Histiocitosis de células de Langerhans con afectación de la órbita en un lactante: reporte de un caso / Langerhans cell histiocytosis with orbital involvement in an infant: Case report

La histiocitosis de células de Langerhans es una enfermedad poco frecuente, caracterizada por la proliferación clonal de células dendríticas mieloides CD1a positivas, asociada a un componente inflamatorio significativo. El compromiso óseo es común; en niños, las lesiones líticas más frecuentes son en la cúpula craneal y son raras las lesiones en la órbita. Se presenta a un lactante de 18 meses que consultó por edema periorbitario y proptosis del ojo derecho de dos meses de evolución. Al momento del examen físico, no se encontraron otras alteraciones. Se realizó una tomografía y una resonancia magnética, que mostraron una masa tumoral en el seno maxilar de crecimiento expansivo y erosión del techo de la órbita. La biopsia confirmó la proliferación CD1a positiva; recibió tratamiento con prednisona y vinblastina, con evolución favorable. Un tratamiento precoz puede evitar secuelas significativas en los pacientes cuando son sensibles al tratamiento. Es una entidad poco frecuente que requiere un alto índice de sospecha y un manejo multidisciplinario.

The Langerhans cell histiocytosis is a rare disease characterized by the clonal proliferation of CD1a + myeloid dendritic cells associated with a significant inflammatory component. The localized form of the disease is called eosinophilic granuloma. Bone involvement is common; in children, lytic lesions are most frequently found in the cranial dome being rare in the orbit. We present an 18-month-old infant who consulted due to periorbital edema and proptosis of the right eye, with two months of evolution. The computed tomography and the magnetic resonance imaging showed a maxillary sinus tumor mass of expansive growth and erosion of the roof of the orbit. The biopsy confirmed CD1a+ proliferation; it was treated with prednisone and vinblastine with favorable evolution. It is a rare entity that requires a high index of suspicion and multidisciplinary management. Early diagnosis and treatment leads to a favorable prognosis for the patient.

Histiocitosis de células de Langerhans: el "camaleón" de la dermatopediatría / Langerhans cell histiocytosis

La Histiocitosis de Células de Langerhans (HCL) es una neoplasia mieloide de las células dendríticas Langerhans (CDL), caracterizada por acúmulos de células dendríticas mieloides CD207+. Corresponden a un espectro de enfermedades, desde sólo cutáneas a variantes multiorgánicas. El objetivo de este reporte es describir el caso clínico de un paciente pediátrico, con diagnóstico de Histiocitosis de Células de Langerhans, enfatizando el algoritmo clínico. Paciente masculino de 1 año y 5 meses, con antecedentes de otorrea persistente, refractaria a tratamiento, de un año de evolución. Consulta en policlínico de dermatología por "dermatitis severa" desde hace 1 año. Al examen físico se constatan lesiones tipo dermatitis seborreica generalizadas en tronco y cuero cabelludo, intertrigo fisurado, pápulas eritemato-costrosas plantares con petequias y pus en conducto auditivo externo bilateral. Presenta Hemoglobina 9,5 mg/dl, Hematocrito31,9%, leucocitos 12.400, linfocitos 33,3%, plaquetas 920.000, VHS 27. Subpoblaciones linfocitarias: CD3: 34,7%, C4: 22,7%, CD8: 9,7%, CD19:47,8%. HTLV negativo, VIH negativo. Acaro-test negativo. Dermatopatología: Denso infiltrado de células linfomonocíticas en dermis papilar, con ensanchamiento de estas y gran epidermotropismo, con abundante citoplasma eosinófilo con núcleos arriñonados, CD1a y langerina positivo. Recomendamos elevar la sospecha diagnóstica ante un cuadro de dermatitis seborreica generalizada que esta fuera del rango etario característico y en casos de dermatitis refractarias, donde a pesar de un adecuado tratamiento médico, el paciente persiste comprometido.

