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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
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
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 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
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
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
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
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
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,
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
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
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
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