This taking place 90% of the time in early life, and characterized by yellow-orange asymptomatic papule or nodule, which undertake head, neck and / or upper body. Up to 82% of cases appears uniquely. The eye is the most frequently committed extracutaneous site. The dermoscopy describes a pattern of "setting sun". His prognosis is good and tends to return to the 3-6 years since their appearance. We present this case because the multiple JX is less common to observe that this singular form, and to remember that their association with neurofibromatosis type 1 predisposes to increased risk of developing childhood chronic myeloid leukemia.

Bilateral juvenile xanthogranuloma of the buttocks - An unusual location: Case report.

Juvenile xanthogranuloma (JXG) predominantly occurs in infancy and childhood. Juvenile xanthogranulomas (JXGs) are asymptomatic; benign; self-healing; self-limiting red, yellow, or brownpapules and nodules composed of histiocytic cells. Most common site of occurrence is skin of the head and neck region and trunk but occurrence at rare sites such as nasal cavity, limbus of the eye, iris of the eye, anterior abdominal wall and tibia had been reported in literature. JXG is the most common form of non–Langerhans cell histiocytosis. Approximately 35% of cases of juvenile xanthogranuloma (JXG) occur at birth, with as many as 71% of cases occurring in the first year. The mean age at presentation is 22 months. Most JXGs resolve by age 5 years. Despite the term juvenile in the disease name, 10% of cases manifest in adulthood. Most of the lesions are solitary. Juvenile xanthogranuloma is a histiocytic inflammatory disorder capable of presenting as different histological patterns. The classic form consists of sheets of foamy histiocytes and numerous multinucleated Touton-type giant cells containing no to very few mitotic figures. We present a case of large juvenile xanthogranuloma (JXG) in a 7 year old child involving an unusual location and bilateral involvement of both the buttocks.

Hallazgos dermatoscópicos del xantogranuloma juvenil / Dermoscopic findings of juvenile xanthogranuloma

El xantogranuloma juvenil es una enfermedad benigna fibrohistiocítica poco frecuente, que forma parte del diverso grupo de desórdenes proliferativos histiocíticos de células no-Langerhans. Se presenta principalmente de forma congénita o antes del primer año de edad, aunque también se han descripto casos aislados en adultos.Presentamos cuatro pacientes sin antecedentes patológicos de importancia, que acudieron a la consulta externa por presentar lesiones papulosas asintomáticas, amarillo-anaranjadas, en las que se encontraron hallazgos dermatoscópicos e histopatológicos característicos de xantogranuloma juvenil.

Juvenile xanthogranulomais a rare, benign fibrohistiocytic disease that belongs to the wide group of non-Langerhans cell histiocytoses. It develops congenitally or before the first year of life; however there are few cases reported in adults.We present four patients with no significant medical history, who attended because of asymptomatic, yellowish-orange papularcutaneous lesions that were dermatoscopic and histopathologically consistent with juvenile xanthogranuloma.

Congenital Juvenile Xanthogranuloma of Foot, a Nodular Lesion: An Unusual Case in 2-month-old Infant.

A 2-month-old infant presented with a circumscribed nodule on left foot since birth. Excision biopsy showed juvenile xanthogranuloma, an uncommon diagnosis in an unusual site; common sites being head and neck. Uncommon sites are groin, genital organs, limbs and even internal organs. It carries a favorable prognosis.

Cataract surgery in juvenile xanthogranuloma: Case report and a brief review of literature.

There is limited literature on the management of cataracts in juvenile xanthogranuloma (JXG). A 2‑month‑old girl presented to us with hyphema, secondary glaucoma OU and skin nodules suggestive of JXG. She developed bilateral cataracts during her follow‑up and was treated successfully with cataract surgery and aphakic rehabilitation.