Langerhans Cell Histiocytosis (HCL) is a myeloid neoplasm of Langerhans dendritic cells (CDL), characterized by accumulations of myeloid dendritic cells CD207 +. They correspond to a spectrum of diseases, from cutaneous to multi-organ variants. The objective of this report is to describe the clinical case of a pediatric patient with diagnosis of, emphasizing the clinical algorithm. Male patient,1 year and 5 months old, with a history of refractory persistent otorrhea, consulted because of long term severe dermatitis. Physical examination revealed generalized seborrheic dermatitis lesions on the trunk and scalp, cleft intertrigo, plantar erythematous-crusted papules with petechiae, and pus in the external auditory canal. Laboratory findings showed: Hemoglobin 9.5 mg / dl, Hematocrit: 31.9%, leukocytes: 12,400, lymphocytes 33.3%, platelets: 920,000, HSV 27. Lymphocyte subpopulations: CD3: 34.7%, C4: 22.7%, CD8: 9.7%, CD19: 47.8%. HTLV negative, HIV negative. Scabies Negative. Dermatopathology: Dense infiltrate of lymphomonocytic cells in the papillary dermis with widening of the papilla and large epidermotropism, cells show abundant eosinophilic cytoplasm with "kidney nuclei", CD1a and langerin were positive. We recommend elevating the diagnostic suspicion in the face of a generalized seborrheic dermatitis that is outside the characteristic age range and in cases of refractory dermatitis, where the patient persists compromised.

Histiocitosis de células de Langerhans con compromiso vertebral y de partes blandas: Caso clínico / Langerhans cell histiocytosis with vertebral involvement and soft tissue extension: Clinical case

La histiocitosis de células de Langerhans es una enfermedad heterogénea de etiología desconocida, que se caracteriza por la proliferación no controlada de histiocitos. Es poco frecuente y, si bien el compromiso óseo es común, la afectación vertebral es rara. Se presenta una niña de 4 años que consultó por dolor abdominal difuso de un mes de evolución, al que se agregó constipación y, posteriormente, debilidad en los miembros inferiores. El examen físico mostraba clonus e hiperreflexia en los miembros inferiores y la marcha era inestable. Se realizó una resonancia magnética, que mostró la vértebra dorsal 9 (D9) plana con tejido blando patológico en el espacio epidural y laterovertebral. Se realizó una cirugía descompresiva, artrodesis para fijar la columna y toma de biopsia, que confirmó el diagnóstico de histiocitosis de células de Langerhans. Recibió 6 meses de tratamiento con metilprednisona y vinblastina, de acuerdo con el protocolo LCH III, con excelente evolución y remisión completa. Conclusión. Frente a una imagen radiológica de vértebra plana o colapso vertebral, debe pensarse en histiocitosis de células de Langerhans como diagnóstico diferencial.

Langerhans cell histiocytosis is a heterogeneous disease of unknown etiology characterized by proliferation of Langerhans cells. It is a rare disease. Bone involvement is common but vertebral disease is rare. We present a 4 year old patient with abdominal pain and neurologic symptoms. Magnetic resonance showed vertebra plana in D9 with involvement of paravertebral soft tissues. The child underwent surgery for decompression and biopsy. Biopsy confirmed Langerhans cell histiocytosis. She was treated with vinblastine and prednisone during 6 months following LCH-III with complete recovery of neurologic symptoms. Conclusion. Langerhans cell histiocytosis is a differential diagnosis in a radiograph with vertebra plana or collapse of vertebral body.

Histiocitosis de células de Langerhans presentada como adenitis aislada en un lactante: Caso clínico / Langerhans cell histiocytosis presenting as isolated adenitis in an infant: Case report

La histiocitosis de células de Langerhans es una enfermedad infrecuente en el lactante y su presentación como una adenitis cervical aislada sin otra sintomatología es excepcional en estos pacientes. Se describe el caso de una lactante de 3 meses de edad que presentaba una tumoración cervical en el ángulo mandibular derecho, con mala respuesta al tratamiento antibiótico. Se realizó una punción-aspiración con aguja fina, que confirmó el diagnóstico de histiocitosis de células de Langerhans. El estudio de extensión no mostró afectación sistémica. Debe considerarse la histiocitosis de células de Langerhans en el diagnóstico diferencial de una masa cervical subaguda de evolución tórpida en los lactantes de corta edad y se debe plantear la realización de una punción-aspiración con aguja fina de manera precoz para establecer el diagnóstico.

Langerhans cell histiocytosis in infants is a rare condition, and presentation as an isolated cervical adenitis is exceptional at this age. We describe the case of a 3-month-old female infant presenting with a neck mass in the right mandibular angle with poor response to antibiotic treatment. Fine needle aspiration was performed and confirmed the diagnosis of Langerhans cell histiocytosis with complementary tests showing no features of systemic involvement. Langerhans cell histiocytosis should be considered in the differential diagnosis of subacute neck masses with poor outcome in infants and physicians should consider performing a fine needle aspiration to establish the diagnosis.

