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histiocytosis/ödem

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Congenital systemic Langerhans cell histiocytosis presenting as hydrops fetalis.

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Congenital Langerhans cell histiocytosis (LCH) encompasses a wide spectrum of disease involvement and severity. Congenital "self-healing" cutaneous LCH represents one end of the spectrum, whereas the case we encountered represents the other extreme. A rare case of congenital LCH with severe

Is Langerhan cell histiocytosis complicated with hydrops fetalis exclusively lethal in premature neonates?

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Langerhans cell histiocytosis is a rare disease and is lethal in premature neonates. A male premature neonate born at gestational 33 weeks presented with generalized vesicles, hydrops fetalis with pleural effusion, bilateral cataracts, and severe respiratory distress syndrome complicated with

Facial edema in a woman with a history of histiocytosis X.

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Bilateral eyelid edema: an uncommon presentation of Rosai-Dorfman disease.

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OBJECTIVE Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a rare entity with ophthalmic manifestations in 11% of patients. Orbital and eyelid involvement as the presenting features is even more unusual. METHODS The authors report the clinical findings of a 41-year-old man

Langerhans cell histiocytosis of the orbit 10 years after involvement at other sites.

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Langerhans cell histiocytosis within the orbit generally occurs in children and is marked by periorbital pain and swelling. Although these lesions respond well to minimal local curettage and concomitant corticosteroid injection, this disorder can be extremely aggressive and destructive. Recurrences

A Case of Orbital Langerhans Cell Histiocytosis in an Adult.

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Langerhans cell histiocytosis remains an enigmatic disease with a very heterogeneous presentation. We describe a rare case of orbital Langerhans cell histiocytosis in a 39-year-old female patient who presented right orbital pain and edema of the upper right eyelid. Surgery showed a friable lesion

An unusual case of non-Langerhans cell histiocytosis.

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A 28-month-old boy had firm, red nodules for 4 months that were mostly localized to the face. The eruption was preceded by conjunctivitis, eyelid edema, and swelling of the root of the nose. He also had dyspnea, anisocoria with areflexia, swelling of the parotid glands, and hepatosplenomegaly. A

[Histiocytosis X (Langerhans cell histiocytosis) of temporal bone. Role of scintigraphy in disease progress evaluation].

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The authors present a case report of a 7-year-old girl with Langerhans cell histiocytosis of temporal bone. Edema and bone destruction involved zygomatic process of temporal bone and temporal fossa without ear occupation. The role of picturing diagnosis in evaluation of lesion extent with special

Periocular intralymphatic histiocytosis or localized Melkersson-Rosenthal syndrome?

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We describe three cases of periocular edema with histopathologic features of intralymphatic histiocytosis without extravascular granulomas. All were elderly males with no other significant medical problems. Previous reports of periocular Melkersson-Rosenthal syndrome are identical clinically, and

A case of Langerhans cell histiocytosis mimicking child abuse.

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Langerhans cell histiocytosis is a rare non-malignant disease with clinical heterogeneity. The disease may present with various clinical findings and may imitate many other conditions. In this report we describe a 34-month-old girl who presented with chronic otitis and otorrhea, skull fracture,

Eosinophilic histiocytosis. Histopathology and immunohistochemistry.

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We review the clinical features, histopathology, and immunohistochemistry in three cases of eosinophilic histiocytosis, comparing lymphomatoid papulosis and eosinophilic histiocytosis. Each of the patients presented with self-healing recurrent papules and ulcerative nodules that were associated with

Association of sinus histiocytosis with massive lymphadenopathy and idiopathic hypereosinophilic syndrome.

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A patient was evaluated because of edema, pruritus and generalized painless lymphadenopathy. Laboratory tests showed marked eosinophilia without known etiology. CT scan of abdomen revealed multiple lymph nodes in retroperitoneal area. Lymph node biopsy was reported as sinus histiocytosis, bone

Histologic and immunohistochemical study comparing xanthoma disseminatum and histiocytosis X.

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METHODS As xanthoma disseminatum and histiocytosis X share clinicopathologic features, difficulties in diagnosis can arise. The use of immunocytochemical markers for S100 protein, factor XIIIa, lysozyme, alpha 1-antitrypsin, for adherence of peanut agglutinin and of antibodies LN3 (HLA-DR), Leu-M1

Pulmonary involvement of malignant histiocytosis: a clinicopathologic spectrum.

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We described five patients in whom pulmonary malignant histiocytosis was histologically confirmed during life. Pulmonary symptoms dominated the clinical presentation in three patients, and one patient had a 5-year history of pulmonary malignant histiocytosis controlled by steroids. Radiologic
OBJECTIVE To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. METHODS The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated
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