Multisystem langerhans cell histiocytosis with advanced orbital involvement: case report.
نویسندگان
چکیده
Langerhans cell histiocytosis (LCH) is a rare disease occurring most frequently in infancy or early childhood. The aetiology is still not completely understood, although some form of immunologic dysfunction has been implicated. Clinically, the disease may either be localised or present with multisystem involvement. Here we discuss the case of a seven year old boy presenting with advanced proptosis. To the best of our knowledge, proptosis of this extent has not been reported previously in association with LCH.
منابع مشابه
Langerhans cell Histiocytosis: Seborrheic dermatitis-like skin signs and polypoid lesion of external ear in a 41-year-old man (Case report)
Langerhans cell histiocytosis includes a broad spectrum of clinical diseases resulted from proliferation and tissue infiltration of differentiated langerhans cells. This term includes Letterer-siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma and congenital reticulohistiocytosis that are more common in children than adults. This entity has recently been classified into restr...
متن کاملLangerhans cell Histiocytosis: Report of a case with cutaneous lesions with favorable response to oral Thalidomide
We report of a 29-year-old man presented with yellow-red, scaling papules on the skin of scalp, retro-auricular folds, inguinal, axillary, chest and abdomen. The lesions had a poikilodermatious appearance in some areas, such as trunk. The patient also had diabetes insipidus and skull osteolytic lesions. The diagnosis of langerhans cell histiocytosis (LCH) was suggested based on H and E staining...
متن کاملLangerhans cell histiocytosis
Introduction: Langerhans cell histiocytosis (LCH), which was previously known as histiocytosis X, refers to a group of lesions that stimulate uncontrolled proliferation of cells. The purpose of this case was to report and describe a LCH case with mandibular bone involvement in a 2.5-year-old boy. Case reports: A 2.5 years old boy with LCH of mandibular jaw who has been treated by surgical inter...
متن کاملIndeterminate cell histiocytosis: a case report
Indeterminate cell histiocytosis is a very rare proliferative disorderof histiocytes which displays both langerhans cell histiocytosisand non langerhans cell histiocytosis immunophenotypic features.The majority of the patients develop multiple lesions which areclinically indistinguishable from generalized eruptive histiocytosis.We report a 14-year-old girl with multiple papules on the face,scal...
متن کاملCurrent Treatment Strategy in Langerhans Cell Histiocytosis
Langerhans cell histiocytosis (LCH) is a rare disorder described as three different entities including eosinophilic granulomaof bone, the Hand-Schuller-Christian syndrome, and Letterer-Siwe disease. LCH is currently classified into singlesystem LCH, and multisystem LCH. Patients with single system LCH have an excellent prognosis, and are mostlytreated with local therapy. Multisystem LCH is subd...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- East African medical journal
دوره 87 10 شماره
صفحات -
تاریخ انتشار 2010