The concentration of IL-1B in saliva of children with oral lesions associated to histiocytosis / Concentracion de il-1β en saliva de niños con lesiones bucales asociadas a histiocitosis

La Histiocitosis de células de Langerhans(HCL) es una enfermedad de etiología y patogenia aún desconocidas. Afecta diferentes órganos y tejidos en los que produce lesiones de distinta gravedad. La histopatología de las lesiones y la clínica sugieren la participación de citoquinas en su patogenia. La IL-1β podría tener un rol importante en el desarrollo de la enfermedad. El objetivo de este estudio fue determinar las concentracionesde IL-1β de las salivas de pacientes pediátricos con diagnóstico de Histiocitosis de Célula de Langerhans con y sin manifestaciones bucales (grupos 1 y 2 respectivamente), en relación a un grupo control (grupo 3), de pacientes pediátricos que no presentaron antecedentes médicos ni lesiones bucales. Fueron estudiadas las salivas de 20 pacientes con la enfer -medad de HCL, en relación a un grupo control de 11 pacientes pediátricos que no presentaron antecedentes médicos. Los niños con Histiocitosis cuyas edades oscilaban entre 4 meses y 16 años fueron derivados del servicio de Oncohematología del Hospital Garrahan y Hospital de Clínicas, a la Cátedra de Odontología Integral Niños de la Facultad de Odontología de la Universidad de Buenos Aires. Se determinaron las concentraciones de IL-1β en los diferentes grupos, y se utilizó el Enzyme Inmune Assay Kit (Cayman, MI, USA), se expresó en pg/ml. El análisis de los resultados se realizó según el test de Kruskall Wallis, se obtuvieron diferencias significativas entre los tres grupos (H = 20,36; P<0,001). Luego se realizó el análisis de comparaciones múltiples de Dunn que mostró diferencias estadísticamente significativas entre los grupos 1 y 2 y entre los grupos 1 y 3 (p < 0,05). Se observaron valores más elevados de IL-1β en los pacientes con Histiocitosis con manifestaciones bucales (grupo 1), en relación con el grupo sin manifestaciones bucales (grupo 2) y con el grupo control (grupo 3).

Langerhans Cell Histiocytosis (LCH) is a disease whosetiology and pathogenesis are still unknown. It affects several organs and tissues, producing lesions of different severity. Its histopathology and clinical picture suggest the participation of cytokines in its pathogenesis. IL-1β might have an important role in its development. The purpose of this study was to determine the concentrations of IL-1β in saliva of pediatric patients diagnosed with LCH, with and without oral manifestations (Groups 1 and 2respectively) compared to a Control Group (Group 3) of pediatric patients without medical antecedents or oral lesions.The saliva of twenty patients with LCH was studied and compared to a Control Group consisting of eleven pediatric patients without medical antecedents. The children with histiocytosis, aged four months to sixteen years, were referred by the Onco haematology Service at Garrahan Hospital and Hospital de Clínicas, to the Department of Comprehensive Children’s Dentistry, School of Dentistry, University of BuenosAires (UBA).The concentrations of IL-1β in the different groups were determined using the Enzyme Immune Assay Kit (Cayman MI,USA) and expressed in pg/ml. Results were analyzed by the Kruskall Wallis test. Significant differences between the three cohorts were found, (H = 20.36, P< 0.001). Dunn ́s multiple comparison analysis was performed, which showed significant differences between Groups 1 and 2, and between Groups 1 and 3 (P < 0.05). Higher values of IL-1βwere found in the patients with histiocytosis with oral manifestations (Group 1) than in patients without manifestations (Group 2) and patients in the Control Group (Group 3).

Neumotórax bilateral espontáneo como forma de presentación de histiocitosis de células de Langerhans / Bilateral spontaneous pneumothorax as a setting of Langerhans cell histiocytosis

La histiocitosis de células de Langerhans pulmonar es una patología intersticial en la que existe un acumulo de células histiocíticas específicas a nivel pulmonar. El neumotórax espontáneo es una complicación reconocida de histiocitosis de células de Langerhans pulmonar y es secundario a la destrucción del parénquima pulmonar con cambios quísticos asociados. Reportamos el caso de un niño de 2 años con neumotórax espontáneo bilateral recurrente, con una tomografía axial computada de tórax con infiltrado intersticial, fibrosis, lesiones quísticas e imágenes bullosas. El diagnóstico fue establecido por examen histológico e inmunohistoquímica de tejido de biopsia pulmonar con anticuerpos CD1 y S100 positivos. El niño recibió tratamiento con prednisona y etopósido, con buena respuesta clínica y tomográfica.

Pulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan.

Langerhans Cell Histiocytosis Presenting with Hypopigmented Macules.

Langerhans cell histiocytosis (LCH) is a complex disease characterized by proliferation of the Langerhans cells. The clinical course is variable and ranges from a solitary lytic bone lesion or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The diagnosis is suspected by clinical signs and symptoms and radiological features commonly in craniofacial bones and skin lesions. Diagnosis is confirmed by biopsy and immunohistochemical studies. We present case of a 8 year old child presenting with proptosis, diabetes insipidus and hypopigmented macules on chest and back showing bilateral distribution which is a rare presentation. Skin biopsy revealed the diagnosis of Langerhans cell histiocytosis.

Langerhans Cell Histiocytosis Followed by Hodgkin's Lymphoma

A 22-year-old man was referred to our institution due to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar spine. The patient achieved complete remission with radiotherapy and chemotherapy. One year later, right cervical lymphadenopathy was observed and Hodgkin's lymphoma was confirmed on biopsy. The patient was treated with chemotherapy and autologous stem cell transplantation, and experienced no further symptoms. Further, no evidence of recurrence was observed on follow-up imaging. This report discusses the association between Langerhans cell histiocytosis and Hodgkin's lymphoma.

Congenital langerhans cell histiocytosis with skin and lung involvement: Spontaneous regression.

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare type of Langerhans Cell Histiocytosis, presenting at birth or during the neonatal period with eruption of multiple, discrete red-brown papules or nodules that undergo spontaneous regression. Systemic signs are generally absent. We describe a 4-month-old infant presenting with reddish brown nodular cutaneous lesions since birth with a past history suggestive of pulmonary involvement. Skin biopsy showed a dermal infiltrate of pleomorphic histiocytes; which were CD1a and S-100 positive, consistent with the diagnosis of CSHLCH. Both pulmonary and cutaneous lesions showed spontaneous resolution.

Histiocitose de células de Langerhans como causa de mielopatia: relato de caso e revisão da literatura / Langerhans-cells histiocytosis as a cause of myelopathy: case report and literature review

Histiocitose de células de Langerhans é uma doença uni ou multifocal que acomete osso, tecido mole ou ambos. Relativamente incomum, abrange entidades clínicas anteriormente conhecidas como doença de Hand-Schüller-Christian, Abt-Letterer-Siwe, Hashimoto-Pritzker, granuloma eosinofílico e histiocitose X. Responsável por menos de 1% dos tumores ósseos, muito raramente leva a acometimento neurológico. Sua etiologia é desconhecida e seu tratamento, controverso. A ocorrência de um caso com comprometimento neurológico nos motivou a este relato. Paciente do sexo feminino, 4 anos deidade, com processo expansivo de C7 a T3 e paraparesia crural; após a cirurgia apresentou melhora importante já no segundo pós-operatório. Encontra-se em terapia complementar com corticoide e quimioterapia estando no nono mês de tratamento. Na revisão da literatura foram encontrados poucos relatos de manejo cirúrgico desse tipo de lesão, em razão de sua característica de doença autolimitada. O tratamento adequado pode variar dependendo das características do paciente e de seu comprometimento neurológico.

Langerhans cell histiocytosis is a uni- or multifocal disease that affects bone, soft tissue, or both. Relatively uncommon, covers clinical entities previously known as Hand-Schüller-Christian disease,Abt-Letterer-Siwe, Hashimoto-Pritzker, eosinophilic granuloma and histiocytosis X. Responsible forless than 1% of bone tumors, rarely causes neurological impairment. Its etiology is unknown and the treatment controversial. We report a rare case with neurological impairment. A 4 year-old female children, presented with paraparesis due to expansive process at C7 to T3; soon after surgery showed significant improvement in the second postoperative day; she is in complementary therapy with corticosteroids and chemotherapy in the ninth month of treatment. In the review of the literature we found few reports ofsurgical management of this type of injury, due to its characteristic of self-limited disease. The appropriate treatment may vary depending on the characteristics of patients and their neurological impairment.