Solitary Xanthogranuloma of the Upper Cervical Spine in a Male Adult

We present the rare case of solitary xanthogranuloma in the upper cervical column mimicking a Brown-Sequard syndrome. A 29-year-old man complained with right hemiparesis and left hypoesthesia after a car accident. Computed tomography and magnetic resonance images revealed a lobulated homogenously well-enhancing mass in between posterior arch of the atlas (C1) and spinous process of the axis (C2) resulting in a marked spinal canal narrowing with cortical erosions. The patient was managed by complete resection of the tumor with partial laminectomy with lower half of C1 posterior arch and upper half of C2 spinous process. The authors advise complete removal of the xanthogranuloma and consideration as a differential diagnosis of lesions among upper cervical lesions.

A Case of Juvenile Xanthogranuloma in the Lacrimal Sac

PURPOSE: To report a rare case of juvenile xanthogranuloma in the lacrimal sac. CASE SUMMARY: An 8-year-old female presented to our hospital with epiphora and palpable mass on the left medial canthal area. As a symptom, a sticky and bloody discharge through the punctum had begun on occasion 3 months previously. When pressure was applied on the left medial canthal area, a mucoid discharge through the punctum occurred, and a solid mass was palpable. The mass localized in the lacrimal sac and upper nasolacrimal duct was observed on orbit CT. No invasion into the surrounding tissue was present. The authors of the present study decided to perform endonasal dacryocystorhinostomy, and subsequently a skin-colored mass was found in the lacrimal sac. The histopathological and immunohistochemical diagnosis was histiocytic proliferation, compatible with juvenile xanthogranuloma. Six months later, the patient complained of epiphora recurrence with palpable mass on medial canthal area. The tumor recurrence was confirmed with enhanced orbit CT finding 14 months after the first surgery. A second surgery using endonasal dacryocystorhinostomy was performed to remove the mass.

A Case of Giant Nonlipidized Juvenile Xanthogranuloma / 대한피부과학회지

Juvenile xanthogranuloma, which is the most common non-Langerhans cell histiocytosis of childhood, presents characteristic histopathologic features including abundant foamy macrophages and Touton giant cells. Generally, juvenile xanthogranuloma shows clinical features of less than 20 millimeters in size, butrarely, some lesions called giant juvenile xanthogranuloma exceed 20 millimeters. We report a case of a 5-month-old boy with solitary giant juvenile xanthogranuloma on his postauricular area with the histologic feature of nonlipidized form. The patient received simple excision. No evidence of local recurrence or systemic involvements have been observed during 4 months of follow up.

Rapid-Growing Juvenile Xanthogranuloma on the Scalp in 18-Month-Old Girl

Juvenile xanthogranuloma (JXG) is an uncommon histiocytic cutaneous lesion. An 18-month-old girl visited our clinic due to rapid growing orange-yellowish lesion on scalp. Enlarging time from 1 mm to 12 mm was just 8 weeks. We excised the tumor and adjacent normal tissue. Histopathological study showed numerous eosinophils and Touton giant cells within the lesion. Immunohistochemical study revealed positive immunoreactivity for CD68 in most areas. No recurrence was seen during 12 months after resection. We report a case with rapidly growing JXG on scalp with peculiar histopathologic findings.

Benign Cephalic Histiocytosis: A Case Report

Histiocytic skin disorders are usually classified as either Langerhans' cell histiocytosis (LCH) or non LCH, based on the pathology. Benign cephalic histiocytosis (BCH) is a rare type of non-Langerhans histiocytitic disorder and is characterized by self-healing multiple small eruptions of yellow to red-brown papules on the face and upper trunk. Histologic features of this disorder show dermal proliferation of histiocytes that have intracytoplasmic comma-shaped bodies, coated vesicles and desmosome-like structures. In this study, we report on a 7-month-old boy who contained small yellow-red papules on his face that spread to his upper trunk. The clinical and histologic features in this patient were consistent with BCH.

Lesión papuloide localizada en mama / Papuloid lesion localized in mamma

Se comunica un caso de xantogranuloma juvenil localizado en mama.

A case of juvenile xanthogranuloma localized in a breast skin is reported.