Juvenile xanthogranuloma: a rare benign histiocytic disorder

نویسندگان

  • Aneta Szczerkowska-Dobosz
  • Dorota Kozicka
  • Dorota Purzycka-Bohdan
  • Wojciech Biernat
  • Marta Stawczyk
  • Roman Nowicki
چکیده

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder that typically affects children. The clinical presentation of this disease is characterized by single or, rarely, multiple yellow and brown skin nodules, most often found on the face and neck. Internal organ involvement has been sporadically observed in JXG and is associated with an increased risk of serious complications. We report two cases with a small and large nodular form of JXG.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

The “Rare” or “Non-LCH” Histiocytic Disorders in Childhood: A Brief Overview

Diseases of the monocyte, macrophage and dendritic cell system are referred to as histiocytoses. Based on improved understanding of their pathobiology and molecular background histiocytoses have been recently re-classified into five groups. Nevertheless, for practical reasons the histiocytoses are grouped into: Langerhans cell histiocytosis (the most common entity), hemophagocytic lymphohistioc...

متن کامل

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

Juvenile xanthogranuloma (JXG xanthogranulomas (JXGs) are asymptomatic; benign; self papules 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 iris of the eye, anterior abdominal wall and tibia had been reported in literature. common form of non–Langerhans cell histiocytosis. Approximately 35% of ...

متن کامل

Juvenile xanthogranuloma of the ear: A case report.

Juvenile xanthogranuloma is a relatively uncommon, benign histiocytic proliferative disorder. A case of juvenile xanthogranuloma in a 13-month-old girl with an unusual clinical form is described. She presented with a yellow-red projecting nodule over the helical rim of her right ear. The lesion was histologically diagnosed as juvenile xanthogranuloma after excisional biopsy. Although primarily ...

متن کامل

Activating MAPK1 (ERK2) mutation in an aggressive case of disseminated juvenile xanthogranuloma

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder that is usually benign and self-limiting. We present a case of atypical, aggressive JXG harboring a novel mitogen-activated protein kinase (MAPK) pathway mutation in the MAPK1 gene, which encodes mitogen-activated protein kinase 1 or extracellular signal-regulated 2 (ERK2). Our analysis revealed that the mutation results in constitut...

متن کامل

Rapid-growing juvenile xanthogranuloma on the nose of a 16-year-old boy

Juvenile xanthogranuloma (JXG) is an uncommon histiocytic cutaneous lesion. It is a type of non-Langerhans cell histiocytosis(WHO Class IIb). The mean age of onset is 2 years of age. The adult form of JXG is relatively rare. The most common affectedarea is the face or the scalp and most of the lesions are less than 5 mm in diameter. This lesion tends to show a self-limited course over several m...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:

دوره 31  شماره 

صفحات  -

تاریخ انتشار 2014