Histiocitosis de células de Langerhans en menores de un año / Langerhans cell histiocytosis in children under one year

Introducción. La histiocitosis de células de Langerhans se debe a la proliferación clonal de histiocitos activados que invaden diversos tejidos. Se produce en todas las edades, desde el nacimiento hasta la adultez, con un pico de mayor incidencia entre 1 a 4 años. Objetivo. Describir las características de 15 pacientes pediátricos menores de 1 año con diagnóstico de histiocitosis de células de Langerhans, las manifestacionesclínicas y evolución de la enfermedad. Métodos. Estudio descriptivo retrospectivo, realizado en el Hospital Ramos Mejía y el Hospital Alemán, entre 1999 y 2007. Resultados. Se revisaron las historias de 15 pacientes, 6 niñas y 9 niños. Las lesiones en 8 casos estaban presentes al nacer y 7 aparecieron entre los 2 y 12 meses de edad. En las congénitas se observó como única manifestación clínica inicial el compromiso cutáneo. Uno de estos pacientes presentó evolución sistémica, con compromiso pulmonar, hepático y esplénico, actualmente en tratamiento. De los pacientes con lesiones posteriores al nacimiento, sólo 3 tenían compromiso cutáneo únicamente, el resto presentó compromiso sistémico. Uno de estos pacientes falleció durante el tratamiento. En la histopatología se observaron histiocitos en la dermis papilar con marcado epidermotropismo. La inmunomarcación con CD1a y S100 fue positiva. Conclusiones. La histiocitosis de células de Langerhans es una entidad con dos variantes: la forma congénita y la de inicio posterior al nacimiento, ambas con capacidad para producir compromiso sistémico.

Introduction. Langerhans cell histiocytosis is characterized by a clonal proliferation of activated Langerhans cells that infiltrate various organs of the body. Occurs at any age, from newborn until adulthood, with an incidence peak at 1-4 years. Objective. To describe the morphologyc characteristics of skin lesions and clinical course of 15 patients with Langerhans cell histiocytosis. Methods. A retrospective review of the medical records of patients with Langerhans cell histiocytosis from Ramos Mejia Hospital and Aleman Hospital, between 1999-2007. Results. Review of medical records from 15 patients, 6 females and 9 males. Skin lesions were congenital in 8 cases and appeared between 2-12 months of age in 7 cases. The patients with congenital presentation only had a cutaneous manifestation; one patient who developed a systemic compromise (lung, liver and spleen) is currently under treatment. Three patients with presentation after birth only had cutaneous lesions, the others had a sistemic disease. One of this patients died during treatment. Histopathology showed a histiocytic infiltrate in the papilary dermis with epidermotrophism; inmunomarking with S100 and CD1a was positive. Conclusion. Both clinical manifestation (congenital and after birth) represent different ends of a spectrum of the same condition, with the potencial to develop into disseminated Langerhans cell histiocytosis.

Alteraciones maxilofaciales en histiocitosis de células de langerhans: reporte de un caso / Maxillofacial abnormalities in Langerhans cell histiocytosis: a case report

La Histiocitosis de células de Langerhans conocida anteriormente como Histiocitosis X, es un término aplicado a un grupo de desórdenes poco comunes del sistema fagocítico mononuclear,  caracterizado por una acumulación clonal y proliferación anormal de células de Langerhans derivadas de la medula ósea. El propósito de esta investigación es reportar las alteraciones maxilo-faciales en un paciente con diagnostico de Histiocitosis de Células de Langerhans. Se presenta el caso de un varón de tres años de edad con diagnostico de Histiocitosis de Células de Langerhans con afección ósea multifocal y otros sitios especiales, con antecedentes de otitis media supurativa recurrente, diabetes insípida, dermatitis seborreica y lesiones hipercrómicas en miembros inferiores. La exploración extraoral evidenció lesiones eritematosas y descamativas en piel de cara y cuero cabelludo; intraoralmente se observó movilidad en dientes posteriores de ambos maxilares acompañada de recesión gingival, caries dental y erupción prematura del 26. Radiograficamente presentó lesiones osteolíticas en alas mayores de esfenoides, techo orbitario izquierdo, pared orbitaria externa derecha, arco cigomático, peñasco del temporal y zonas posteriores de ambos maxilares, además de agenesia de los gérmenes dentarios del 35 y 45. Adicionalmente al tratamiento sistémico de quimioterapia se realizó control de la infección dentobacteriana y tratamiento quirúrgico de las zonas maxilares afectadas. Se concluye que los maxilares al igual que los huesos del cráneo deben ser evaluados en pacientes con diagnóstico de Histiocitocis de Células de Langerhans, ya que estos sitios pueden ser los únicos huesos afectados como sucedió en el caso estudiado

Langerhans Cell Histiocytosis, known earlier as Histiocytosis X is a term applied to a group of rare disorders of the reticuloendothelial system, characterized  by an abnormal proliferation of Langerhans cells derived from bone marrow. The purpose of this research is to report the maxillofacial alterations of a patient with diagnostic of Langerhans Cell Histiocytosis. A three-year old male patient with diagnostic of Langerhans Cell Histiocytosis with multifocal bone involvement and special sites of disease is presented. He had a previous history of exophtalmus, recurrent suppurative otitis media, diabetes insipidus, seborrheic dermatitis and hyperchromic lesions in lower members. The extraoral exam showed erythematosus and desquamative lesions in face skin and the scalp, intraorally back teeth mobility of both maxilars was found together with gingival recession, dental caries and premature eruption of 26. Radiographically, he presented osteolitic lesions in the sphenoid major wings, left orbital roof, right external orbital wall, zygomatic arch, temporal bone and back areas of both maxilars, besides dental germ agenesia of 35 and 45. In addition to the systemic chemiotherapy treatment, a control of the dentobacterial infection and surgical treatment of the affected maxillary areas was made. The conclusion is that maxilars as well as the skull bones must be evaluated in patients with a diagnostic of Langerhans cell Histiocytocis, because this could be the only bone involvement to be found as it happened in this case

Histiocitose cutânea não-Langerhans como causa de diabetes insípido central / Cutaneous non-Langerhans cells histiocytoses as cause of central diabetes inspidus

As histiocitoses são doenças raras, resultantes de alterações na linhagem monocítica-histiocítica, com manifestações clínicas diversas. Entre as síndromes cutâneas de células não-Langerhans, o xantoma disseminado é a única entidade desse grupo classicamente associada ao diabetes insípido central (DIC). O caso clínico relatado refere-se a um paciente de 30 anos de idade que, dois anos após o diagnóstico de DIC, evoluiu com lesões cutâneas papulosas, eritêmato-acastanhadas, difusas, discretas e não confluentes. Os achados histológicos, imuno-histoquímicos e a microscopia eletrônica mostraram resultados compatíveis com a histiocitose de células não-Langerhans e sugestivos do xantogranuloma juvenil. A avaliação endócrino-metabólica não mostrou alterações durante o seguimento por 10 anos, com exceção do DIC. A ressonância magnética da hipófise demonstrou ausência do sinal hiperintenso (mancha brilhante) correspondente à neuro-hipófise. As radiografias e a cintilografia dos ossos não mostraram lesões osteolíticas. Este caso desperta a atenção para a importância do exame da pele nos casos de DIC e de sua associação com a histiocitose de células não-Langerhans de maneira mais ampla, e não restrita aos casos de xantoma disseminado.

The histiocytoses are rare diseases caused by alterations in the monocyte-histiocytic series with several clinical findings. Among the cutaneous syndromes of non-Langerhans cells, xanthoma disseminatum is the only disease of this group that has been classically associated to the central diabetes insipidus (CDI). The case reported describes a 30-year-old man that two years after presenting with CDI developed non confluent disseminated cutaneous brown papular lesions throughout the body. The histopathology, immunohistochemistry, and electronic microscopy were compatible with the diagnosis of non-Langerhans histiocytoses, suggesting the diagnosis of juvenile xanthogranuloma. The endocrine-metabolic evaluation did not show other alterations besides CDI in a 10-year follow up. The magnetic resonance of hypophysis showed absence of the pituitary hyperintense sign (bright spot). The radiologic and scinthigraphic evaluation of the bones did not show the presence of osteolytic lesions. This case prints out the importance of skin examination in cases of CDI and its association with cutaneous non-Langerhans histiocytoses in a broader spectrum, rather then restricted to the cases of xanthoma disseminatum.

PUO due to Langerhans Cell Histiocytosis.

Langerhan Cell Histiocytosis (LCH) is a disorder in which cells with a phenotype similar to that of epidermal langerhans cells cause tissue damage possibly through excessive cytokine production. The clinical spectrum of the disease is wide. We are reporting a case of LCH who presented with prolonged pyrexia and a clinically benign bony swelling of mandible of long duration, which was otherwise ignored as being unrelated. The biopsy from the swelling confirmed the diagnosis of LCH. Another biopsy from lower end of tibia where he had pain also demonstrated typical findings. There was no evidence of other system involvement. There was good remission of the swellings and the symptoms with steroid alone initially but later relapsed and is now on treatment as per LCH III protocol. The case is being reported for its rarity and for the unusual presentation as PUO.